AM2 Neurology

I.          Meningitis and Encephalitis

            1.      Identify Streptococcus pneumoniae and Neisseria meningitidis as the two most common causes of acute bacterial meningitis in adults.

Jenn, CMDT pp1351 &1367

Streptococcus pneumoniae is the most common cause of meningitis in adults and the second most common cause of meningitis in children over the age of 6 years. Head trauma, cerebrospinal fluid leaks, and sinusitis may precede it.

Meningococcal meningitis is caused by Neisseria meningitidis of groups A, B, C, Y, W-135, and others. Meningitis due to serogroup A is uncommon in the United States. Serogroup B generally causes sporadic cases. The frequency of outbreaks of meningitis caused by group C meningococcus has increased in recent years, and this serotype is the most common cause of epidemic disease in the United States. Up to 40% of persons are nasopharyngeal carriers of meningococci, but relatively few develop disease. Infection is transmitted by droplets. The clinical illness may take the form of meningococcemia (a fulminant form of septicemia without meningitis), meningococcemia with meningitis, or predominantly meningitis. Chronic recurrent meningococcemia with fever, rash, and arthritis can occur, particular in those with terminal complement deficiencies.

(Jenn, Noble pp1551)

The most common organisms responsible for bacterial meningitis in people over the age of 2 months are Neisseria meningitidis, Streptococcus pneumoniae, Haemophilus influenzae, and Listeria monocytogenes, but other organisms such as Escherichia coli and group B streptococci must be considered in neonates.

Megan Noble 1551

The most common organisms responsible for bacterial meningitis in people over 2 months are Nesseria menengitidis and Streptococcus pneumoniae.

Dustin, CMDT pg. 1351, 1367

Streptococcus pneumoniae is the most common cause of meningitis in adults.

Meningococcal meningitis is caused by Neisseria meningitides of groups A, B, C, Y, W-135, and others.  Meningitis due to serogroup A is uncommon in the U.S.  Serogroup B generally causes sporadic cases.  The frequency of outbreaks of meningitis caused by group C meningococcus has increased in recent years, and this serotype is the most common cause of epidemic disease in the U.S.

Anonymous Current p. 1253, Table 30-1.

Strep pneumo and N. meningitidis are the two most common microorganisms causing pneumonia in those 18-50 years of age.

Anonymous Nobel pg 1551

The most common organisms responsible for bacterial meningitis in people over the age of 2 months are Neisseria meningitis, Streptococcus pneumoniae, H. flu, & Listeria moncytogenes, but other organisms like E. Coli and B strep must be considered in neonates.   

Anonymous  Noble 1554

Neisseria Meningitis has a prevalence of 30-40% in adult cases and Strep pneumoniae has a prevalence of about 40-50%.  

            2.      Describe or recognize the classic clinical picture seen in a patient with acute bacterial meningitis.

Jenn, Noble pp1552

Acute Bacterial Meningitis

Clinical Features.

Acute meningitis evolves over hours or, at most, days. Patients who have been quite healthy or have only had an upper respiratory infection now begin to complain of the symptoms of meningeal irritation and of raised intracranial pressure (ICP). Malaise; fever; headaches of traction type; nausea; vomiting; anorexia; stiff, painful neck; and photophobia are the usual initial symptoms. Depression in the level of consciousness, delirium, seizures, and various focal neurologic signs may follow. The association of fever and any form of mental deterioration must make one consider meningoencephalitis as a possible diagnosis. Inflammatory cells in the CSF and accompanying cerebral edema may cause a rapid rise in ICP and produce false localizing signs.

Examination usually shows meningism, with stiff neck and resistance to flexion of the spine or to straight leg raising. Such signs may be absent in overwhelmingly ill adults and in neonates, who may just show the features listed in Box 165-2 , such as a bulging fontanelle, neck stiffness, and drowsiness. Children may also show head retraction, which can be so marked as to warrant the term opisthotonos.

General examination may show a rash (particularly if the meningococcus is involved or echovirus or coxsackievirus is involved); signs of infection in the ears, joints, bones, chest, or heart; and sometimes purpura and hypotension. Shock is common if a gram-negative organism is the cause of septicemia with secondary meningitis and in meningococcal septicemia causing adrenal infarction (WaterhouseFriderichsen syndrome). The combination of fever and purpura in this context can occur with N. meningitidis, echovirus, E. coli, Pseudomonas, Proteus, L. monocytogenes, streptococcus, gonococcus, or Staphylococcus aureus infections.

Megan Noble 1552

Acute meningitis evolves over hours or at most days.  Patients who have been white healthy or have only had an upper respiratory infection now begin to complain of the symptoms of meningeal irritation and of raise intracranila pressure (ICP).  Malaise, fever, headaches of traction type, nausea, vomiting, anorexia, stiff, painful neck and photophobia area the usual initial symptoms.  Depression in the level of consciousness, delirium, seizures and various focal neurologic signs may follow.  The association of fever and any form of mental deterioration must make one consider meningoencephalitis as a possible diagnosis.

Dustin, Noble online

Acute meningitis evolves over hours or, at most, days.  Patients who have been quite healthy or have only had an upper respiratory infection now begin to complain of the symptoms of meningeal irritation and of raised intracranial pressure (ICP).  Malaise; fever; headaches of traction type; nausea; vomiting; anorexia; stiff, painful neck; and photophobia are the usual initial symptoms.  Depression in the level of consciousness, delirium, seizures, and various focal neurologic signs may follow.  The association of fever and any form of mental deterioration must make one consider meningoencephalitis as a possible diagnosis.

Anonymous Current p. 1253

A. Purulent Meningitis.

Presentation: acutely within hours or 1-2 days after onset of symptoms. 

Responsible organisms: Strep pneumo and N. meningiditis in 18-50 year olds.

Anonymous  Noble 1152

Acute meningitis evolves over hours, or at most, days.  Patients who have been quite healthy or have only had an upper respiratory infection begin complaining of meningeal irritation and of increased intracranial pressure.

•        Malaise

•        Fever

•        Headaches of traction type

•        Nausea and vomiting

•        Anorexia

•        Stiff, painful neck

•        Photophobia

•        Depressed LOC, delirium, seizures, and focal neurological signs may follow

Examination usually shows meningism, with a stiff neck and resistance to flexion of the spine or to straight leg raises.  Such signs may be absent in overwhelmingly ill adults and in neonates.  Neonates and older infants may present with a bulging fontanelle, neck stiffness, and drowsiness. General examination may show a rash (particularly if the meningococcus is involved or echovirus or coxsackievirus is involved); signs of infection in the ears, joints, bones, chest, or heart; and sometimes purpura and hypotension.  Shock is common if a gram-negative organism is the cause with secondary meningitis and in meningococcal septicemia causing adrenal infarction.

Anonymous   Noble, p.1552

Malaise, fever, headaches of traction type, nausea, vomiting, anorexia, stiff painful neck, and photophobia. Progressive sx include depression in level of consciousness, delirium, seizures, and various focal neurologic signs. Bulging fontanelles in kids under 2.

            3.      Describe or recognize the typical CSF findings seen in acute bacterial meningitis; identify the WBC with differential and blood cultures as important additional studies to be obtained.

Jenn, Noble pp1552

Evaluating the Cerebrospinal Fluid.

Whenever there is evidence of meningeal inflammation, a lumbar puncture (LP) must be done eventually, even though some patients with meningitis show early papilledema.

The fluid may be turbid or clear; microscopy may show any number of mononuclear or polymorphonuclear cells. PMNs almost always predominate in acute bacterial meningitis. Mononuclear cells usually predominate in the other forms of meningitis (due to Mycobacterium tuberculosis, viruses, or fungi).

Megan Noble 1552-1553

Whenever there is evidence of meningeal inflammation a lumbar puncture must be done.  

               CSF protein levels are raised in all forms of meningitis, particularly those of longer duration or with very high cell counts.  The technology of DNA amplification and PCR can identify the causative agent within 30 minutes.The CSF blood sugar levels are normal with viral meningitis, values are low ( <50% of blood level) in bacterial meningitis.  High PMN counts are to be expected in all cases->50,000/mm³ if the meningitis is due to rupture of an abscess.

               If there is clinical uncetainity as to whether the patient has bacterial or aseptic meningitis and the patient is not seriously ill, the physician may properly maintain careful observation and repeat the LP in 12-18 hours.  With aseptic meningitis, the second CSF sample will almost always show a shift toward mononuclear cell predominance, whereas later examinations will show the eventual disappearance of all PMNs.  In bacterial meningitis, PMNs increase quickly, CSF sugar fall and the protein level rises.

            Blood cultures should be obtained and any skin lesions swabbed before the antibiotic is started.

Anonymous CMDT 1252

Central Nervous system infection constitutes a medical emergency.  Immediate diagnostic steps must be instituted to establish the specific cause.  Normally these include the history, PE, blood count, blood culture, LP, and a chest film.

Dustin, Noble online.

The CSF pressure need not be taken if the initial fluid is cloudy or bloodstained because knowledge of the pressure does not contribute to diagnosis in these circumstances. The fluid may be turbid or clear; microscopy may show any number of mononuclear or polymorphonuclear cells. PMNs almost always predominate in acute bacterial meningitis. Mononuclear cells usually predominate in the other forms of meningitis (due to Mycobacterium tuberculosis, viruses, or fungi).

CSF protein levels are raised in all forms of meningitis, particularly those of longer duration or with very high cell counts.

If there is clinical uncertainty as to whether the patient has bacterial or aseptic meningitis and the patient is not seriously ill, the physician may properly maintain careful observation, withhold antibiotics, and repeat the LP in 12 to 18 hours. With aseptic meningitis, the second CSF sample will almost always show a shift toward mononuclear cell predominance, whereas later examinations will show the eventual disappearance of all PMNs. In bacterial meningitis, PMNs increase quickly, CSF sugar falls, and the protein level rises.

The LP in Meningococcal meningitis typically reveals a cloudy or purulent CSF, with elevated pressure, increased protein, and decreased glucose content.  The fluid usually contains more than 1000 cells/uL, with polymorphonuclear cells predominating and containing gram-negative intracellular diplococci.  (CMDT pg. 1367)

The CSF in pneumococcal meningitis typically has more than 1000 white blood cells per microliter, over 60% of which are polymorphonuclear leukocytes; the glucose concentration is less than 40 mg/dL, or less than 50% of the simultaneous serum concentration; the proteinusually exeeds 150 mg/dL. (CMDT 1351)

Current p. 1253, Table 30-2.

Typical CSF findings in purulent meningitis (bacterial) community acquired:

-200-20,000 polymorphonuclear neutrophils (PMNs)

-Low glucose (<45)

-High protein (>50)

-Opening pressure markedly elevated

Anonymous  Noble 1552

The fluid may be cloudy; microscopy may show polymorphonuclear cells.  PMN’s almost always predominate in acute bacterial meningitis.  CSF protein levels are raised in all forms of meningitis.  A simultaneous estimation of the CSF blood sugar is mandatory when the LP is done.  CSF sugar values will be less than 50% of the blood level in bacterial meningitis.

(Tininalli 1486) A blood culture is mandatory in all cases of meningitis.  Blood cultures (two specimens drawn 15 minutes apart) yield the responsible organism in about 50% of cases of bacterial meningitis, but CSF analysis is paramount.  A WBC with differential is an important additional study.

Anonymous Noble pp. 1552-1555

The fluid may be turbid or clear; microscopy may show any # of mononuclear or PMN cells. PMNs almost always predominate in ABM while mononuclear cells predominate in other types. High PMNs are to be expected in all cases - over 50,000/mm³ if the meningitis is due to rupture of an abscess. Protein levels are raised in all forms of meningitis, particularly with high cell counts or long duration. CSF blood sugar estimations are mandatory when the LP is done – values will be low (< 50% of the blood level) in bacterial meningitis.

In ABM, PMNs increase quickly, CSF sugar falls, and the protein level rises.

Hold the CSF up to the light to see if it is cloudy (over 500 cells/ml) or clear, and then take it to the lab, where a Gram’s stain, culture, cell count, and differential should be performed and the protein and sugar levels estimated. Virus cultures and cytology might be indicated if septic meningitis is excluded by these tests. In some cases (endocarditis, prior head trauma) the CSF may not show many leukocytes and may be sterile. A blood culture is mandatory in all cases of meningitis so the correct diagnosis should not be missed.

            4.      Describe the importance of obtaining CT scan, when possible, prior to lumbar puncture if there is any suspicion of a space-occupying lesion.

Jenn, Noble pp1552

Intracerebral abscess or expanding mass lesions, particularly in the posterior fossa, must be ruled out by physical examination, although in the presence of any focal signs an emergency computed tomography (CT) scan is necessary. In this situation an expert opinion should be requested if available.  LP should be performed when any such masses are excluded because identification of the organism and determination of its sensitivities are essential; however, if a bacterial process is suspected, antibiotics should be started as soon as blood cultures are drawn and before an LP is done.

Megan CMDT 1252

Since performing a lumbar puncture in the presence of a space-occupying lesion ( brain abscess, subdural hematoma, subdural abscess) can result in brain stem herniation and death, a CT scan is performed prior to LP if a space occupying lesion is suspected on the basis of papilledema, coma, seizures, or focal neurologic findings.

Dustin, Noble online.

Whenever there is evidence of meningeal inflammation, a lumbar puncture (LP) must be done eventually, even though some patients with meningitis show early papilledema. Intracerebral abscess or expanding mass lesions, particularly in the posterior fossa, must be ruled out by physical examination, although in the presence of any focal signs an emergency computed tomography (CT) scan is necessary. In this situation an expert opinion should be requested if available. LP should be performed when any such masses are excluded because identification of the organism and determination of its sensitivities are essential; however, if a bacterial process is suspected, antibiotics should be started as soon as blood cultures are drawn and before an LP is done.

Anonymous Current p. 1252.

The Answer:  Since performing a LP in the presence of a space-occupying lesion (brain abscess, subdural hematoma, subdural abscess) can result in brain stem herniation and death, a CT scan is performed prior to LP if a space-occupying lesion is suspected on the basis of papilledema, coma, seizures, or focal neurologic findings.

Additional Info:  If there is a delay in obtaining a CT scan and bacterial meningitis is suspected, blood cultures should be drawn and antibiotics should be administered even before CSF is obtained for culture to avoid treatment delay.  Studies suggest that antibiotics given within 4 hours before obtaining CSF will not affect culture results.

Anonymous   Tininalli 1487

A cranial CT scan should be done first if the patient exhibits papilledema or focal neurological signs.  If the differential includes parenchymal brain infections and mass lesions, a LP is potentially dangerous since it can lead to transtentorial or tonsillar herniation.  A CT scan is the preferred mode in diagnosing subarachnoid hemorrhage.

(Noble 1552) Whenever there is evidence of meningeal inflammation, a lumber puncture must be done eventually, even though some patients with meningitis show early papilledema.  A head CT scan may need to be done if a intracerebral abscess or expanding mass lesion is suspected first and expert opinion sought.  LP should be performed when any such masses are excluded because identification of the organism and determination of its sensitivities are essential; however, if a bacterial process is suspected, antibiotics should be started as soon as blood cultures are drawn and before an LP is done.

Anonymous Tintinalli, pg. 1425

Noncontrast CT scan can usually exclude critical lesions or mass effects of tumors with increased intracranial pressure (ICP).  Contraindications to Lumbar Puncture (LP include the suspicion of raised ICP, which can be excluded by the absence of papilledema, normal level of consciousness, and normal findings on neurological exam.

Venous pulsations seen at the disc margins on fundoscopic exam with the patient upright effectively rule out ICP.  Venous pulsations may be terminated temporarily with pressure on the globe.  If these conditions are met, then a CT scan need not necessarily be carried out prior to LP, especially if the CT is likely to be delayed.

            5.      Since antibiotic choices change rapidly, you will not be ask ed to learn  specific recommendations for this.  Be aware that you may have to make empiric antibiotic choices based on patient's age, clinical circumstances, etc., before a  definitive bacterial pathogen can be identified.

Anonymous Current p. 1252, Table 30-1.

-18 to 50: cefotaxime or cephtriaxone (for S. pneumo, N. meningitides)

-Over 50: ampicillin, cephotaxime or cephtriaxone (for same as above and L. monocytogenes, gram negative bacilli)

-Impaired cellular immunity: ampicillin plus ceftazidime (for L. monocytogenes, gram negative bacilli, S. pneumo)

-Post surgical or posttrauma: vancomycin plus cephtazidime (for S. aureus, S. pneumo, gram negative bacilli)

            6.      Identify the need for prophylaxis of close contacts of patients with meningitis caused by Neisseria meningitidis and Hemophilus influenzae type b.

Suzy Noble 1555

Rifampin, is used for prophylaxis should be used for all household contacts and others closely associated with patients suffering from meningococcal disease (eg.,children in the same nursery school). Vaccination against meningococcal is also available and is an important public health measure.

Shauna Noble 1555

Prophylaxis should be used for all household contacts and others closely associated with patients suffering from meningococcal disease (eg.,children in the same nursery school). Rifampin is the recommended treatment for prophylaxis. Vaccination against meningococcal is also available and is an important public health measure.

Anonymous Current p. 1368.

Household members exposed to a person with meningococcal meningitis are at increased risk and should be given rifampin prophylaxis.  Day care center contacts are treated in same manner.  School and work contacts need NOT be treated.  Hospital contacts need NOT be treated unless intense exposure has occurred (mouth to mouth).

Anonymous  Noble 1555

• Prophylaxis should be used for all household contacts and others associated with patients suffering from meningococcal disease.

• Rifampin 600 mg bid for 2 days for adults, and 10 mg/Kg bid in children, and in infants 3 months to 1 year 5 mg/Kg bid.

• Prophylaxis treatment is also recommended in contacts of cases of H. influenzae; rifampin is recommended at a dosage of 20 mg/Kg/day (not to exceed 600 mg/day).

Anonymous Noble says  pp. 1555 :

Prophylaxis should be used for all household contacts and others closely associated with patients suffering from meningococcal disease (e.g., children in the same nursery school).  They list Rifampin as drug of choice.

            7.      Describe or recognize the typical clinical syndrome of acute aseptic (viral) meningitis, including CSF findings.

Suzy Noble 1555, CMDT 1253

Fever, headache, and meningeal signs. Change in consciousness and localizing neurologic signs are rare. Patients look and feel wretched but are not severely ill. CSF examination will usually show an increase in mononuclear cells to fever than 500/mm³ (although PMNs may be increased in the early stages); the protein is normal or only slightly elevated, and the CSF sugar is usually normal, except with mumps infection.

Shauna Noble 1555

Characterized by fever, headache, and meningeal signs. Change in consciousness and localizing neurologic signs are rare. Patients with this disease look and feel wretched but are not severely ill. CSF examination will usually show an increase in mononuclear cells to  500/mm³ ; the protein is normal or only slightly elevated, and the CSF sugar is usually normal, except with mumps infection.

Anonymous Current p. 1253, Table 30-2.

Typical CSF findings in aseptic meningitis (viral):

-25 to 2,000 cells, mostly lymphocytes

-Normal or low glucose

-High protein (>50)

-Slightly elevated opening pressure

Anonymous  Noble 1555

• Acute aseptic meningitis is characterized by fever, headache, and meningeal signs. Change in consciousness and localizing neurologic signs are rare.

• CSF findings will usually show an increase in mononuclear cells to fewer than 500/mm3 , the protein is normal or slightly increased, and the glues level is usually normal, except with mumps infection.

Anonymous  Noble chap 165

Characterized by fever, HA and meningeal signs (irritability, drowsiness, possibly a stiff neck, vomiting). A change in consciousness and localizing signs are rare. Patients look and feel wretched, but are not severely ill. CSF exam will usually show an increase in mononuclear cells to fewer than 500/mm³. PMN’s may be increased in the early stages. Protein is normal or only slightly elevated, and the CSF sugar is usually normal, except with a mumps infection.

            8.      Describe the usual clinical course and lack of neurological sequelae seen in most cases of acute viral meningitis.

Suzy Noble 1555

Requires only analgesics and reduction in fever; the prognosis here is much better than in bacterial meningitis - the pt. is seldom as ill and usually recovers readily. An attempt to identify the virus by culture of the CSF or by PCR. A chest x-ray, tuberculosis skin test, syphilis serology, blood cultures, and a collagen-vascular screen are commonly ordered. CT scan to rule out a parameningeal focus.

Shauna Noble 1555, CMDT 1253

Known causes are common viral infections (coxsackievirus, echovirus, Epstein-Barr, mumps and lymphocytic choriomeningitis). Change in consciousness and localizing neurologic signs are rare.  Management requires only analgesics and reduction in fever. Prognosis is much better than in bacterial meningitis. It’s much more benign and is self limiting

Anonymous Current p. 1253.

Aseptic (viral) meningitis is much more benign and self limited than purulent meningitis.

Noble p. 1555.

Treatment requires only analgesics and fever reduction.  The patient is rarely ill and usually recovers readily.  Aseptic meningitis is characterized by fever, headache and meningeal signs.  Change in consciousness and localizing neurologic signs are rare.

Anonymous

• Most common causes of aseptic meningitis are viral infections; coxsackievirus, echovirus, Epstein-Barr virus, mumps, and the LCM virus.

• Management usually requires only analgesics and reduction in fever. The prognosis is much better than bacterial meningitis, the patient is seldom ill and usually recovers readily.

Anonymous  Noble, pg. 1555

-70% of the known causes of aseptic meningitis are common viral infections (coxsackievirus, echovirus, Eptein-Barr virus, mumps, and lymphocytic choriomeningitis [LCM]).  In about 25% of the cases the virus is not defined.  The remaining long list of viruses identified includes measles, chickenpox, and influenza.  Rarely, the clinical picture of aseptic meningitis may be seen in the preicteric stage of infectious hepatitis or in uremia and in patients with inadequately treated bacterial meningitis.

-Management of acute septic meningitis requires only analgesics and reduction in fever; the prognosis here is much better than in bacterial meningitis – the patient is seldom as ill and usually recovers readily.

            9.      Identify tuberculosis and fungi as common causes of subacute and chronic infectious meningitis.

Suzy Noble 671, 1555

Inadequately treated bacterial meningitis, fungal infections, and tuberculosis meningitis (TBM) are the more common causes of subacute meningitis.

            TBM-most likely to occur in native and immigrant populations, among alcoholics, and those with immunologic deficiency or generalized illness.

Shauna Noble 1555

Inadequately treated bacterial meningitis, fungal infections, and tuberculosis meningitis (TBM) are the more common causes of subacute meningitis; viral causes are much less common.

Anonymous Current p. 1253

B. Chronic Meningitis.

-Patients with chronic meningitis present less acutely with a history of symptoms lasting weeks to months. 

-The most common pathogens are:

Mycobacterium tuberculosis

fungi (cryptococcus for example)

atypical mycobacteria

spirochetes

Anonymous

Inadequately treated bacterial meningitis, fungal infections, and tuberculosis meningitis are the most common cause of subacute meningitis

Anonymous  Noble Ch. 165 p.

Subacute meningitis from these causes is much more common than viral causes. They are most likely to occur in North American native, immigrant, alcoholic + immunocompromised populations. About 1/3 of cases happen prior to 10y/o. Half of cases are from known TBM. Illness usually occurs in the active stage of primary infection, but can present years later.

Chronic meningitis is uncommon but caused by the same organism as subacute meningitis + is characterized by

            10.    Identify cryptococcal meningitis as a common cause of CNS infection in AIDS patients.

Suzy Noble 292, 1556

Cryptococcal meningitis has an insidious onset, with evidence of a confusional state, focal signs, and raised ICP. Although a smoldering meningitis is the usual presentation, abscesses may form. This condition occurs in those with immunologic deficiencies, AIDS, diabetes, or chronic alcoholism.

Shauna Noble 1556

Cryptococcal meningitis also has an insidious onset, with evidence of a confusional state, focal signs, and raised ICP. This condition occurs in those with immunologic deficiencies, AIDS, diabetes, or chronic alcoholism.

Anonymous Current, p. 972, 1487.

-Cryptococcus is the most common cause of fungal meningitis (overall – not just in AIDS patients).

-Factors that predispose patients to cryptococcal infections (all cryp. infecs., not just cryp. mening.) include HIV infection.

-Cryptococcal meningitis is a common opportunistic infection in AIDS patients.

Anonymous  Noble p. 1556  True  objective explains itself

A clinical point of interest in cryptococcal meningitis is prolonged and severe nature of the headache that precedes cranial nerve palsies, meningism, or increased ICP. Diagnosis is made with India ink-stained CSF preparations, but cryptococcal antigen should be detected in the blood and CSF.

Anonymous  Noble 1556

Cryptoccal meningitis has an insidious onset with evidence of a confusional state, focal signs, and raised ICP.  Although a smoldering meningitis is the usual presentation, abscesses may form.  This condition also occurs in those with immunologic deficiencies, AIDs diabetes or chronic alcoholism.  Patients with AIDS need long-term suppressive treatment with fluconazole.  

            11.    Describe or recognize the typical clinical syndrome of acute viral encephalitis.

Aprilt Current p. 1254

Encephalitis due to herpes, arbo, rabies and flaviviruses ( West Nile encephalitis, Japanese encephalitis ) and many other viruses produce disturbances in sensorium, seizures, and many other manifestations.  Patients present with more acutely and are more ill thatn patients with aseptic meningitis.  Cerebrospinal fluid may be entirely normal or may show lyphocytes.

            Aprilt Noble 2nd ed. p. 1369

Consists of a febrile illness with headache, vomiting, photopobia, and rapid  evolution wover a day or two of progressive stupor, convulsions, and a combination of upper motor neuron, cerebellar, and brainstem derangements.  The  CSF profile is similar to that of acute viral (aseptic) meningitis: reveals a leukocyte count <1000 with a predominance of mononuclear cells and a protein level <100.  Sugar and gram stain are normal.

Anonymous Noble p. 1557.

-Acute viral encephalitis involves infection of the brain parenchyma.

-HSV, enterovirus and arbovirus are common causative agents.

-Clinical features: fever, meningism, increased ICP signs, decreased LOC, generalized or focal seizures, other focal signs (dependent upon brain region affected).

-Consider encephalitis if you see: fever plus meningism with signs of cerebral dysfunction (generalized = delerium, seizures, coma OR focal = hallucinations, hemiparesis).

Anonymous  Noble p. 1557-1558  

Infection of the brain parenchyma and is most commonly viral, if bacterial it is called cerebritis. In North America, HSV, enterovirus (polio, echovirus, and coxsackievirus), and arbovirus are the most causative agents. Typical clinical features of encephalitis include fever, meningism, and signs of raised ICP. Reduction in consciousness level from drowsy to coma, seizures that are often focal, and various other focal signs also occur, depending on the areas of the brain affected by the inflammatory process. The combination of fever and meningism with evidence of cerebral dysfunction, either generalized (delirium, seizures, coma) or focal (hallucinations, hemiparesis) must make one consider encephalitis.

Anonymous Noble, p. 1558

Typical features of encephalitis include fever, meningism and signs of raised ICP. Reduction in consciousness level from drowsiness to coma, seizures that are often focal and various other focal signs also occur depending on which areas of the brain are affected by inflammatory process. Combination of fever and meningism with evidence of cerebral dysfunction, either generalized (delirium, seizures, coma) or focal 9hallucinations, hemiparesis and so forth) must make one consider encephalitis.

            12.    Identify acute viral encephalitis as an important cause of substantial mortality and serious neurological morbidity in survivors.

Aprilt Noble 2^nd ed. p. 1368.

OK.  It also talks about how these viruses get there by bitten by a misquito carrying a virus and occurs in summer and early fall.

Anonymous -HSV encephalitis has the highest morbidity and mortality of the common viral encephalitides.  Untreated, mortality = 70% with <5% survivors having normal neurological function.

-ICP monitoring is needed in severe cases.

Anonymous True

HSV encephalitis has the highest morbidity and mortality of the common viral encephalitides (untreated mortality 70%; <5% of survivors have normal neurological function). ICP monitoring is needed in severe cases. Prognosis is based on infecting agent, though still poor.

Anonymous

The incidence of the various types of viral encephalitis varies from year to year, depending on whether sporadic or epidemic outbreaks occur.  In relative terms, the incidence is about one-tenth that of bacterial meningitis.  The yearly incidence ranges form 20 to 2000 reported cases (US).  It has a 20% mortality rate in the elderly.  Disease is most severe in the very young, causing seizures in 90% of affected infants and permanent neurologic deficits in 50%.  There is a breakdown of the prognosis for each of the specific viruses responsible for encephalitis on page 1489 of Tintinalli

            13.    Identify herpes simplex encephalitis as presenting with focal neurological signs, requiring differences in diagnosis (e.g., MRI, CT, or possible brain  biopsy) and treatment  (e.g., IV acyclovir) .

Aprilt Noble 2^nd ed. 1369

OK.  It also talks about how HSV-1 has a focal focal encephalitis of the inferiorfrontal and temporal lobes with severe necrosis and intraneuronal inclusion bodies.  It enters the brain from a peripheral tract.  Cowdry type A inclusion bodies are seen.

Anonymous Current p. 1305, D.

Encephalitis and Recurrent Meningitis.

-Herpes simplex encephalitis presents with nonspecific symptoms:

flu-like prodrome

headache, fever, behavior and speech disturbances

seizures (focal or generalized)

-Distinguishing feature: tendency for temporal lobe to be involved (resulting in mass effect on imaging studies and temporal lobe seizure foci on EEGs)

-CSF WBC pleocytosis is common

-Rapid, sensitive and specific tool for Early Diagnosis: HSV DNA PCR in the CSF.

-Untreated disease + coma = high mortality rate. (Those who survive often have neurological sequelae).

Noble p. 1559.

-CSF features: increased pressure, increased protein, lymphocytes + RBCs present.

-Characteristic EEG pattern (including slow temporal region activity).

-CT scan: swelling/enlargement of temporal lobes that is asymmetrical.

-MRI: shows CT scan findings but even better.

-Diagnosis via: PCR of the CSF, fluorescent antibody staining or culture of brain biopsy.

-LP: may be dangerous b/c mass effect of lesion.  CT or MRI should be done 1^st to r/o presence of a pressure cone.

-Acyclovir reduces morbidity and mortality if given early.

Anonymous True  Noble p. 1559  

The CSF shows an increase in pressure and protein levels; lymphocytes and RBC’s are often present. A characteristic EEG pattern (diffuse, mainly temporal region slow activity with periodic discharges) is described but not specific. The CT scan may demonstrate swelling and enlargement of the temporal lobes (usually asymmetrically), although MRI shows this even better. The specific methods of diagnosis are PCR of the CSF, fluorescent antibody staining, or culture of brain biopsy specimen. Because of the mass effect of the lesion, LP may be dangerous and CT or MRI should be done first to R/O the presence of a pressure cone. Acyclovir reduces mortality and morbidity if it is given early, 10mg/kg q 8hx10 days, reduces mortality by 20%; half of the patients recover completely.

Anonymous

Clinical features: May have an acute or subacute onset. Typically, a few days of malaise, fever, HA, anorexia, nausea, and other nonspecific symptoms progress to a subtle changes of personality, with evolving depression, paranoia, or abnormal behavior and confusion. Photophobia, signs of raised ICP, meningeal irritation, and focal signs appear next; the latter include hemiparesis, facial weakness, dysphasia, dysarthria, decerebrate rigidity, ocular palsies or nystagmus, seizures, and in the late stages stupor or coma.

Although the whole brain is involved, the temporal lobes are particular affected. The CT scan may demonstrate swelling and enlargement of the temporal lobes (usually asymmetrically), although MRI scans show this even better. The specific method of Dx is PCR of the CSF, fluorescent antibody staining, or culture of the brain biopsy specimen. B/c of the mass effect of the lesion, LP may be dangerous and CT and MRI should be done first to R/O the presence of a pressure cone.

Treatment: Acyclovir reduces mortality and morbidity if it is given early, and the low toxicity of this drug has led to its use empirically in most cases of acute, sporadic encephalitis. Acyclovir, 10 mg/kg given IV q 8 h for 10 days, reduces mortality to about 20%; half of the pts completely recover.

Ravel chptr. 19

Compare CT and MRI and brain scanning.

Ravel 298-299

Most of this material is already covered in the objectives for meningitis. What is most important is learning the different CSF pictures for bacterial meningitis vs viral meningitis vs TB and fungal meningitis. Also worth reviewing is material comparing CT w/ MRI and brian scanning on pp. 298-9

Radionucleotide advantages:            No xray contrast media needed, lower cost, ability to inspect major blood vessels - flow studies.

CT advantages: CT over brain scan in ability to visualize ventricles, better detection of brain tumor, better specificity in brain lesions(more reliable in posterior fossa lesions), better delineation of CNS anatomy over MRI. Lower cost and better results in early CVA/hemorrhage (significantly better in subacute subdural or epidural hemorrhage) and ability to detect calcifications and bone detail.

MRI advantages: No radiation, sometimes better in lesions detection (than scan or CT) better tissue detail, better visualization of spinal cord, sometimes more specific diagnosis. (More cost, slower, problems with Pts. with cardiac pacers, internal metal objects and clostrophobia).

*Ct and MRI are about equal in diagnosing subdural hematoma (MRI more expensive though).

Different CSF (cerebral spinal fluid) pictures for bacterial vs viral vs TB  vs fungal meningitis.  

Ravel 294-295

General CSF info: CSF should be clear/colorless. It will be pink-red with blood present. It will be white with WBC/protein present and yellow if they are present over 4 hours.

                                    Glucose           Protein            Lactic Acid     Highest WBC counts

Bacterial                     low                   *elevated          elevated            polymorphonuclear

                                                                                    along with         neutrophils

                                                                                    leukocytosis      predominate

Viral                                                                            no elevated       lymphocytes or

                                                                                    or mild elev.      mononucleocytes

TB                               low                                           elevated            lymphocytes

                                                                                                            predominate

Fungal                         low                                           elevated            sometimes leuks.

                                                                                                            predominate, some-

                                                                                                            times neutrophilic

* Protein reaches adult levels after 6 months. It is higher in newborns-6mths. Generally protein in CSF increases proportionally to leukocytosis in CSF. Notify lab of Pt. age - scale is different!

In early infection glucose may  be normal. In metastasis and subarachnoid hemorrhage there may also be a low glucose.

CSF Cultures:           

1-2 Months:    1) Group B strep    2) E. Coli    3) Listeria monocytogenes/other bacteria

3mths-6yrs:     1) H. Flu    2) Meningococcus    3) pneumococcus

peds/adolesc: 1) meningococcus    2) pneumococcus

adults:             1) meningococcus    2) (some) pneumococcus    3) staphylococci

                                                                                                    (assoc. with surgery)

elderly:                        1) pneumococci    2) meningococci    3) gram neg./others

*A negative gram stain does not r/o acute bacterial meningitis

** Albuminocytologic dissociation = Marked protein elevation without corresponding CSF cell    increase. (Assoc w/ Guillain-Barre syndrome and Giant cell arteritis).

*** The most important change in CSF glucose is a decrease

****CSF pressure varies directly w/venous pressure and has no relationship to arterial pressure. Two most common causes of CSF pressure elevation: meningitis and subarachnoid hemorrhage.

III.       Cerebrospinal Fluid Examination

1.            Be able to describe the typical cerebrospinal fluid findings in the normal patient and in a patient with:

a.       Community acquired bacterial meningitis

b.      Mycobacterial or fungal meningitis

c.       Spirochetal meningitis

d.      Aseptic or viral meningitis and meningoencephalitis

e.       Neighborhood reaction (brain abcess, vertebral osteomyelitis, epidural abcess, subdural empyema, bacterial sinusitis or mastoiditis)

TIM CMDT pg 1253

Normal: 0-5 WBC’s/uL, glucose 45-85 mg/dL, protein 15-45 mg/dL, opening pressure= 70-180 mm H20

a.       Community acquired bacterial meningitis

Typically has between 200-2000 polymorphonuclear neutrophils; the glucose concentration is < 45 (low) mg/dL; the protein usually > 50 mg/dL.  The opening pressure is markedly elevated.

                CMDT pg 1253

b.      Mycobacterial or fungal meningitis

Typically has between 100-1000 WBC’s, mostly lymphocytes.  Glucose levels are < 45 mg/dL (low), protein > 50 (high) mg/dL. The opening pressure is moderately elevated.

                CMDT pg 1253

c.       Spirochetal meningitis

Typically has between 100-1000 WBC’s, mostly lymphocytes. Glucose levels are normal. Protein levels are > 50 (high) mg/dL. The opening pressure is normal to slightly elevated.

                CMDT pg 1253

d.      Aseptic or viral meningitis and meningoencephalitis

Typically between 25-2000 WBC’s, mostly lymphocytes. Glocose levels are normal or low.  Protein levels are > 50 (high) mg/dL. The opening pressure is slightly elevated.

e.       Neighborhood reaction (brain abscess, vertebral osteomyelitis, epidural abscess, subdural empyema, bacterial sinusitis or mastoiditis)

WBC’s are variably increased. Glucose levels are normal. Protein levels are normal or high.  The opening pressure is variable.

Anonymous Current p. 1253, Table 30-2

Typical CSF findings in various CNS diseases.

-Normal:

0-5 lymphos, 45-85 mg/dL glucose, 15-45 mg/dL protein, 70-80mm opening pressure.

-Purulent meningitis (bacterial) community acquired:

200-20,000 PMNs, low glucose (<45), high protein (>50), high opening pressure.

-Granulomatous meningitis (mycobacterial, fungal):

100-1,000 (mostly) lymphos, low glucose (<45), high protein (>50), semi high pressure.

-Spirochetal meningitis:

100-1,000 (mostly) lymphos, normal glucose, high protein (>50), maybe normal pressure.

-Aseptic meningitis, viral or meningoencephalitis:

25-2,000 (mostly) lymphos, normal or low glucose, high protein, slightly high pressure.

-Neighborhood reaction:

variably increased cells, normal glucose, normal or high protein, variable pressure.

Additional info:

-CSF glucose must be considered in relation to blood glucose level.  Normally, CSF glucose is 20-30 mg/dL lower than blood glucose (30-50% lower).

-In granulomatous, spirochetal and aseptic meningitis cases, PMNs may predominate (vs. lymphocytes found later) early on in the disease course.

-Neighborhood reaction may occur in mastoiditis, brain abscess, epidural abscess, sinusitis, septic thrombus, brain tumor.  CSF culture results usually negative.

IV.       Seizure Disorders

            1.      Distinguish between the following major categories of seizures.

                              partial seizures:

                                    simple partial seizures (focal)

                                    complex partial seizures (aka: psychomotor or temporal epilepsy)

                              generalized seizures:

                                    absence, or petit mal

                                    tonic-clonic, or grand mal

                                    status epilepticus

Kim R. CMDT pg 953

PARTIAL:

1. Simple partial (focal)- may be manifested by focal motor symptoms(convulsive jerking) or somatosensory symptoms(paresthesias or tingling) that spread to different parts of the limb or body depending upon their cortical representation.  In other instances, special sensory symptoms (light flashes or buzzing) indicate involvement of visual, auditory, olfactory, or gustatory regions of the brain, or there may be autonomic symptoms of signs(abnormal epigastric sensations, sweating, flushing, pupil dilation). 

2. Complex partial- impaired consciousness may be preceded, accompanied, or followed by the psychic symptoms mentioned above, and automatisms may occur.  Such seizures may also begin with some of the simple symptoms mentioned above.

GENERALIZED:

1. absence, or petit-mal- characterized by impairement of consciousness, sometimes with mild clonic, tonic, or atonic components(reduction or loss of postural tone), autonomic components(enuresis), or accompanying automatisms.  Onset and termination of attacks are abrupt.  If attack occur during conversation, the person may miss a few words or may break off in mid sentence for a few seconds.  The attack is so brief that the person may not be aware of it.  These type of seizures almost always begin in childhood and frequently cease by the age of 20, although occasionally they are replaced by other forms of generalized seizure.

2. Tonic-clonic, or grand mal- characterized by sudden loss of consciousness, the patient becomes rigid and falls to the ground, and respiration is arrested.  This tonic phase, which usually lasts for less than a minute, is followed by a clonic phase in which there is jerking of the body that may last for 2 to 3 minutes and is then followed by a stage of flaccid coma.  During the seizure the patient may bite his/her lip, tongue, loose bowel and bladder control.  Immediately after the seizure, the patient may either recover consciousness, drift into sleep, have further convulsion without recovery of consciousness between attacks (status epilepticus), or after recovering consciousness have a further convulsion (serial seizures).  In other cases, patients will behave in an abnormal fashion in the immediate postictal period, without subsequent awareness or memory of events (postepileptic automatism).  Headache, disorientation, confusion, drowsiness, nausea, soreness of muscles, or some combination of these symptoms commonly occurs postictally.

3. status epilepticus- convulsions without recovery of consciousness between attacks.

Anonymous Current p. 953.

-Partial seizures:

simple partial (focal) & Complex Partial (psychomotor/temporal epilepsy)

-Generalized seizures:

absence (petit mal) & tonic-clonic (grand mal) & status epilepticus

Simple Partial Seizures (Focal Seizures):

-Manifested by:            focal motor symptoms (convulsive jerks)

                                    somatosensory symptoms (paresthesias/tingling) that spread

                                    special sensory symptoms (light flashes, buzzing, etc.)

                                    autonomic symptoms (flush, sweat, pupils dilate, epigastric sxs)

                                    dysphagia

                                    dysmnesic symptoms (déjà vu)

                                    illusions

                                    affective disturbance

                                    hallucinations

(This is a list of potential manifestations; not all S/Sxs occur.)

-Complex Partial Seizures (Psychomotor or Temporal Epilepsy):

May be manifested by:  S/Sxs listed above.  Theses S/Sxs are generally accompanied, preceded or followed by impaired consciousness.  Automatisms may also occur.

-Absence Seizures (Petit Mal Seizures):

May be manifested by: impairment of consciousness

                                                mild clonic, tonic or atonic components (postural tone loss)

                                                autonomic components (enuresis)

                                                automatisms

                                                abrupt onset & termination of attacks

                                                breaking off in mid sentence for seconds if px is speaking

                                                very brief impairment of awareness

                                                patient is often unaware seizure occurred

                                                almost always begin in childhood

                                                seizures often stop by age 20

-Tonic-Clonic Seizures (Grand Mal Seizures):

May be manifested by:  sudden loss of consciousness

                                                patient becomes rigid and falls to ground

                                                respiration stops

                                                tongue or lip bites

                                                urinary or fecal incontinence

                                                patient injury

                                                tonic phase is 1^st and lasts < 1 minute

                                                clonic phase (jerking) is 2^nd and lasts up to 2-3 minutes  a stage of flaccid coma often follows the seizure

Immediately after the tonic-clonic seizure, the patient may recover consciousness, drift into sleep OR have a further convulsion without recovery of consciousness between attacks (STATUS EPILEPTICUS).  Headache, disorientation, confusion, drowsiness, nausea, soreness of muscles can all occur postictally.

Additional Info:

-Partial Seizures – only a restricted part of one cerebral hemisphere is activated with this seizure.  Partial seizures are subdivided into simple seizures, in which consciousnessis preserved, and complex seizures, in which it is impaired.

-Generalized Seizures – nothing special to say about these.  Generalized seizures include absence seizures, tonic-clonic seizures, status epilepticus, and others.

Anonymous  Noble 1485-1486

•        partial seizures (begin locally):

o simple partial seizures (focal) – without impairment of consciousness; diverse manifestation determined by the region of cortex activated by the seizure (may progress from thumb → hand → arm → shoulder → girdle → trunk → entire body.   They typically last 20-60 seconds

o complex partial seizures (aka: psychomotor or temporal epilepsy) – with impairment of consciousness lasting 30 sec to 2 minutes; often associated with purposeless movements such as lip smacking or hand wringing; confusion, amnesia, full coordination (i.e. dressing, undressing), and aura

o partial with secondarily generalized tonic-clonic seizure – when a simple or complex partial seizure evolves into a tonic-clonic seizure with loss of consciousness and sustained contractions (tonic) of muscles throughout the body, followed by periods of muscle contraction alternating with periods of relaxation (clonic), typically lasting 1-2 minutes; may be associated with ↑ heart rate, ↑ BP, and loss of bowel/bladder control.

•        generalized seizures (bilaterally symmetrical and without local onset):

o absence (aka petit mal) – abrupt onset of impaired consciousness with staring and cessation of ongoing activities typically lasting less than 30 seconds; common in children, usually disappears after adolescence.

o tonic-clonic (aka grand mal) – NOT preceded by a partial seizure; loss of consciousness and sustained contractions (tonic) of muscles throughout the body, followed by periods of muscle contraction alternating with periods of relaxation (clonic), typically lasting 1-2 minutes; may be associated with ↑ heart rate, ↑ BP, and loss of bowel/bladder control.  A vocal “cry” or “scream” sometimes occurs as the result of air being expelled through the contracted vocal cords.

o status epilepticus (prolonged partial or generalized seizures without recovery from attacks) – life-threatening emergency requiring immediate medical management along with anti-epileptic drugs; seizure episodes last > 30 minutes or intermittently > 30 minutes without a regain in consciousness

o febrile seizure – seizure in healthy infants and children (6mo – 5yr) associated with a fever > 38.0 C (rectal) without evidence of intracranial infection or defined cause

Anonymous

Partial seizures:  begin in one area of the brain and initially produce symptoms that are referable to the region of cortex involved.

Simple partial seizures (focal): focal neurologic events in which consciousness remains intact.

Complex partial seizures (aka: psychomotor or temporal epilepsy):  consciousness is impaired.

Generalized seizures: bilaterally symmetric and without focal onset.  An episode may begin with a premonitory aura that is followed by a sudden loss of consciousness.  A tonic phase of limb extension ensues, lasting 10 to 30 seconds, followed by a clonic phase of limb jerking of at least 30 seconds.  The patient then becomes flaccid and comatose before regaining consciousness.  Postictal confusion is characteristic and can last for hours, although 10 to 30 minutes is more typical.  Tongue biting and incontinence are other characteristic features.

Absence, or petit mal:  very brief, generally lasting only a few seconds.  The patient suddenly loses consciousness without losing postural tone. They appear confused, detached, or withdrawn, and current activity ceases.  They may stare and have twitching of their eyelids.  They do not respond to voice or to other stimulation, exhibit voluntary movements, or lose continence.  The attack ceases abruptly, and the patients are able to resume their previous activity with no postictal symptoms.  Both the patients and witnesses may be unaware that anything has happened.  Classic absence seizures are limited to school-aged children and are often attributed by parents and teachers to daydreaming or not paying attention.  The attacks may be very frequent, sometimes occurring 100 or more times daily, and may result in poor school performance.  They usually resolve as the child matures.  (Tintinalli 1463)

Tonic-clonic, or grand mal:  they begin with abrupt loss of consciousness; there is usually no warning or aura.  In a typical attack, the patient suddenly becomes rigid, trunk and extremities are extended, and the patient falls to the ground.  Patients are often apneic during this period and may be deeply cyanotic.  

Status epilepticus: defined as either continuous seizure activity for 30 minutes or more, or two or more seizures that occur without full recovery of consciousness between the attacks.

            2.      Identify the main elements of the history, physical examination, and diagnostic tests which are obtained in working up a patient with seizure disorder.

Kim R. CMDT pg 954

HX: nonspecific changes such as HA, mood alterations, lethargy, and myoclonic jerking.  A sense of an aura may precede an attack.

PE: the presence of lateralized or focal signs postically suggests that seizures may have a focal origin.  In pts with symptomatic epilepsy, the findings on exam will reflect the underlying cause.

TESTS: MRI is indicated for pts with focal neurologic symptoms of signs, focal seizures, or EEG findings of focal disturbances; some clinicians order imaging studies for all patients with new-onset seizure disorder.  EEG supports the clinical DX of epilepsy. LABS: CBC, blood glucose, liver and renal function tests, and serologic testing for syphilis. 

Anonymous Current p. 954.

-History:  Headache, mood alterations, lethargy, myoclonic jerking can occur hours before seizures.  Auras can occur minutes or seconds before seizures.  Sometimes seizures occur at specific times (like during sleep).  Sometimes seizures occur in relation to external precipitants (like lack of sleep, missed meals, stress, menstruation, alcohol consumption or withdrawal, drug use).  Fever and infections can precipitate seizures in known epileptics, as can flashing lights, flickering tvs, music and reading.

-Physical Exam:  Clinical exam between seizures shows no abnormality in patients with idiopathic epilepsy, but in the immediate postictal period, exstensor plantar responses may be seen.  The presence of lateralized or focal signs postictally suggests that seizures may have a focal origin.  In patients with symptomatic epilepsy, the findings on exam will reflect the underlying cause.

-Diagnostic Tests:

~MRI for patients with ANY focal S/Sx.  Order imaging studies for all patients with new onset seizure disorders, especially if the disorder is progressive or if patients are over 20 years of age.  Also get a CXR in such patients (lungs are common site for primary and secondary neoplasms).

~Full blood count

~Blood glucose

~Liver and renal function tests

~Serologic tests for syphilis

~EEG

Anonymous  Noble 1488, Goroll 964-965

• History – essential to gain exact description of events from both patient and a witness

o       Questions:  What happened just before you lost consciousness?  Do you ever have a warning or a feeling that you are going to have a seizure?  Do you ever think you are going to have a seizure and it doesn’t occur?  Were you injured during the convulsion?  Did you lose control of your bladder or bowels?

o       History of head injury and an estimate of its severity, previous  intracranial sepsis (meningitis or encephalitis), birth and developmental milestones, and a family history of seizures or other neurological disorders

o       Other areas include drugs (etoh, cocaine, meth, antidepressants, sedatives, theophyllin, insulin, diuretics), cardiac arrhythmias, valvular disease, previous malignancy, and stroke

o       History of febrile seizures in childhood should be distinguished from the pt’s habitual seizure disorder

o       Pt’s state after the ictus – how long before they return to awareness, is there any limb paralysis or dysphasia?

• PE:

o       Evaluate for signs of hypo/hyperglycemia, meningeal irritation (stiff neck, HA)

o       Neurological exam

o       Check for postural hypotension, abnormalities in heart rate and rhythm, head trauma, carotid disease, cardiac disease, systemic infection, and signs of alcohol or drug abuse

• Diagnostic tests:

o       Electroencephalography (EEG) – abnormal EEG with epileptiform features such as spikes or sharp waves supports the dx of seizures and may provide information about the type of seizure disorder; there are limitations in that an abnormal pattern is not adequate for diagnosis, and a normal EEG can be found in up to 20% of patients with seizures

o       Neuroimaging:

•   MRI (with/without contrast) – more sensitive than CT, may be more useful in demonstrating abnormalities in the medial temporal region

•   CT scan – may be sufficient in pts who have had a history of brain insult that could explain the etiology of the seizure disorder

•   Positron emission tomography (PET) and single-photon emission computed tomography (SPECT) – may confirm the presence of an organic abnormality and provide an outline of the abnormal region for which surgical treatment might be considered

o       Other testing:

•   Blood chemistries, electrolytes, calcium, etoh, and toxic screens are important

•   All pts with risk factors for HIV should be tested

•   Lumbar puncture if evidence of infection is found (this is not routine for first seizure)

Anonymous   Tintinalli p. 1464-66

History:  Important avenues of inquiry include preceding aura, abrupt or gradual onset, progression of motor activity, loss of bowel or bladder control, and whether the activity was local or generalized, symmetrical or not.  The duration of the attack and any postictal confusion or lethargy should be sought.  The patient should be asked whether he or she has any recollection of the attack.  If the patient is a known epileptic, the baseline seizure pattern should be established.  If there is no previous history of seizures, a more detailed history is needed. Symptoms that might suggest previous unwitnessed or unrecognized seizures, such as blank or staring spells in school, involuntary movements, unexplained injuries, nocturnal tongue biting, and enuresis, may be clues to a more long-standing problem.  A history of recent or remote head injury should be sought.  Concurrent pregnancy or recent delivery suggests the possibility of eclampsia.  A history of metabolic derangements or electrolyte abnormalities, hypoxia, systemic illness (esp. CA), coagulopathy or anticoagulation, drug ingestion or withdrawal and alcohol use may help identify factors that predispose patients to seizures.

PE:  The PE should be directed toward discovering any injuries, especially head or spine that might have resulted from the seizure.  Fractures, sprains, bruises and posterior dislocations of the shoulder are common and may be overlooked.  Tongue lacerations and aspiration are frequent sequelae.  A search for any systemic illness that may have caused the attack should be undertaken.  Temperature should be noted.

Diagnostic Tests:  With the patient who has a history of seizure disorder, the only test that may be needed is an anticonvulsant level.  In the case of a patient with a first seizure or when the history is unclear:

Glucose

Electrolytes

BUN

Creatinine

Calcium

Magnesium

Pregnancy test

Toxicology screen

UA (if hemoglobin positive, but no red cells, then a CPK should be done to rule out rhabdomyolysis.)

            3.      Identify each of the following as a common cause of acute or recurrent seizure disorder.

                              idiopathic epilepsy

                              metabolic and toxic encephalopathies

                              posttraumatic epilepsy (especially penetrating head injuries)

                              CNS neoplasms

                              cerebrovascular disease

                              intracranial infection

Kim R. CMDT pg 953

1.   Idiopathic epilepsy- seizures usually begin between 5 and 20 years of age, but may start later in life.  No specific cause is known and there is no other neurologic abnormality.

2. Metabolic and toxic enephalopathies-withdrawl from alcohol or drugs is a common cause of recurrent seizures, and other metabolic disorders such as uremia and hypoglycemia or hyperglycemia may also be responsible.

3. posttraumatic epilepsy- trauma is an important cause of seizures at any age, but especially in young adults.  Posttraumatic epilepsy is more likely to develop if the dura mater was penetrated and generally becomes manifest within 2 years following injury. 

4. CNS neoplasms-these may lead to seizures at any age, but they are an especially important cause of seizures in middle and later life, when the incidence of neoplastic disease increases.  The seizures are commonly the initial symptoms of the tumor and often are partial(focal) in character.  They are most likely to occur with structural lesions involving the frontal, parietal, or temporal regions.  Tumors must be excluded by appropriate imaging studies in all pts with onset of seizures after 30 years of age, focal seizures or signs, or a progressive seizure disorder.

5. cerebrovascular disease- is becoming an increasingly frequent cause of seizures with advancing age and are the most common cause of seizures with onset at age 60 years or older.

6. intracranial infection- infectious disease must be considered in all age groups as potentially reversible causes of seizures.  Seizures may occur with an acute infective or inflammatory illness, such as bacterial meningitis or herpes encephalitis, or in pts with more long-standing or chronic disorders such as neurosyphilis or cerebral cysticerosis.  In pts with AIDS, they may result from CNS toxoplasmosis, crytococcal meningitis, secondary viral encephalitis, or other infective complications.  Seizures are a common sequela of supratentorial brain abscess, developing most frequently in the first year after treatment.

Anonymous Current p. 953.

-Idiopathic Epilepsy:

aka Constitutional Epilepsy

seizures usually begin between 5 and 20 years of age 

no identifiable cause

no neurologic abnormality

-Metabolic and Toxic Encephalopathies:

a type of symptomatic epilepsy (vs. idiopathic epilepsy)

commonly caused by drug or alcohol withdrawal

commonly caused by metabolic disorders like uremia, hypoglycemia, hyperglycemia      

-Posttraumatic Epilepsy (especially penetrating head injuries):

especially seen in young adults

this epilepsy is more likely to develop if the dura mater is penetrated

manifestation can occur up to 2 years post injury

seizures occurring immediately after injury do not imply that future attacks are a given

-CNS Neoplasms:

neoplasms can lead to seizure at any age

neoplasms are more likely in middle and later life

if middle/later life pxs present w/ seizures r/o neoplasm-common things occur commonly

these seizures are often initial symptom of the tumor

these seizures are often partial (focal)

*Tumors must be excluded by appropriate imaging studies in all patients with onset of seizures after 30 years of age, focal seizures or signs, or a progressive seizure disorder.

-Cerebrovascular Disease:

vascular diseases become increasingly frequent cause of seizures with advancing age

for seizure onset at age 60 or above, vascular disease is most likely cause

-Intracranial Infection:

infection must be considered in all age groups presenting with seizures

infections are a potentially reversible cause of seizures (so they’re a Do Not Miss)

possible acute causes – bacterial meningitis, HSV encephalitis

possible chronic causes – neurosyphilis, cerebral cysticerosis

possible AIDS related causes – toxo, crypto

-Degenerative Disorders (I added this one to the list b/c it’s in the book):

Alzheimer’s disease and other degenerative disorders are a cause of seizures in later life.

Anonymous  Goroll 963-4

• Primary or idiopathic epilepsy – most common cause of recurrent seizures in children

o       In the young adult (18-45) population, the demonstrated causes include drugs, neoplasm, and trauma

o       In the older adult population , underlying pathology is equally divided among neoplasm, trauma, and CV disease

• Metabolic and toxic encephalopathies – A nonepileptic convulsion may occur in the context of a transient metabolic disturbance, such as cerebral hypoperfusion, hypoglycemia, a hyperosmolar state, or hyponatremia

• Posttraumatic epilepsy – higher incidence with penetrating head injuries and severe trauma causing a loss of consciousness for more than a half hour; also if there was a lobar hematoma or depressed skull fracture

o       With a history of closed head injury, epilepsy usually develops within 2 years, whereas with open head trauma, seizures may develop at a longer interval after the original injury

• CNS neoplasms

• cerebrovascular disease

• intracranial infection

Anonymous

Idiopathic epilepsy -  most common cause of recurrent seizures in children, but increasingly rare in the young adult population.

Metabolic and toxic encephalopathies - common cause of seizures in young adult and older adult populations. Metabolic more common in older adults. Toxic includes alcohol (common cause in young adults), prescription drugs, street drugs, and ingestion of toxic substances.

Posttraumatic epilepsy (especially penetrating head injuries) - common cause of seizures in the young adult population.

CNS neoplasm’s -  common cause of seizures in young adult population and accounts for ~1/3 of seizures in older adult population.

Cerebrovascular disease - Common cause of seizures in older adult population (~1/3 of cases).

Intracranial infection -  Less common cause of seizures, more common in pediatric population.

Goroll, p. 964, Noble, p. 1485

            4.      Read the material about treatment of seizure disorders, but  pharmacology questions will be deferred to the Patient Management  course.

Anonymous Current p. 955.

V.        Multiple Sclerosis

            1.      Identify multiple sclerosis (MS) as the most common chronic neurologic disease of young adults.

Stephen CMDT pg 983

This common neurologic disorder, which probably has an autoimmune basis, has its greatest incidence in young adults.

Anonymous Current p. 983.

This common neurologic disorder, which probably has an autoimmune basis, has its greatest incidence in young adults.

Anonymous  Noble p. 1623

MS rarely occurs in children or the elderly; most pts develop their first Sx between the ages of 20 and 40 (30 is average age of onset). There is increasing prevalence at latitudes farthest from the equator – therefore more common in Washington. About 20% of pts have a FH of this disorder, which is strong evidence for genetic influences in MS.

Anonymous  Noble p. 1623

MS rarely occurs in children or the elderly; most pts develop their first Sx between the ages of 20 and 40 (30 is ave. age of onset). There is increasing prevalence at latitudes farthest from the equator – therefore more common in Washington. About 20% of pts have a FH of this disorder, which is strong evidence for genetic influences in MS.

            2.      Recognize that the most popular current theory of MS is that it is an autoimmune, inflammatory demyelinating disorder of the CNS resulting in the delay, dispersion, and blockage of electrical impulses.

Stephen

OK!!

Anonymous Current p. 983.

Pathologically, focal areas of demyelination with reactive gliosis are found scattered in the white matter of brain and spinal cord and in the optic nerves.

Noble p. 1623.

MS is a recurrent progressive disorder whose signs and symptoms are due to plaques of demyelination in the white matter of the brain.  Changes in the blood-brain barrier initiate new lesions and axonal damage is likely responsible for progression.  It is an autoimmune, inflammatory, demyelinating disorder resulting in the delay, dispersion and blockage of electrical impulses.

Anonymous  Noble 1623

Recurrent, progressive disorder whose signs and symptoms are due to plaques of demyelination in the white matter of the brain.  Changes in the blood-brain barrier initiate new lesions, and axonal damage is likely responsible for progression.  It is an autoimmune, inflammatory, demyelinating disorder resulting in the delay, dispersion and blockage of electrical impulses.

Anonymous Noble 1623

Recurrent, progressive disorder whose signs and symptoms are due to plaques of demyelination in the white matter of the brain.  Changes in the blood-brain barrier initiate new lesions, and axonal damage is likely responsible for progression.  It is an autoimmune, inflammatory, demyelinating disorder resulting in the delay, dispersion and blockage of electrical impulses.

            3.      Describe various clinical manifestations of MS, especially the following:

                              visual disturbances: optic neuritis, nystagmus, diplopia,

                              paresthesias/dysesthesias,

                              spasticity, limb weakness, and ataxia,

                              urinary urgency, frequency, or incontinence,

                              disorders of mental function.

Stephen CMDT pg 985

Visual disturbances

Optic atrophy, nystagmus, and diplopia

Parathesias/dysesthesias

Altered sensations, weakness, nunbness, and tingling.

Spasticity Conn’s Current Therapy pg 901

Spasticity results from interruption of inhibitory influiences on anterior horn cells that can occur with lesions of the brain, spinal cord, and brain stem. It is associated with increased muscle tone, weakness, hyperreflexia, extensor plantar responses, and spontaneous muscle spasms.

Disturbances of balance, gait or coordination.

Urinary urgency , frequency, or incontinence CMDT pg 983 result from sphincter disturbance.

Conn’s pg 902 Urinary freq., urgency, and subsequent urge incontinence usually result from a spastic detrusor muscle. Urinary retention and hesitation result from sphincter dyssynergia, with incomplete relaxation of internal and external sphincters during detrusor contraction.

Disorders of mental function Conn’s pg 901

Affective disorders and anxiety disorders are common in MS, and pts with MS are at increased risk for suicide.

Pts with MS can also experience inappropriate laughter or crying as a result of bilateral disruption of subcortical pathways.

Anonymous Goroll 975.

-Visual Disturbances: Optic Neuritis, Nystagmus, Diplopia

~Optic neuritis is characterized by unilateral vision loss that develops suddenly.  Optic neuritis is particularly associated with demyelinating disease, occurring in patients known to have MS and as a 1^st manifestation of the disease in others.  In patients with clinically isolated optic neuritis, as many as 75% will have developed clinically definite MS within 15 years.  (Current p. 166)

~Nystagmus in the abducting eye and a failure of adduction are often noted in MS patients.

~Diplopia resulting from internuclear ophthalmoplegia or an oculomotor deficit is another common symptom heralding MS.  Bilateral internuclear ophthalmoplegia is very characteristic of MS and strongly suggests the diagnosis.

-Paresthesias/Dyesthesias – see below (paraparesis is only thing I found).

-Spasticity, Limb Weakness & Ataxia – The common initial presentation of MS is usually weakness, numbness, tingling or unsteadiness in a limb; spastic paraparesis; retrobulbar neuritis; diplopia; disequilibrium; or a sphincter disturbance such as urinary urgency or hesitancy.  Symptoms may disappear after a few days or weeks but examination often reveals a residual deficit.  (Current p. 983)

-More on Ataxia – Ataxia and intention tremor are manifestations of cerebellar involvement.  Re: motor deficits, legs are more likely to be involved than arms, initially asymmetrically.

-Urinary Urgency, Frequency or Incontinence – see above (sphincter disturbance…).  Urinary problems are consequences of upper motor nerve injury in the spinal cord.

-Disorders of Mental Function – Later in the course of illness, cerebral involvement may produce memory loss, personality change and emotional lability.  More than 60% of MS patients demonstrate abnormalities on formal neuropsychiatric testing, even if asymptomatic.

Anonymous  Goroll, pp. 975-976

Clinical Presentation is a function of the site of the inflammatory process. Attacks are by definition those that produce symptoms that last more than 24 hours. They average up to one per year and decrease in frequency over time. Transient sensory deficits are the most common initial presentation, affecting about 40% to 50% of patients.

Paresthesias or diminution of sensation in the upper or lower extremities. The sensory disturbance may be bilateral and symmetric, extending to involve the adjacent trunk. About 15% to 20% experience acute monocular visual loss because of optic neuritis. A central scotoma, transient pain on eye movement, and decreased pupillary reaction to light (Marcus-Gunn pupil) are characteristic features.

Diplopia resulting from internuclear ophthalmoplegia or an oculomotor defect is another common symptom heralding MS. Bilateral internuclear ophthalmoplegia is very characteristic of MS and strongly suggests the diagnosis. A failure of adduction and coarse nystagmus in the abducting eye are noted. Other oculomotor functions remain intact.

Ataxia and intention tremor are manifestations of cerebellar involvement. Motor deficits may occur acutely or insidiously, with the insidious variety particularly common in older patients. Legs are more likely to be involved than arms, initially asymmetrically. However, upturned toes are common bilaterally, even in patients with unilateral problems.

Urinary difficulties (frequency, urgency, incontinence) are consequences of upper motor nerve injury in the spinal cord. The external sphincter fails to relax adequately, causing incomplete emptying. Such autonomic injury may also produce constipation and impotence.

Later in the course of illness, cerebral involvement may produce memory loss, personality change, and emotional lability. More than 60% of MS patients demonstrate abnormalities on formal neuropsychiatric testing, even if asymptomatic. Paroxysmal symptoms may result from dysfunction of partially demyelinated axons and simulate a transient ischemic attack or focal seizure, or produce an attack of tic douloureux.  Fatigue may be prominent and even predate exacerbations

Anonymous

Visual disturbances: optic neuritis, nystagmus, diplopia, paresthesias/ dysesthesias, spasticity, limb weakness, and ataxia, urinary urgency, frequency, or incontinence, disorders of mental function.  Goroll, pp. 975-976.

Clinical Presentation is a function of the site of the inflammatory process. Attacks are by definition those that produce symptoms that last more than 24 hours. They average up to one per year and decrease in frequency over time. Transient sensory deficits are the most common initial presentation, affecting about 40% to 50% of patients.

Paresthesias or diminution of sensation in the upper or lower extremities. The sensory disturbance may be bilateral and symmetric, extending to involve the adjacent trunk. About 15% to 20% experience acute monocular visual loss because of optic neuritis. A central scotoma, transient pain on eye movement, and decreased pupillary reaction to light (Marcus-Gunn pupil) are characteristic features.

Diplopia resulting from internuclear ophthalmoplegia or an oculomotor defect is another common symptom heralding MS. Bilateral internuclear ophthalmoplegia is very characteristic of MS and strongly suggests the diagnosis. A failure of adduction and coarse nystagmus in the abducting eye are noted. Other oculomotor functions remain intact.

Ataxia and intention tremor are manifestations of cerebellar involvement. Motor deficits may occur acutely or insidiously, with the insidious variety particularly common in older patients. Legs are more likely to be involved than arms, initially asymmetrically. However, upturned toes are common bilaterally, even in patients with unilateral problems.

Urinary difficulties (frequency, urgency, incontinence) are consequences of upper motor nerve injury in the spinal cord. The external sphincter fails to relax adequately, causing incomplete emptying. Such autonomic injury may also produce constipation and impotence.

Later in the course of illness, cerebral involvement may produce memory loss, personality change, and emotional lability. More than 60% of MS patients demonstrate abnormalities on formal neuropsychiatric testing, even if asymptomatic. Paroxysmal symptoms may result from dysfunction of partially demyelinated axons and simulate a transient ischemic attack or focal seizure, or produce an attack of tic douloureux.  Fatigue may be prominent and even predate exacerbations

            4.      Recognize that the natural history of MS is very variable; identify factors which may precipitate symptoms or attacks.

Stephen CMDT pg 983

A number of factors (eg, infection, trauma) may precipitate or trigger exacerbations.

Relapses are also more likely during the 2 or 3 months following pregnancy, possibly because of the increased demands and stressors that occur in the postpartum period.

Anonymous Current p. 983.

A number of factors may precipitate or trigger exacerbations (infection, trauma, etc.).  Relapses are also more likely during the 2 or 3 months following pregnancy, possibly b/c of the increased demands and stresses that occur in the postpartum period.

Noble Chapter 171.

Factors that may precipitate symptoms or attacks:

Acute infections, postpartum, hot baths, exercise, hot humid weather, physical and emotional stress.

Anonymous    Noble Ch. 171

Factors that may precipitate symptoms or attacks are:

• -Acute infections

• -6 mo. postpartum w/o pregnancy

• -Hot baths

• -Exercise

• -Hot humid weather

• -Physical/emotional stress

Anonymous Noble Ch. 171

Factors that may precipitate symptoms or attacks are:

-Acute infections

-6 mo. postpartum w/o pregnancy

-Hot baths

-Exercise

-Hot humid weather

-Physical/emotional stress

            5.      Identify diagnostic tests which help confirm the diagnosis of MS.

Stephen CMDT

MS should not be diagnosed unless there is evidence that two or more different regions of the central white matter have been affected at different times. The diagmosis is probable in pts with multifocal white matter disease, but only on clinical attack, or with a histoty of at least two clinical attacks but signs of only a single lesion.

MRI of the brain or cervical cord is often helpful in demonstrating the presence of a multiplicity of lesions. CT scans are less helpful.

Anonymous Conn’s 897 MRI is the most sensitive measure of disease activity in MS.

Goroll p. 976, Noble p. 1625.

-MRI is the most sensitive diagnostic testing tool for MS.  More than 90% of MS patients have multiple periventricular plaques (white matter lesions).

-CSF reveals abnormalities in 95% of MS patients.

Anonymous  Goroll 976

•      Along with the development of signs and symptoms of MS there are some diagnostic tests that can aid

         in diagnosing this disease.

•      Lab studies may help support the diagnosis, but no single lab test is diagnostic and clinical evidence is

               necessary for a diagnosis.

•      MRI is the most sensitive test.  More than 90%  of MS patients have multiple periventricular plaques

   (white matter lesions), presenting as areas of increased signal intensity on long TR-weighted

   and proton density-weighted images.  The problem is that these plaques also appear in normal

   aging, chronic uncontrolled HTN, advanced lyme disease and CNS vasculitis.

•      The CSF reveals abnormalities in 95% of MS patients including the following:

•      Moderate increase in cell count and protein are common but not specific.

•      Increase in IgG and oligoclonal IgG bands on electrophoresis are more specific

•      (Noble pg 1625 says only 30% of MS patients have increase cell counts and mostly lymphocytes)

•      Evidence of MS may also be supported by visual or auditory evoked potentials being abnormal in

demyelinated tracts. (impulses are slower in remyelinated areas)

            6.      Describe the role of corticosteroids and beta-interferon in the treatment of MS.

Jennyb Current p 984

Recovery from acute relapses may be hastened by treatment with corticosteroids, but the extent of recovery is unchanged.  Long-term treatment with steroids provides on benefit and does not prevent further relapses.

In pts with relapsing-remitting or secondary progressive disease treatment with beta interferon reduces the frequency of exacerbations.

Anonymous Current p. 984.

-Corticosteroids can speed recovery from acute relapses but extent of recovery is not changed.  Longterm treatment with steroids provides no benefit and does not prevent further relapses.

-Beta interferon treatment reduces frequency of exacerbations in patients with relapsing-remitting or secondary progressive disease. (Interferons can alter the course of the disease.)

Anonymous  Noble 1626, Goroll 976

•      Corticosteroids have proven to be beneficial in treating acute attacks of MS by marginally improving the signs and symptoms.  High dose methylprednisolone (pulsed therapy) is often used to treat the acute attacks.

•      Interferons have been shown to be beneficial in MS and were the first agents shown to alter the course of the disease.  Interferon Beta-1a reduces the number and severity of attacks and also may modestly alter the progression of the disease.

•      Interferon beta-1a include Avonex and Rebif

•      Interferon beta-1b include Betaseron

•      Studies show that it is important to start the disease modifying agents early in the course of MS as MRI

      studies have shown that the inflammatory process is active in many patients during

      clinical remission and irreversible axonal injury accumulates with time even during the

      phase of relapsing-remitting disease.

Anonymous  Goroll, pgs. 976-7

High dose parenteral corticosteroids appear capable of shortening an acute attack.  One study looked at a 3-day course of high-dose IV methylprednisolone followed by several weeks of oral prednisone vs. oral prednisone alone vs. placebo.  At 3 years, the IV corticosteroid showed a 66% reduction in relative risk for the development of definite MS.  For now, a course of high-dose IV therapy is preferred.  Whether it should be followed by a more prolonged course of oral prednisone remains unclear because the study did not examine such a program.

The FDA has approved 3 drugs for patients with relapse-remitting MS.  These drugs are recombinant interferon beta-1b (Betaseron), recombinant interferon beta-1a (Avonex), and glatiramer acetate (Copaxone).  Reductions of relapse rates of 33%, 37%, and 29%, respectively, were demonstrated in studies.  The interferons have also been shown to reduce the numbers of new lesions on MRI.  The further indication of primary prevention has recently been added for Avonex.  When Avonex was given after a single episode secondary to demyelination, fewer patients progressed to clinically definite MS.  The drugs currently are available only by injection.  The interferons have been associated with flulike symptoms, at times severe and persistent.  Patients taking Copaxone have experienced uncommonly a systemic reaction of flushing, sweating, and palpitations.  A consensus is growing that disease-modifying therapy should be initiated early in the course of MS, before irreversible disability has developed.

VI.       Movement Disorders: Parkinson's and Huntington's

            1.      List or recognize the triad of tremor, rigidity, and bradykinesia which characterize Parkinsonism.

Pam    Current, p.976

Tremor, rigidity, bradykinesia, and postural instability are the cardinal features of parkinsonism and may be present in any combination.

Anonymous Current p. 976.

Tremor, rigidity, bradykinesia and postural instability are the cardinal features of parkinsonism and may be present in any combination.

Anonymous  Noble 1612, Goroll 986

            The leading symptoms as the disease progresses are rigidity, tremor, bradykinesia.  Other symptoms include masked face, stooped posture, and shuffling gait.

            Patients may exhibit vague symptoms of aching pains in the limbs, neck or back with decreased axial dexterity before tremor is noted.

Anonymous Goroll chap 174

Parkinson’s is a neurodegenerative condition resulting from a loss of dopamine containing neurons. The classic triad of diagnostic signs are those stated in the question. Resting tremors, bradykinesia, which is ”a decrease in spontaneity and movement”, and rigidity. The bradykinesia manifests as a rigidity and slowness in initiation of movements which make voluntary and spontaneous tasks more difficult. Cogwheel rigidity is ultimately manifested in Parkinson’s, which we have already learned.

            2.      Identify idiopathic Parkinson's disease as tending to begin in the 50's – 60's age group.

Pam    Current, p.976

Parkinsonism is a relatively common disorder that occurs in all ethnic groups, with an approximately equal sex distribution.  The most common variety, idiopathic Parkinson’s disease (aka paralysis agitans) begins most often between 45 and 65 years of age.

Anonymous Current p. 976.

The most common variety of parkinsonism, idiopathic Parkinson’s disease (paralysis agitans), begins most often between 45 and 65 years of age.

Anonymous  Noble 1612, Goroll 986

•         Idiopathic is the most common form of the disease with many possible mechanisms including infections, toxins, and drugs.

Onset of symptoms may occur prior to age 50, but a higher percentage present between age 50 and 60.

Anonymous Merck p. 1497

Parkinson’s disease is the 4^th most common neurodegenerative dz of the elderly.  It affects about 1%of the population > 65 y/o and 0.4% of the population> 40 y/o.  The mean age of onset is about 57 y/o.  Onset in childhood or adolescence (juvenile parkinsonism) also occurs.

            3.      Identify dopamine depletion and degeneration of the substantia nigra as the most characteristic pathophysiologic features of Parkinson's disease.

Pam    Current, p. 976

In idiopathic parkinsonism, dopamine depletion due to degeneration of the dopaminergic nigrostriatal system leads to an imbalance of dopamine and acetylcholine, which are neurotransmitters normally present in the corpus striatum.

Anonymous Current p. 976.

In idiopathic parkinsonism, dopamine depletion due to degeneration of the dopaminergic nigrostriatal system leads to an imbalance of dopamine and acetylcholine.

Noble p. 1612.

Neuronal loss in the dopaminergic nigrostiatal (sp?) system (which includes the substantia nigra) leads to the depigmentation of the substantia nigra that is typical in Parkinson’s disease.

Anonymous  Noble, pg 1612

Parkinsonism is a pathophysiologic state caused by degeneration or dysfunction of the dopaminergic nigrostiatal system. This system includes the substantia niagra, caudate nucleus, putamen, and globus pallidus. The cell bodies are in the substantia niagra, and their axons run to the upper basal ganglion structures where dopamine normally acts as an inhibitory neurotransmitter, balancing an excitatory cholinergic system. Neuronal loss in this system leads to the depigmentation of the substantia niagra that is typical in Parkinson’s disease. This disorder may be more complex than this, as other biochemical changes are also apparent and neuronal degeneration occurs not only in the substantia niagra but also more widely within the basal ganglia and sometimes diffusely in the cerebral cortex.

Anonymous  Goroll Ch. 174, pp. 986

Parkinson's disease is a neurodegenerative condition. Its most characteristic pathologic feature is a loss of dopamine-containing neurons; the nuclei of these neurons reside in the pars compacta of the substantia nigra, and the axons terminate in the caudate nucleus and putamen (the striatum). Other pigmented and nonpigmented nuclei in the brainstem and elsewhere are also affected. Associated with neuronal loss is the development of concentric hyalin inclusions in the cytoplasm of affected neurons, called Lewy bodies. Symptoms are believed related to the imbalance between dopaminergic and cholinergic influences on striatal tissue created by the loss of dopamine-containing neurons. Proper striatal function depends on this balance.

            4.      Identify levodopa (L-dopa) as the most common medication used for Parkinson's disease.

Pam    Current, p.977,978

Levodopa, which is converted in the body to dopamine, improves all of the major features of parkinsonism, including bradykinesia, but does not stop the progression of the disorder.

Anonymous Current p. 976.

Treatment is directed at addressing the imbalance described in answer #3.  Treatment options include administration of levodopa, the precursor of dopamine.

Noble p. 1617.

Levodopa is the natural precursor of dopamine.  Symptoms do progress despite L-dopa therapy.  L-dopa is contraindicated in patients with psychotic illness, narrow angle glaucoma and those on MAOI’s.

Anonymous  Noble, pg 1617 and Goroll, pg 988

Levodopa is the natural precursor of dopamine. It crosses the blood-brain barrier and enhances dopaminergic activity. However, because much of drug is converted peripherally by decarbocylase into dopamine (which cannot cross blood-brain barrier), levodopa is best given in combo with peripeheral decarboxylase inhibitor, such as carboxylase. Should be started at small dose and slowly increased to a qid or 4-hourly dose when the patient starts to respond. Average dose is one 250/25 tablet tid. If the effect is variable during the day, with swings in response and a tendency to wear off before next dose, the controlled-release preparation should be used; the dosage increased; frequency of doses increased; or an agonist added.

Long term results have not been encouraging because symptoms progress despite therapy. Suggested levodopa be delayed and not given on diagnosis as it appears to lose its effects after a few years. In patients on longterm therapy, has been observed to cause significant mental changes, although may be due to therapy or natural history of disease. Estimated drug loses 1/3 of effect in 2/3 of patients within 3 years.

Early complications are nausea caused by stimulation of vomiting center in brain and possible direct gastric irritation; postural hypotension; anorexia; depression and confusion; abnormal movements such as retocollis, dystonic postures, akathasias; and cardiac arrythmias. Less common complication is hip fracture from over-enthisiastic resumption of physical activity as the benefits of levodopa therapy appear.

In patients given levodopa of any form, protein intake should be concentrated in the evenings because of the reduction in available dopamine when given with protein, and foods high in pyridoxine (an antagonist to levodopa) should be restricted.

Anonymous

L-dopa is the naturally occurring precursor of dopamine.  It crosses the blood-brain barrier and enhances dopaminergic activity.  However, because much of the drug is converted peripherally by decarboxylase into dopamine (which can not cross the BBB) L-dopa is best given in combination with a peripheral decarboxylase inhibitor such as carbidopa.  Peak effect after 30 minutes to 2 hrs and has a half-life of 1-3 hrs and is decreased by ingestion of protein-rich meal.  Adverse effects: nausea, vomiting, anorexia, hypertension, dyskinesias (chorea, athetosis and dystonia) and hallucinations.

            5.      Identify chorea and dementia as the characteristic features of Huntington's disease.

Pam    Current, p.979-980

Huntington’s disease is characterized by chorea and dementia.  It is inherited in an autosomal dominant manner and occurs throughout the world, in all ethnic groups, with a prevalence rate of about 5 per 100,000.

Anonymous Current p. 979.

Huntington’s disease is characterized by chorea and dementia.  It is inherited in an autosomal dominant manner.

Anonymous  Noble, pg 1531

Chorea of the face, tongue, head and neck, trunk, and limbs is the dominant symptom accompanied by lurching gait and early impairment of voluntary eye sccades. Irritability, paranoia, and antisocial behavior are common and occur early, followed by cognitive deterioration. Depression and suicide are prevalent. Rigidity and bradykinesthia eventually supervene.  

Anonymous  Noble, p. 1530-1531/Goroll, p. 958 :  

Huntington’s disease presents as a specific form of choreiform movements (involuntary jerky movements of the face, tongue, head and neck, trunk, and limbs) combined with what is referred to as a subcortical dementia, in which there is significant motor dysfunction but no prominent aphasia or agnosia.  

            6.      Describe the typical age of onset, usual progressive nature, and ultimate prognosis of Huntington's disease.

Fassil  CMDT  979-80

•        The disease is characterized by chorea and dementia. It is inherited in an autosomal dominant manner and occurs throughout the world, in all ethnic groups. Onset is usually between 30-50 years of age. The disease is progressive and usually leads to a fatal outcome with in 15-20 years.

Colleen CMDT 980

Typical age of onset: 30-50 yrs old

Usual progressive nature:

Initial symptoms may consist of either abnormal movements or intellectual changes but ultimately both occur. Progressive rigidity and akinesia sometimes occur in association with dimentia.

Ultimate prognosis of Huntington's disease: Fatal within 15-20 years

Anonymous Current p. 980.

Clinical onset age is usually 30 to 50.  The disease is progressive and usually leads to fatal outcome within 15 to 20 years.  The initial symptoms may consist of either abnormal movements or intellectual changes, but ultimately both occur.  The earliest mental changes are often behavioral; dementia develops eventually.

Noble p. 1531.

Huntington’s is characterized by dementia and chorea; it has a gradual onset, slow progressive impairment and a fatal outcome.

Anonymous  Noble, pg 1531

Autosomal dominant neurological disease caused by mutation on chromosome 4 with expansion to above 40 CAG triplets coding glutamine. By unknown mechanisms, this causes a progressive loss of GABA-ergic and substance P interneurons of the striatum. A combination of involuntary movement and neuropsychiatric changes appears insidiously and worsens steadily. Onset usually in 4^th & 5^th decades, with incidence of 7/100,000.  Symptoms as stated in previous question. Diagnosis is based on clinical picture and supported on MRI by selective atrophy of heads of caudate nuclei and confirmed by DNA testing. Unfortunately, there is no specific treatment. Chorea, when functionally disabling, can be treated by dopamine blockers such as haloperidol, low-dose bromocriptine, or benzos such as clonazepam. Depression and obsessive compulsive behaviors may respond to SSRI such as fluoxetine.

Anonymous  Noble, 1530-1531 :

Onset is in the 4^th-5^th decade.  Chorea of the face, tongue, head and neck, trunk, and limbs is the dominant symptom, accompanied by lurching gait and early impairment of voluntary eye saccades.  Irritability, paranoia, and antisocial behavior are common and occur early, followed by cognitive deterioration.

Depression and suicide are prevalent.  Rigidity and bradykinesia eventually supervene.  

Unfortunately, there is no cure and no specific treatment.  

            7.      Identify Huntington's disease as an autosomal dominant disorder and describe the implications for genetic counseling for patients with a family history of Huntington's disease.

Fassil  CMDT 980

•        It is inherited in an autosomal dominant manner. There is no cure for Huntington’s disease, at this time.

•        Offspring should be offered genetic counseling. Genetic testing permits presymptomatic detection and definitive diagnosis of the disease.

Colleen  CMDT 980

Huntington’s disease is an autosomal dominant disorder.

Offspring should be offered genetic counseling. Genetic testing permits presymptomatic detection and definitive diagnosis of the disease.

Anonymous Current p. 980.

Huntington’s disease is inherited in an autosomal dominant manner.  Offspring should be offered genetic counseling.  Genetic testing permits presymptomatic detection and definitive diagnosis of the disease.

Anonymous  Noble, p. 1531

Huntington’s Disease/Chorea is an autosomal dominant neurologic disease caused by mutation of a gene on chromosome 4 with expansion to above 40 CAG triplets coding for glutamine.  It is characterized by dementia and chorea that has a gradual onset and slow progressive impairment and a fatal outcome.  Symptoms do not develop until fourth or fifth decades. Symptoms include chorea of the face, tongue, head and neck, trunk and limbs, lurching gait and early impairment of the voluntary eye saccades. Rigidity and bradykinesia eventually supervene.  Diagnosis is based on clinical picture and family history and is supported on MRI scans and DNA testing.

Anonymous Pathophysiology book pg. 558

Huntington disease is inherited as an autosomal dominant trait with high penetrance.  The genetic defect is on the short arm of chromosome Recombinant genetic techniques may someday prevent or control the disorder.  

VII.     Headache

            1.      Describe the history questions, physical exam, and possible lab tests that you would obtain in any of the common headache disorders.

Ky.  Currentp 946-50. Noble p1497-1516*. Gorroll p931-940

Hx Qs:  Goal: of workup is to distinguish primary HA syndromes (migraine +/- aura, cluster HA, tension HA) from HA secondary to mechanical or systemic process. Questions: profile of current HA & hx over years. Separate profiles of different types of HAs.  Duration, onset, radiation, relieving/aggravating factors, type of pain.  Danger signals:  first or worst, rapid onset. Change in pattern, new onset in middle age or later. Progressive or new daily persistent HA. Head pain precipitated by cough, sneeze, or bending down. Systemic sx like myalgias, fever, malaise, wt loss, scalp tenderness or jaw claudication.  Focal neurologic sx or confusion, seizures or LOC.

PE:  blood pressure, heart rate, cardiac status, sinuses, scalp arteries, cervical paraspinal muscles, TMJ, ROM & pain in c-spine.  Neck flexion (meningeal irritaition), bruits over cranium, orbits, neck.  Optic fundi, visual fields, papillary rxn, 5^th cranial nerve sensory function, corneal reflexes. Test motor power in face and limbs, muscle stretch reflexes, plantar responses. Gait.

Lab:  EEG only if associated with seizures or LOC or atypical migrainous aura.  Lumbar puncture if HA is first or worst in pts life.  LP if suspect meningitis, encephalitis, subarachnoid hemorrhage or high/low pressure HA syndromes.  CT or MRI warranted in adults whose HAs fit broad definition of recurrent migraine & the pattern changes, hx of seizures, or presence of focal neuro sx.  ESR.

Anonymous Current p. 946.

-History Questions:

~Intensity, quality and site of pain.

~Duration of headache and presence of associated neuro symptoms (can indicate cause).

~Sharp lancinating pain suggests neuritic cause.

~Dull or steady ache is typical of intracranial mass lesion.

~With sinusitis there may be tenderness of overlying skin and bone.

~With intracranial mass headache may be focal or generalized.

~With trigeminal or glossopharyngeal neuralgia, pain follows nerve pathway.

~Ask about precipitating factors.

~Ask of recent sinusitis, hay fever, dental surgery, head injury, systemic viral infection.

~Ask about TMJ (possible cause)

~If worse on awakening, possibly sinusitis or intracranial mass cause.

~See DDx lists under #4 for more history questions.

-Physical Exam:   Noble p. 1498

Should be performed at the 1^st consultation for a headache problem and should evaluate BP, HR, cardiac status, sinuses, scalp arteries, cervical paraspinal muscles, TMJs, ROM of and presence of pain in C-spine.  Neuro exam capable of detecting most of the abnormal signs likely to occur in patients with headaches caused by intracranial or systemic disease should include:

~neck flexion (check for meningeal irritation).

~check for bruits at neck.

~optic fundi, visual fields, papillary reactions, CN V, sensory fx, corneal reflexes.

~motor power in the face and limbs, muscle stretch reflexes, plantar responses and gait.

-Labs:   Goroll p. 936, Noble p. 1498

~CT scan if life threatening lesions are suspected OR if headache is of recent onset (<6 mo.) and H&P cannot diagnose cause OR if headache is becoming progressively worse and doesn’t fit a patten of tension headache.

~LP if you suspect meningitis, encephalitis, subarachnoid hemorrhage.

~ESR if giant cell temporal arteritis is suspected (elderly, new onset headache, sensitivity over scalp or cranial artery).  Also consider temporal artery biopsy.

Anonymous Goroll, p. 935

• History- Obtaining a full description of the headache, particularly its clinical course, associated symptoms, precipitants, aggravating and alleviating factors, associated neurological deficits, fever or neck stiffness and patients concerns. A medication history is essential because a number of agents (i.e., indomethacin, nifedipine, cimetidine, captopril, nitrates, atenolol, Bactrim, oral contraceptives) can trigger non-specific headaches. Sudden onsets of the “worst headache ever experienced” requires immediate attention especially if fever, neck stiffness, ataxia, alteration in mental status, focal neuro deficit or visual impairment is reported.

• PE-  Noble, p. 1498 -  Should be performed at the first consultation for a headache problem and should evaluate BP, HR, cardiac status, sinuses, scalp arteries, cervical paraspinal muscles, TMJ’s, ROM of and presence of pain in the cervical spine.  Neuro exam capable of detecting most of the abnormal signs likely to occur in patients with headaches caused by intracranial or systemic disease should include evaluation of the following:

               1. Neck flexion (for evidence of meningeal irritation)

               2. Presence of bruits over the cranium, orbits, or neck

   3. Optic fundi, visual fields, papillary reactions, fifth cranial nerve

   sensory function, and corneal reflexes.

   4. Motor power in the face and limbs, muscle stretch reflexes, plantar                                       

   reponses, and gait.

                        The presence of any such abnormalities suggests the need for further investigation.

• Labs-  Goroll, p. 936, Noble, 1498  A CT scan should be ordered if suspected life-threatening lesions(midline cerebellar hemorrhage, CNS mass), headache of recent onset (< 6 months) which cause is still not apparent after a thorough history and PE, or a headache that is worsening with time and does not fit the pattern of a tension-type headache.  A lumbar puncture with culture if meningitis, encephalitis, subarachnoid hemorrhage, high-pressure or low-pressure headache symptoms are considered possible.  In a new onset headache in an elderly patient, especially if accompanied by cranial artery or scalp tenderness, an ESR should be done to check for giant cell arteritis, with consideration for a temporal artery biopsy.

Anonymous  Nobel p. 1497

Hx: will determine the likely diagnosis; by the brief, structured of CODDARA MOAP FLEAS FOSH – You should know this!

Must be able to determine during the interview what the pt wants: is it pain relief, reassurance the no serious disease underlies the symptoms experienced, or simply and explanation?

Danger signals: In Hx

-The first or worst headache of the pt’s life, particularly if of rapid onset (Subarachnoid hemorrhage)

-A change in frequency, severity, or features of the headache attack from that commonly experienced in the past (any pathology?)

-The new onset of headache in the middle age or later, or a significant change in any long-standing headache pattern (new pathology?)

-The appearance of a progressive or new daily, persistent headache (medication-induced headache)

-The precipitation of head pain w/ coughing, sneezing, or bending down (mass lesion, Chiari malformation?)

-The presence of systemic symptoms such as myalgias, fevers, malaise, wt loss, scalp tenderness, or jaw claudication (cranial arteritis?)

-The presence of focal neurologic symptoms or any abnormalities on neurologic examination, or of confusion, seizures, or any impairment in the level of consciousness (mass lesion?)

PE: Evaluate the BP, HR, cardiac status, sinuses, scalp arteries, cervical paraspinal muscles, TMJ, and the range of motion of and presence of pain in the cervical spine.

A screening neurologic examination capable of detecting most of abnormal signs likely to occur in pt w/ headaches caused by intracranial or systemic disease: should include

-Neck flexion (for evidence of meningeal irritation),

-The presence of bruits over the cranium, orbits, or neck

-The optic fundi, visual fields, papillary reactions, 5^th cranial nerve sensory function, and corneal reflexes

-Motor power in the face and limbs, muscle stretch reflexes, plantar responses, and gait.

Possible Labs: Hx is the most important step in the management of headache patient.

-Electroencephalogram (EEG) not useful in routine evaluation of pts w/ headache, but associated symptoms suggesting such as seizure disorder, such as atypical migrainous aura or episodic loss of consciousness

-Lumbar puncture (LP) may have value if the headache is the first or worst in the pt life; in the presence of severe, rapid onset, recurrent headache, or progressive headache w/o signs of raised intracranial pressure; w/ atypical, chronic, and intractable headache; or when headache is associated w/ fever. And if the DDx is meningitis, encephalitis, Subarachnoid hemorrhages, or high-pressure or low-pressure headache syndrome are considered possible.

-CT scan to R/O or confirm the diagnosis if DDx is Subarachnoid hemorrhage

-CT or MRI scans are only warranted in adults pts whose headache fit a broad definition of recurrent migraine, and substantial changes in headache pattern, a Hx of seizures, or presence of focal neurologic symptoms or signs.

            2.      Distinguish between new, acute headaches and chronic, recurrent headaches in your differential diagnosis and approach to the patient.

Ky.  Current p 946-50. Noble p1497-1516.  Gorroll p931-940*

New Acute:  Approach the same as in Q1.  Severity of pain helps identify ↑ risk of serious pathology.  In pt unaccustomed to having headaches who presents with sudden onset of “worst” HA ever deserves prompt attention, particularly if fever, neck stiffness, ataxia, altered mentation, focal neuro deficit, or visual impairment.  Cerebellar hemorrhage = stiff neck + gait ataxia + profuse N/V.  Ruptured cerebral aneurysm = abrupt onset & reaches maximum intensity immediately.  Hypertensive encephalopathy = diffuse HA, N/V altered mental status.  Acute glaucoma = eye pain + blurred vision.  Temporal arteritis:  new onset in elderly

Chronic Recurrent:  Approach the same as in Q1.  Intensity of pain little value b/c it typically varies.  Associated sx of more value and lead to other causes of HA.  Giant Cell arteritis = jaw claudication + scalp vessel tenderness & may have concurrent shoulder/ hip-girdle discomfort.  Cervical radiculopathy = neck pain.  Hx of head trauma, parameningeal infxn, depression , situational stress, substance abuse & family hx of HA, use/overuse of medication.

Anonymous Current p. 946.

The onset of a severe headache in a previously well patient is more likely than chronic headache to relate to an intracranial disorder such as subarachnoid hemorrhage or meningitis.

Anonymous  Goroll, p. 934-36

•                    New, acute onset- In exam, BP and temperature should be checked for elevations, scalp for artery tenderness(temporal arteritis), sinuses for purulent discharge and tenderness(acute sinusitis), pupils for loss of reactivity, corneas for clouding (acute glaucoma) disc margins for papilledema, neck for rigidity on anterior flexion, neuro exam for ataxia, alteration of mental status, focal deficits and meningeal signs.  

o       Differential Dx for acute headache includes: meningitis, intracranial hemorrhage, stroke, acute increase in ICP, acute glaucoma, acute sinusitis, acute metabolic disturbance (CO poisoning, hypoglycemia) acute viral illness, initial presentation of a persistent or recurrent headache.

•                    Chronic or recurrent headache- Should include the entire acute onset headache exam and expand into areas based on findings from the history. (i.e. history that includes facial pain, the oral cavity should be examined for a trigger zone indicative of trigeminal neuralgia, teeth for signs of bruxism, TMJ for limited ROM and crepitus, neck for signs of degenerative disease and movements that reproduce pain.)

o       Differential Dx for persistent, or recurrent headache-Intracranial mass lesion (neoplasm abscess, subdural hematoma, large A-V malformation, tension-type headache, migraine, with and without aura, cluster headache, indomethacin-responsive headache, postconcussion syndrome, cervical spine disease, giant cell arteritis, trigeminal neuralgia, HTN, A-V malformation, bruxism/TMJ dysfunction, medications, substance abuse.

•                    Labs should be ordered as appropriate (see L1 above for criteria).

Anonymous  Nobel p. 1498

New and Acute:  DDX

Meningitis, encephalitis

Cerebral abscess

Intracerebral bleed/ Subarachnoid hemorrhage/ acute Subdural hemorrhage

Acutely raised intracranial pressure from other causes ( ball-valve III ventricle cyst)

Migraine, cluster headache

Triggering agents w/ MAOI’s (cheese, monosodium glutamate, alcohol, chocolate, red wine)

Nitrites

HTN encephalopathy

Pheochromocytoma

Post-seizure headache

Chronic and Recurrent headaches: will be diagnosed from the Hx i.e. induced by medications, HTN, Post-traumatic headache, withdrawal from steroids, or ETOH, or recreational drugs, Whiplash, TMJ, stress: tension-type, Glaucoma, Migraine HA: familial, w and w/o aura, Cluster HA, Metabolic abnormality, Cold sinuses HA  -- so must ask a lot of questions to be able to diagnosis the cause of the Chronic or recurrent headache!

            3.      Recognize signs and symptoms of potential organic or serious disease in a headache patient.

Ky.  Noble p1497

Danger signals:  first or worst, rapid onset. Change in pattern, new onset in middle age or later. Progressive or new daily persistent HA. Head pain precipitated by cough, sneeze, or bending down. Systemic sx like myalgias, fever, malaise, wt loss, scalp tenderness or jaw claudication.  Focal neurologic sx or confusion, seizures or LOC.

Anonymous Current p. 946.

~A progressive headache disorder, new onset of headache in middle or later life, headaches that disturb sleep or are related to exertion, and headaches that are associated with neurologic symptoms or a focal neurologic deficit all have potential of organic or serious disease.  These usually require cranial MRI or CT scan to exclude an intracranial mass lesion. 

~Signs of meningeal irritation and impairment of consciousness also indicate need for further investigation b/c of the potential for serious disease.  Investigation includes CT, MRI and CSF analysis.

Anonymous  Noble, p. 1497

• First or worst headache of the patient’s life, particularly if of rapid onset (subarachnoid hemorrhage?)

• A change in frequency, severity or features of the headache attack from that commonly experienced in the past (new pathology?)

• The new onset of headache in middle age or later or a significant change in nay long-standing headache pattern (new pathology?)

• Appearance of a progressive or new daily, persistent headache (medication-induced?)

• Precipitation of head pain with coughing, sneezing or bending down (mass, lesion, Chiari malformation?)

• The presence of systemic symptoms such as myalgias, fever, malaise, weight loss, scalp tenderness or jaw claudication (cranial arteritis?)

• Presence of focal neurologic symptoms or any abnormalities on neurological exam, or of confusion, seizures, or any impairment in the level of consciousness (mass lesion?)

Anonymous

-A patient who’s frequent HA begins after age 25.

-Progressive HA over days or weeks, localized to one area, and increasing in severity

-HA caused by exertion (Bending over, coughing, exercise, sneezing, or Orgasm)

-HA w/ an abrupt onset and intense severity

-HA associated w/ altered mental status, meningismus, neurologic deficits, papilledema, or seizures.

Signs:                                                              Symptoms:

Fever                                                               Migraine: w and w/o aura

Malignant HTN                                                Photo/phonophobia

Sleep apnea                                                      Anorexia / N/V

Depression                                                       Exacerbation w/ exertion

Hypoxemia                                                       Cluster:  Lacrimation / rhinorrhea / Horner’s

Chronic renal failure                                          syndrome

Polycythemia / anemia                                       Tension type: Dysthymia, Depression

Systemic Lupus erythematosus                         

Fibromyalgia                                                     Symptom suggesting Organic HA:

Hyponatremia                                                   New, massive, or gradually increasing HA

Hypothyroidism                                                Personality changes

Hyperthyroidism                                               Constant, worsening in horizontal position

Adrenal insufficiency                                         HA in late nights or morning

Hyperparathyroidism / hypercalcemia

            4.      Describe the major features of the history, physical exam, diagnosis and basic principles of treatment* for each of the following headache syndromes:

                              tension headaches (aka: muscle contraction headaches),

                              migraine with aura (aka: classic migraine),

                              migraine without aura (aka: common migraine),

                              cluster headaches.

                 * For treatment, concentrate on patient education and non-pharmacologic therapy, appropriate use of analgesics, and avoiding  drug dependence.  For migraines, read carefully about prophylaxis, ergotamines, and sumitriptan. There has been alot of  new treatment options recently, focus more on the presentations and characteristics of the various types of headaches and keep in mind that you will need to stay abreast of the current treatment options.

Ky

Tension headaches (aka: muscle contraction headaches). Goroll: 933

Hx:  Bandlike sensation about head. feeling of pressure.  Steady dull pain. Worse as day pregresses. Occipital & nuchal soreness.  Last days, weeks, months.  Nausea & throbbing attributed to vasoconstriction.  Precipitants: anxiety, depression, situational stress.

PE:  As in Q1

Tx:  life style modification. Tx psych comorbidity.  Ergotamine or analgesic abuse can lead to vicious cycle of HA, meds, HA.  Prescribe with caution.

migraine with aura (aka: classic migraine) Noble p1501

Hx:  More disabling than w/out aura.  Same criteria as migraine w/out aura.  Auras: sharp beginning and ending.  Lasts 10-60 minutes (mean 20 min).  followed by beginning of HA.  Visual aura in form of scintillating scotoma is most common.  It starts as a small twinkling, corrugated circle of tiny white, golden, or colored lights that form pattern similar to medieval fortifications.  Lights appear in one homonymous field, spread peripherally and followed by visual loss.  Gradually apparition disappears, vision returns, and HA begins.  Also:  prickling in fingers of one hand that creeps up arm to face.  Hypoesthesia in prickly area.  Aphasia, confusion, fugeulike states, distortion of size of objects, Other aura description:  flashing lights or seeing through heat waves.

PE:  As in Q1

Tx: focused on seratonin receptors.  Prophylaxis: Serotonin Antagonist =methysergide.  B-blockers, TCAs, Calcium Channel blockers = verapamil.  NSAIDs = naproxen. Anticonvulsants.  Abortive options: ergotamine, triptans.  Nonpharm:  relaxation techniques, exercise.  Stress management therapy.

migraine without aura (aka: common migraine) Noble p1500

Hx:  More common that with aura.  See box 160-3 in Noble for Dx criteria.  HA last 2 to 72 hours (+/- tx).  Unilateral location (30-40% are bilateral). Pulsating quality.  Moderate to severe intensity.  Aggravated by walking up stairs (or similar activity).  During HA 1 or more of: N/V; Photophobia/phonophobia/osmophobia (aversion to odors).  Additionally, HA occur perimenstrually. HA relieved by sleep (hypersomnia).  Irritability.  Mood lability.  Food cravings.

PE:  As in Q1

Tx: focused on seratonin receptors.  Prophylaxis: Serotonin Antagonist =methysergide.  B-blockers, TCAs, Calcium Channel blockers = verapamil.  NSAIDs = naproxen. Anticonvulsants.  Abortive options: ergotamine, triptans.  Nonpharm:  relaxation techniques, exercise.  Stress management therapy.

cluster headaches.  Goroll p933-934.

Hx:  extracerebral vasodilation is a component.  Predominantly in middle aged men (only type where men>women).  Distinguished by: location, timing, periodicity.  50% describe intense nonthrobbing, unilateral HA behind the eye in which pain is searing, stabbing, or burning and has ipsilateral lacrimation, nasal stuffiness and facial flushing.  Ipsilateral ptosis & miosis.  HA begins few hours after pt goes to bed and lasts 30-90 minutes.  Attacks occur nightly for 2-3months, disappear and return several months to years later.  10% have cluster HA.  Stress and alcohol precipitate attacks-etoh okay between attacks.

PE:  As in Q1. 

Tx:  Prophylactic and abortive tx available.  Abortive options:  O₂, Ergotamine suppository. Bihydroergotamine (IM/IV).  Sumatriptan (SQ).  Dexamethasone or prednisone.  Prophylactic options:  daily verapamil. Methysergide. Lithium.

Anonymous Current p. 946.

Please read p. 946-952.

History:

-Tension headaches

~pulsating or throbbing

~sense of pressure or tightness

~band like pain

~worse with stress or at end of day

~poor concentration and other vague symptoms

~constant daily headaches

~viselike

~worse with stress, fatigue, noise, glare

-Migraine with aura

~pulsating or throbbing

~ocular or periorbital icepick like pains

~lateralized headache common

~may be exacerbated by stress, fatigue, nitrite, tyramine, menstruation, oral contracep.

~episodic

~often begin in adolescence or early adult life

~family history is common

-Migraine without aura

~pulsating or throbbing

~ocular or periorbital icepick like pains

~lateralized headache common

~may be precipitated by stress, fatigue, nitrite, tyramine, menstruation, oral contracep.

~episodic

~often begin in adolescence or early adult life

~family history is common

~This one obviously involves somekind of visual disturbance (stars, sparks, flashes)

-Cluster headaches

~ocular or periorbital icepick like pains

~lateralized headache common

~may be precipitated by alcohol

~occur at same time each day or night they occur

~mostly middle aged men are affected

Physical Exam (there’s not much on this), Diagnosis (use history info above in #1 and #4, again b/c there’s not much on this in the books) & Treatment:

-Tension Headaches

~When simple analgesics are ineffective, use antimigrainous agents, hot baths & biofeedback.

~Look into underlying causes of chronic anxiety.

-Migraine

~Avoid precipitating factors.

~Use prophylactic or symptomatic pharmacologic treatment.

~Consider prophylaxis if migraines occur >2-3 times per month.

~Rest in quiet, darkened room.

~Start tx with simple analgesic (like aspirin) at start of migraine.

~Take a cafergot (combo of ergotamine tartrate, 1 mg and caffeine, 100 mg) tablet at onset of headache, then one every 30 minutes, up to 6 per attack and up to 10 per week.

~Cafergot is also available via suppository, SC, IM, inhalation, sublingual, etc.

~Ergotamine products are contraindicated in pregnancy.

~Sumatriptan is a rapidly effective agent for aborting attacks when given subQ.

~Sumatriptan has a high affinity for serotonin receptors.

~Avoid sumatriptan in pregnancy.

~Zolmitriptan is another selective serotonin receptor agonist.

-Cluster headaches

~Examination reveals no abnormality except Horner’s syndrome that either occurs transiently during attacks or remains as a permanent deficit.

~Treatment with oral drugs is usually ineffective.

~Treatment with subQ sumatriptan or an ergotamine product can be effective.

Anonymous  Goroll 932-939

Tension HA- leading cause of chronic and recurrent HA. 90% are bilateral and are often described as a feeling or pressure or a band-like sensation about the head. Pain is dull, and steady in most; characteristically get worse as the day progresses and sometimes accompanied by occipital and nuchal soreness. HA may last for days, wks, or even months. Recording of the myographic potentials from head to neck muscles reveals vigorous contractions in some but not all pt w/ this time of HA. Vasoconstriction can also be detected and may account for migraine symptoms like nausea or throbbing.

Precipitants include anxiety, depression, and situational stress. Pt describe the HA usually in vivid terms (feels like an ax, or lighting, or something exploding), yet they do so w/out demonstrating discomfort. May occur secondary to muscle strain from cervical spondylosis or TMJ disease.

Tx w/ mild analgesics such as ASA, Tyl, or non-prescriptive NSAID. Address underlying source of psychological distress. Stress reduction and antidepressant therapy may help.

Migraine w/ aura (classic migraine)- affects 10% of adults, women 3times more than men. Migraine is associated w/ high rates of disability, but most pr w. migraine have not been dx w/ by a doctor or treated w/ prescription meds. Family hx is positive in 2/3^rds, especially w/ pt that have a hx of migraines w/ aura. Usually dx in childhood or young adult life. Condition tends to improve in many women during pregnancy, although BC pills have been known to precipitate migraines or convert a migraine w/out aura to migraine w/ aura.1 in 7 women w/ migraine HA occurs only during the 1^st few days of menses.

Attacks may comprise as many as five phases: prodrome, aura, HA, termination, and postdrome. Prodrome is characterized by lassitude, irritability, difficulty concentrating, and nausea. Aura often reported as visual phenomena (scotoma, zigzag patterns, diplopia, hemianopsia), vertigo, aphasia, or even hemiplegia preceding the HA, which is typically unilateral and throbbing. Termination usually occurs w/in 24hrs, but sometimes not until after 48hrs. The postdrome includes feelings of fatigue, sleepiness, or irritability. Both migraines w/ and w/out aura have nausea and photophobia.

            Dx criteria:

a)      A least 2 attacks fulfilling B

b)      At least three of the following four characteristics:

1.      One or more fully reversible aura symptoms indicating focal cerebral cortical or brainstem dysfunction.

2.      At least 1 aura symptom develops gradually over more than 4 minutes, or 2 or more symptoms occur in succession.

3.      No aura symptom lasts more than 60 minutes. If more than 1 aura is present accepted duration is proportionally increased.

4.      HA follows aura w. a free interval of less than 60 minutes (It may also begin before or simultaneously w/ the aura.)

Tx:  Combination of a tolerable prophylactic med, w/ occasional use of analgesic once a wk, it is reasonable to consider prophylactic med. Education the migraine chocolates, citrus fruit, nuts, red wine, caffeine, tobacco, ETOH), inadequate sleep, and prolonged fasting. Useful to keep HA diary help determine precipitants to HA.

Nonpharmacologic tx:  Regular exercise, and relaxation, and biofeedback.   Pharmacological tx for prophylaxis each drug should be given at least a 2month trial before determining that something does not work. Tricyclic antidepressants, beta-blockers, CCB (verapamil), NSAID (naproxene), Valproate.  Abortive tx is for pt w/ occasional migraines and fro pt w/ generally well controlled but occasionally severe migraine.  

Tx of aural s/sx w/ NSAIDs and a prokinetic and antiemetic such as metoclopramide and compazine. Other drugs dihydroergotamine, and triptans (sumatriptan, zolmitripran, naratriptan). Analgesics and sedatives it is best to avoid their use in migraine pt. Popular combination drug that are used contain acetaminophen or ASA, w/ butalbital, and caffeine (fioricet).  Sometimes narcotic analgesics are used, but their regular use can lead to a vicious cycle of HA, narcotic intake, more HA, and more narcotic intake, etc…

Migraine w/out aura (common migraine):

A) At least 5 attacks fulfilling B-D.

B) HA lasting 4-72hrs (untreated or unsuccessfully tx)

C) HA w/ at least 2 of the following-

•        Unilateral location

•        Pulsating quality

•        Moderate or severe intensity (inhibits daily activity)

•        Aggravated by walking stairs or similar physical routine

D) During HA at least one of the following-

Nausea or vomiting

Photophobia or phonophobia

Cluster Headache:

Pathology is unknown. Occurs predominately in middle-age men and is the only type of HA more common in men than in women. Distinguished by its location, timing, and periodicity. Most present as an intense, non-throbbing, unilateral HA “behind the eye” that is searing, stabbing, or burning and accompanied by ipsilateral lacrimation, nasal stuffiness, and facial flushing. Occasionally ipsilateral ptosis or miosis can be seen. HA begins a few hrs after pt goes to bed and lasts 30-90 minutes. Attacks occur nightly for 2 –3 months and then disappear, only to return several months later. Stress and ETOH are believed to be precipitants.

Tx: Both abortive and prophylactic tx available. One abortive is the use of O2 via inhalation 5-8l/min X 10”.  Ergotamine suppositories may be effective and taken at bedtime during a cluster HA. Dihydroergotamine, sumatriptan SQ, dexamethasone or prednisone can be used. Prophylactic tx w/ verapamil, methysergide, lithium, or a radiofrequency trigeminal rhizotomy.

Anonymous

Tension headaches (muscle contraction headaches) - A bilateral headache having a pressing or tightening quality of mild to moderate severity.  It is not aggravated by physical activity or associated with vomiting.  Chronic tension-type headache is usually a daily or almost-daily headache; associated sx may include depression and dysthymia.  PE consists of normal physical and neurological exams; TX includes NSAIDS, antidepressants, and muscle relaxants.

Migraine with aura (classic migraine) – onset usually in childhood, adolescence, or early adulthood.  Family HX is often positive and migraine is more common in women.  Classic triad: visual scotomata or scintillations, unilateral throbbing headache, accompanied by nausea and vomiting.  An attack lasting 2-6 hours is common with relief after sleep.  Attacks may be triggered by wine, cheese, chocolate, contraceptives, stress, exercise, travel, or smoke.  PE finds photophobia, vomiting, normal neuro exam.  TX is removal of environmental triggers, NSIADS, Sumatriptan, ergotamine, dihydroergotamine, amitriptyline, propranolol, verapamil, valproate, ergonovine.

Migraine without aura (common migraine) – unilateral or bilateral headache with nausea, but rarely with vomiting or visual complaints.  More common in women.  Onset is usually more gradual than in classic migraine and pain becomes more generalized and may persist for hours or days.  PE usually normal.  TX same as above.

Cluster headaches – marked by cycles of headache lasting 1 to 4 months, separated by remissions of 6 to 24 months.  The attacks are always unilateral and located around the eye, temple, or upper jaw.  Associated sx include reddening and tearing of the eye, drooping of the eyelid, nasal stuffiness, and rhinorrhea.  The attacks usually last from 15 min to 2 hours, occur 1 to 4 times daily, and often awaken the patient from sleep.  Pain is excruciating, and the pt may pace the floor during the attack.  PE shows above mentioned findings with a usual normal neuro exam.  TX consists of verapamil, corticosteroids, methysergide, lithium, oxygen, sumatriptan, ergotamine, or lidocaine.

            5.      Recognize the presentation of any of the following other causes of headaches by typical signs and symptoms.

                     intracranial hemorrhage (especially subarachnoidhemorrhage)

                     sinusitisheadache

                     ocular headache (refraction error/glaucoma)

                     temporal arteritis

                     meningitis

                     posttraumatic headache

                     lumbar puncture headache

                     intracranial neoplasm headache

                     TMJ dysfunction/bruxismheadache of neck origin (cervical radiculopathy)

Ky.  Currentp 946-50. Noble p1497-1516. Gorroll p931-940

intracranial hemorrhage (especially subarachnoidhemorrhage):  first and worst HA of life. Rapid onset.

Sinusitis headache:  acute in onset and worse on awakening. Improves upon arising and worsens as day progresses.  Purulent nasal d/c, pain and skin sensitivity over involved sinus.  Can be throbbing.  Worsens when bending over.

ocular headache (refraction error/glaucoma):  astigmatism causes ocular muscle imbalance and sustained contrationc of extraocular, frontal and temporal muscles and then aching discomfort about orbit and frontotemporal region.  Refraction corrects problem.  Acute glaucoma may produce an orbital HA of sudden onset that is accompanied by cloudy vision.  HA often erroneously attributed to eyestrain—refraction fails to improve problem when this is true.

temporal arteritis:  dz of elderly.  Affects medium/large arteries (esp extracranial vasculature) can cause blindness.  HA begins as a throbbing discomfort and progress to dull aching pain.  Some not burning others not lancinating.  Scalp tenderness (esp combing hair) localized to involved vessel.  Jaw claudication.  Polymyalgia rheumatica.  Blindness.  Diplopia may precede and is predictive of visual impairment.  Once visual loss sets in, it progresses quicly over several hours to total visual loss.

meningitis:  acute  onset of severe pain that is generalized and constant.  Sx intense at base of skull.  Aggravated by forward flexion of neck or by leg raise with knee extension with foot dorsiflexion

posttraumatic headache:  (post concussion):  poorly characterized state manifested by chronic refractory HA, neck pain, nervousness, emotional lability, crying spells, and inability to concentrate.  Usually appears w/in a day or so of injury.  Worsen over ensuing weeks and gradually resides.  Constant dull ache with superimposed throbbing.  Nausea & vomiting. Scintillating scotomas.

lumbar puncture headache:  (if you find this please let me know)

intracranial neoplasm headache:  HA may be sole initial complaint w/out neuro deficits.  New neurologic deficits usually ensue.

TMJ dysfunction/bruxism (nocturnal teeth grinding):  masticator muscle fatigue and spasm.  Chronic dull, aching, unilateral discomfort about jaw, behind eyes and ears, even down neck into shoulders.  Jaw pain, clicking sounds, difficulty opening mouth in morning. Jaw locking common.  Chewing may exacerbate sx.  Molar flattening.  Jaw deviation upon opening.

Headache of neck origin: (Tension HA?)  poor concentration and other vague nonspecific sx.  Vise-like or tight in quality and may be exacerbated by emotional stress, fatigue, noise, or glare.  Usually generalized.

Cervical radiculopathy:  HA commonly first sx of CR.  Pain arises during mechanical irritation of upper cervical root.  Pain often localized to one side of occiput or base of skull in conjunction with tenderness to palpation.  May start in neck and radiate to forehead or eye.  Pain described as nagging or aching and aggravated by neck movement.  Worse on awakening.  Sudden lancinating pain in distribution of greater occipital nerve.  Often relieved by sitting up.

Anonymous Current p. 949.

-Intracranial hemorrhage (especially subarachnoid hemorrhage):

-Sinusitis headache:

-Ocular headache (refraction error/glaucoma):

-Temporal arteritis:

-Meningitis:

-Posttraumatic headache:

-Lumbar puncture headache:

-Intracranial neoplasm headache:

-TMJ dysfunction/bruxism headache of neck origin (cervical radiculopathy):

Anonymous  Bates 74-77  

Intracranial hemorrhage (particularly subarachniod hemorrhage):

• Process: bleeding, most often from a ruptured intracarnial aneurysm.

• Location: generalized.

• Quality & severity: very severe, “ the worst HA of my life”

• Onset: usually abrupt, prodromal symptoms may occur.

• Duration: variable, usually days.

• Course: a persistent HA in an acute illness.

• Associated S/Sx: N/V, possible loss of consciousness, neck pain.

• Aggravating factors or provoke: n/a

• Misc: acute illness w/ very severe HA.

Sinusitis headache:

• Process: mucosal inflammation of the paranasal sinuses and their openings.

• Location: usually above the eye (frontal sinus) or the cheekbone area (maxillary sinus), one or both sides.

• Quality & severity: aching or throbbing, variable, in severity

• Onset: variable, usually acute.

• Duration: often several hrs at a time, recurring over days or longer.

• Course: often recurrent in a repetitive pattern; start in AM or in the afternoon, and usually worse upon waking, better upon arising, only to worsen as day progresses

• Associated S/Sx: local tenderness, nasal congestion, discharge, and fever.

• Aggravating factors or provoke: by coughing, sneezing, or jarring the head. Also by bending over.

Ocular headache (refraction error/glaucoma)

• Process: probably the sustained contraction of the extraocular muscles, and possibly of the frontal, temporal, and occipital muscles.

• Location: around and over the eyes, may radiate to the occipital area.

• Quality & severity: steady, aching, dull

• Onset: gradual

• Duration: variable

• Course: variable

• Associated S/Sx: eye fatigue, “sandy” sensations in the eyes redness of the conjunctiva.

• Aggravating factors or provoke: prolonged use of the eyes, particularly for close work.

• Misc: face pain.

Acute glaucoma

• Process: sudden increases in intraocular pressure

• Location: in and around one or both eyes

• Quality & severity: steady, aching, often severe

• Onset: rapid onset

• Duration: variable may depend on tx

• Course: variable may depend on tx

• Associated S/Sx: diminished vision, sometimes N/V

• Aggravating factors or provoke: sometimes provoked by drops that dilate the pupils

• Misc: face pains

Temporal arteritis:

• Process: chronic inflammation of the cranial arteries, cause unknown, often associated w/ polymyalgia rheumatica

• Location: localized near the involved artery (most often the temporal also the occipital) may become generalized.

• Quality & severity: aching, throbbing, or burning, often severe

• Onset: gradual or rapid

• Duration: variable

• Course: recurrent or persistent over wks to months

• Associated S/Sx: tenderness of the adjacent scalp; fever, malaise, fatigue, and anorexia; muscular aches and stiffness; visual loss or blindness.

• Aggravating factors or provoke: n/a

• Misc: disease of the elderly pt’s. Can cause blindness if ophthalmic arteries become involved.

Meningitis

• Process: infection of the meninges that surround the brain

• Location: generalized

• Quality & severity: steady, or throbbing, very severe

• Onset: fairly rapid

• Duration: variable, usually days

• Course: a persistent HA in an acute illness

• Associated S/Sx: fever, stiff neck

• Aggravating factors or provoke: n/a

• Misc: acute illness with severe HA, symptoms may be particularly intense at the base of the skull and aggravated by forward flexion of the neck or by leg raising in conjunction w/ knee extension and foot dorsiflexion.

Post traumatic headache

• Process: mechanism unclear

• Location: may be localized to the injured area, but not necessarily

• Quality & severity: variable

• Onset: within a few hrs of the injury

• Duration: wks, months, and yrs

• Course: tends to diminish over time

• Associated S/Sx: poor concentration, giddiness, or vertigo, irritability, restlessness, tenseness, and fatigue

• Aggravating factors or provoke: mental and physical exertion, straining, stooping, emotional excitement, ETOH

• Misc: HA following head trauma. The S/Sx are suggestive of an agitated depression following trauma. Is a complicated poorly characterized state manifested by chronic refractory HA, neck pain, nervousness, crying spells, and inability to concentrate.

Lumbar puncture headache:

Intracranial neoplasm:  goroll 931

            About 1/3^rd of patients w/ a mass lesion have HA as an early S/Sx.   Pain is localized to the side of the lesion.   Presentations vary widely. HA may be mild or severe, intermittent or persistent, aching, sharp, pressure-like, or even throbbing.   Characteristically the HA remains in the same location, but is progressive; increases in the duration and severity of pain over several months occur in conjunction w/ subtle changes in mental status or the development of focal neurological deficits.  As intracranial pressure increases, lying down may exacerbate the HA, as may straining at bowel movements, coughing, or bending over, and a more generalized HA may develop. Nocturnal awakening is common but not dx.   Projectile vomiting is a late complication.

TMJ dysfunction/bruxism headache of neck origin  goroll 931

            Sometimes an overlooked cause of chronic refractory HA. Occasionally the problem is caused by joint changes resulting from malocclusion.  However, the problem in most cases is not malocclusion, but rather tension-induced jaw clenching and nocturnal teeth grinding (bruxism).  Chronic involuntary oral habits lead to masticator muscle fatigue and spasm.  Chronic dull, aching, unilateral discomfort may be described about the jaw, behind the eyes and the ears, and even down the neck into the shoulders.  Jaw pain, clicking sounds, and difficulty opening the mouth in the morning are characteristic.  Chewing may exacerbate s/sx; locking of the jaw is common.  PE may reveal masticatory muscle tenderness, mandibular hypomotility, and joint click and deviation on opening are noted. Molar prominences may be flat from chronic grinding of the teeth.

Anonymous  Noble, p.1510-1515

Intracranial hemorrhage (especially subarachnoid hemorrhage) – acute, severe headache

Sinusitis headache –  unilateral or bilateral nasal obstruction, purulent rhinorrhea, facial pain and pressure overlying the paranasal sinuses.  The pain is worsened by bending over or straining.

Ocular headache ( refraction error/glaucoma) – recurring pains in one or both eyes and forehead.  Need to measure ocular tension.

Temporal arteritis – a mild to moderate constant, unilateral temporal pain that moves between head regions and is associated with local tenderness and redness over the scalp.  Immediate tx is necessary.

Meningitis – headache, stiff neck, low-grade fever, and sterile pleocytosis in the CSF.  The headache is present in all positions.

Posttraumatic headache – the territory of the pain is denoted by marked skin tenderness on gentle pinching.  HX of head trauma!

Lumbar puncture headache – caused by a CSF leak after a LP; headache usually caused when a patient sits or stands up and resolves when the patient lies flat.

Intracranial neoplasm headache – steady dull bursting or pressure sensation felt deep within the head that is not as severe as migraine.  The pain has no rhythm to it but tends to be continuous and is seldom throbbing.  Accompanying sx depend on the growth of lesion.

TMJ dysfunction/bruxism headache of neck origin (cervical radiculopathy) – the pain may be felt in the face and may be misidentified as trigeminal neuralgia.  PE finds TMJ tenderness, asymmetric opening and closing of the mouth, the presence of notable overbite.

VIII. Alterations in Mental State

            1. Define Coma.

Deb/Noble,Section XII,Chap.157

It is a disturbance of consciousness in which pt cannot be aroused by any stimulus no matter how vigorous, with return of any responsiveness, coma ends

sg Current p. 985

Coma: major complication of serious central nervous system.  Can result from seizures, hypothermia, metabolic disturbances or structural lesions.  Patient is unarousable and unable to respond to external events or inner needs, though reflex movements and posturing may be present.

Anonymous Current p. 985.

-Coma – The comatose patient is unarousable and unable to respond to external events or inner needs, although reflex movements and posturing may be present.

-(vs.)Stupor – The stuporous patient is unresponsive except when subjected to repeated vigorous stimuli.

Anonymous  Tabers 458

            A state of unconsciousness from which one cannot be aroused. Coma is the most severe of the alterations of consciousness. It differs from sleep in that a comatose pt will not awaken w/ stimulation; it differs from lethargy, drowsiness, or stupor in that comatose pt is completely unresponsive.

Anonymous

Coma is a disturbance of consciousness in which the patient cannot be aroused by any stimulus, no matter how vigorous. With

the return of any form of responsiveness, coma ends. The recovering patient then progresses through various levels of

disordered consciousness until finally attaining a clear sensorium. An understanding of the pathogenesis is essential to the

clinical management of the comatose patient.

2. Describe the rapid assessment needed to determine the anatomic level of

 dysfunction in a comatose patient.

Deb/Noble,Section XII,Chap.157

State of consciousness, pupils, eye movements, respirations and remaining motor functions.

sg Current p 986

Supportive therapy for respiration or blood pressure is initiated; in hypothermia, all vital signs may be absent, all such pts should be rewarmed before the prognosis is assessed. 

Position pt to keep airway open, labs for chemistry, abg, liver and reanl function tests, tox studies as indicated. Further details include hx, circumstances surrounding onset, time course of subsequent events. Abrupt onset suggests subarachnoid hemorrhage, brain stem stroke, or intracerebral hemorrhage.  Slower onset and progression occur with other structural or mass lesions.  Metabolic cause likely with preceding intoxicated stated or agitated delirium. Behavioral response to painful stimuli, pupil response, eye position and movement in response to passive movement of the head and ice-water caloric stimulation, and respiratory pattern are observed to determine cause of coma and location of lesion.

Anonymous Current p. 986.

-The diagnostic workup of the comatose patient must proceed concomitantly with management.  Supportive therapy for respiration or BP is initiated; in hypothermia, all vital signs may be absent and all such patients should be rewarmed before the prognosis is assessed.

-On exam, attention is paid to:

~the behavioral response to painful stimuli

~the pupils and their response to light

~the position of the eyes and their movement in response to passive movement of the head and ice water caloric stimulation

~the respiratory pattern

Noble p. 1463.

See Noble for the Glasgow coma scale.  Main categories of assessment:

-Motor response to deep pain stimuli

-Pupils

-Extraocular movements

-Respirations

Anonymous  Noble 1463  Use of Glasgow coma scale.

Anonymous

Early management of the comatose patient: The overriding concern in the early management of the comatose patient is the

immediate treatment of any remedial cause of brain damage. Several steps should be taken even before a full diagnostic

assessment is made:

1. ABCs. Assure adequate airway, respiratory exchange, circulation, and metabolic substrate, glucose, and thiamine.

2. Draw blood for labs

3. Give 100mg of thiamin IV

4. Follow that with 50-ml 50% glucose solution IV

5. Naloxone should be administered if narcotic ingestion is suspected.

6. Flumazenil given empirically for pill ingestion as well as suspected benzodiazapine ingestion.

7. Position the patient to both protect cervical spine and prevent aspiration.

8. Once these are all satisfied, continue with a rapid general medical and neurologic examination.

Evaluation of the comatose patient: The neurologic examination of the comatose patient involves assessing for deterioration in

the nervous system function. The Rostral-caudal deterioration refers to the sequential loss of certain functions, beginning with

• the cerebral cortex and followed by

• the diencephalons,

• midbrain,

• pons, and finally

• the medulla.

A rapid assessment of the anatomic level of a given patient can be made by examining the state of consciousness, pupils, eye

movements, respirations, and remaining motor functions.

Major Neuroloic Signs Reflacting Anatomic Levels of Rostral-Caudal Progression in Coma.

3. Describe the two major categories considered in the diagnosis of coma.

Deb/Current,pg.986

Structural lesions (subarachnoid hemorrhage, brain stem stroke, mass) or Metabolic disturbance

sg Current p 986

Stupor and coma due to:

• structural lesions

• Metabolic disturbances.

Anonymous Current p. 986.

-Stupor & coma due to structural lesions

Supratentorial mass lesions tend to affect brain function in an orderly way; symptoms are often progressive.  Subtentorial (brain stem) lesions may cause an early & abrupt disturbance of consciousness w/o an orderly progression of neurologic signs.

-Stupor & coma due to metabolic disturbances

Patients with a metabolic cause of coma generally have signs of patchy, diffuse, and symmetric neurologic involvement that cannot be explained by loss of function at any single level or in a sequential manner.

Anonymous   Noble 1464

            A rapid assessment of the anatomic level of a given patient can be made by examining the state of consciousness, pupils, eye movements, respiration’s, and remaining motor functions.  Examination of extraocular eye movements is another important means of assessing the anatomic level of brain stem involvement.  With coma depending on the level, oculocephalic reflexes may become  overly facile or disappear completely.  The pupils are no longer reactive to light; they tend to be midposition .  As the pons is destroyed, breathing becomes ataxic, irregular, and unpredictable.  At this point respiratory arrest is imminent.

Anonymous  Noble, pg1464

The initial diagnostic consideration is to determine whether the alteration in consciousness is caused by a primarily

--intracranial structural lesion, or a

--systemic toxic or metabolic disorder.

Structural Central Nervous System Lesions: The intercranial processes are subdivided into those in which focal signs are likely

vs. those in which no focal signs may be anticipated. The Major categories of intracranial processes with focal brain

dysfunction include

-trauma,

-intraparenchymal hemorrhages,

-tumors,

-certain forms of infection, and

-brain infarction

The other intracranial processes that cause coma without focal signs are caused by:

-meningitis,

-encephalitis, and

-subarachnoid hemorrhage.

-require CSF examination for diagnosis

Toxic-Metabolic Causes: The differential dianosis of endogenous metabolic derangement is extensive and includes

disturbances in all organ systems.

Exogenous Intoxications General Medical Diseases

Alcohol, barbiturates Disturbances of hydration, electrolytes, osmolarity

Tranquilizers Cardiovascular disturbances

Belladonna derivatives Pulmonary failure

Psychomimetics Hepatic encephalopathy

Others: Salicylates, caffeine, Uremia and the postdialysis syndrome

heavy metals Endocrine disorders: hypoclycemia, thyroid, adrenal

Withdrawal syndromes and pituitary syndromes

Porphyria

Vasculitides

Acute infections outside the CNS

Toxemia of pregnancy

4. Describe the physical and laboratory exams that may be helpful in the

 differential diagnosis of the cause of coma.

Deb/Noble,Section XII,Chap.157

Look for head trauma-raccoon eyes, battle signs, MRI,CT, lumbar puncture (do CT 1^st-avoid risk of cerebral herniation), pupil dilation, light reflex, cheyne-stokes respiration

sg. Current p. 986

Response to painful stimuli, ocular findings and respiratory patterns (Cheyne-Stokes, hyperventilation, apneustic, or atactic breathing). (Ocular findings: pupil response, EOM, oculomotor response to passive head turning (doll’s-head eye response) , oculovestibular reflex testing by caloric stimulation using irrigation with ice-water. Normal subjects-jerk nystagmus response, response perverted and finally disappears with impairment of brain function.) 

Anonymous Current p. 987.

-CT (possibly before or instead of an LP as LP may worsen condition) especially in patients suspected of having structural lesion.

-CSF examination especially in patients suspected to have metabolic disturbance.

-For physical exams, see #2.

-From Noble: look for signs of head trauma (Battle’s sign), MRI, CT, LP, pupil dilation, light reflex, Cheyne-Stokes respiration.

Anonymous  Noble 1463-1464

            1.  A rapid assessment of the anatomic level of a given patient can be made by examining the level of consciousness- evaluated by repeated efforts to wake the patient and gain his or her attention.  

            2.  Examination of the pupils should include size, symmetry, and response to light.  A magnifying glass may be needed to see if the papillary response to light has been preserved.

            3.  Examination of the Extra Ocular Eye Movements is important, as is Conjugate gaze, even before EOM.  Instilling cold water to the external ear canal can induce conjugate deviation of the eyes to the stimulated side.  The maximal stimulus consists of 40ml of ice water instilled over 30 seconds. Doll’s Head maneuver- consists of rapidly turning the head from side to side or flexing and extending the neck.  Conjugate movement of the eyes to the side opposite the direction of the head movement demonstrates that brain stem centers for eye movement are intact.  Once the pons and medulla have been destroyed, the lateral eye movements do not respond to either test.

            4.  Respiratory rate and pattern is assessed.  (See table) Removing mechanical ventilation (apnea test)

            5.  Simple observation of spontaneous movements or the movements in relation to painful stimuli is done.  Sternal rub is often done along with an illiac crest rub is done with the patients arms across the abdomen to see if the patient is reacting.  

            6.  Trying to elicit a oro pharyngeal (gag)  reflex.  

            7.  Confirmatory diagnosis may be made with an EEG, looking for electro cerebral silence, or absence of circulation to the brain.

***The physician must rule out all other possible causes of coma such as, sedative drugs, severe hypothermia, CV disturbances, pulmonary failure, hepatic encephalopathy, uremia and the post dialysis syndrome, endocrine disorders, porphyria, Vasculitides, acute infections outside the CNS, toxemia of pregnancy. ***

Anonymous

Coma must first be determined to be of primarily intracranial structural lesions or a systemic toxic or metabolic disorder.

Intracranial structural lesions include trauma, intraparenchymal hemorrhages, tumors, certain forms of infection, and brain

infarction. These can be diagnosed using the following:

a. Computerized tomography (CT scan)

b. Spinal tap to isolate blood in the CSF

c. Magnetic resonance imaging (MRI)

Physical tests depend on the location of the lesion and include:

a. Dolls head maneuver (p.1464 Noble)

b. Pupilary response (size, symmetry, and response to light)

c. Ice water-caloric test (ice water in the ear to induce deviation of the eyes toward the side of the stimulus. 40ccπs in 30

seconds)

d. Deviation of the eyes toward the side of the lesion.

e. Irregular breathing patterns (cheynes-stokes, apneustic, neurogenic hyperventilation)

f. Decorticate or decerebrate posturing.

g. Failure to respond to deep pain.

5. Define brain death (according to the 1981 President's commission)

Deb/Current,pg.987

It is controversial, in order to establish it, the irreversibly comatose pt must be shown to have lost all brain stem reflex responses, including the papillary, corneal, oculovestibular, oculocephalic, oropharyngeal, and respiratory reflexes, and should have been in this condition for at least 6 hours.

Anonymous Current p. 987.

-The definition of brain death is controversial.  In order to establish brain death, the irreversibly comatose patient must be shown to have lost all brain stem reflex responses, including the papillary, corneal, oculovestibular, oculocephalic, oropharyngeal, and respiratory reflexes, and should have been in this condition for at least 6 hours. 

-Spinal reflex movements do not exclude the diagnosis but ongoing seizure activity or decerebrate or decorticate posturing is not consistent with brain death. 

-The apnea test determines whether patient is capable of respiratory activity.

-Reversible coma simulating brain death may be seen with hypothermia & OD; these conditions must be excluded.

Anonymous  Noble 1467

            Patients are not brain dead if they have reactive pupils, corneal or gag reflexes, or decerebrate or decorticate posturing.  

Anonymous Brain death is defined as:

l. Cessation of all function of the entire brain.

a. Unresponsive coma

b. absent brainstem reflexes

1. Pupillary light reflex

2. Corneal reflex

3. Cephalic (caloric) reflexes

4. Oropharyngeal (gag) reflex

5. Respiration (apnea testing)

ll. Irreversibility

A. Coma of known cause without potential for reversibility

B. Exclusion of contributory, reversible condition

1. Drug intoxication

2. Neuromuscular blockade

3. Hypothermia (<32.2, 90F)

4. Shock

5. Major metabolic disturbance

C. Persistence for an appropriate period (6-12 hours, depending on the cause of coma and local practice)

lll. Confirming investigations (may be optional or required)

A. EEG

B. Absence of circulation to the brain

6. Define delirium (acute confusional state Noble pages 1468-1473).  

Brent CMDT 1059

Delirium (acute confusional state) is a transient global disorder of attention, with clouding of consciousness, usually a result of systemic problems (eg, drugs, hypoxemia).

Jam, CMDT 51

An acute, fluctuating disturbance of consciousness, associated with a change in cognition or the development of perceptual disturbances. It’s a consequence of an underlying medical condition like infection, coronary ischemia, hypoxemia, or metabolic derangements

Anonymous Noble, Current p. 1059.

-Delerium = acute confusion.  Acute confusion can be thought of as an acquired incapacity to think with customary speed and clarity.  The major feature of this syndrome is the failure to maintain normal sequential thought, reflecting an inability to rank the priority of stimuli.  Inability to rank priority of stimuli impairs adaptational interaction with the environment. 

-Delerium (acute confusional state) is a transient global disorder of attention, with clouding of consciousness, usually a result of systemic problems (drugs, hypoxemia, etc.).  Onset is usually rapid.  Mental status fluctuates.

Anonymous  Noble 1468-1473 .

            (DSM IV definition)

            1.  Clouding of consciousness, that is, a reduced capacity to shift, focus, and sustain attention to environmental stimuli

            2.  At least two of the following:  (a) perceptual disturbances (I.e., misinterpretations, illusions, hallucinations), (b) incoherent speech, (c ) disturbances of the  sleep-wake cycle, or (d) increased or decreased psychomotor activity.

            3.  Disorientation and memory disturbance.

            4.  Clinical features that develop over a short period and tend to fluctuate over the course of the day.  

            5.  Elements from the history and physical examination, and laboratory tests that suggest a specific organic factor, judged to be etiologically related to the disturbance.

            Noble’s definition- Acute confusion, also know as acute encephalopathy, acute toxic psychosis, or delirium, can be thought of as an acquired incapacity to think with customary speed and clarity.  The major feature of this syndrome is the failure to maintain, normal, sequential thought, reflecting an inability to rank the priority of stimuli.  Patients thus are unable to maintain a coherent stream of thought.  The consequent failure in the designation of behavioral priorities causes an immediate and drastic disintegration of the adapatational interaction with the environment.

Anonymous  p. 1468-1470 Noble

Also known as acute confusional state, toxic psychosis, and acute encephalopathy.

An acquired incapacity to think with customary speed and clarity. The major feature of

this syndrome is the failure to maintain normal, sequential thought, reflecting an

inability to rank the priority of a stimuli.

7. Describe the common presenting clinical features of delirium.

Brent CMDT 1059

-Rapid onset

-fluctuating mental status (usually least in the AM) with difficulties concentrating, maintaining attention, and sustaining purposeful behavior.

-“Sundowning”: mild-moderate delirium at night

-deficit in short term memory and recall

-anxiety and irritability

-retrograde amnesia (impaired recall of past memories)

-anterograde amnesia (difficulty with recall of events after the onset of delirium)

-disoriented

-perceptual disturbances (often visual hallucinations)

-psychomotor restlessness and insomnia

-autonomic changes (eg, tachycardia, dilated pupils, sweating)

-1 wk duration

Jam, CMDT 1059

•     Rapid onset, fluctuating mental status (impairment is least in the morning), with varying ability to concentrate, maintain attention, and sustain purposeful behavior.

•     Sundowning, mild to moderate delirium at night, is more common in patients with preexisting dementia and may be precipitated by hospitalization, drugs, and sensory deprivation.

•     Anxiety, irritability, psychomotor restlessness and insomnia are common; retrograde and anterograde amnesia

•     Autonomic changes include tachycardia, dilated pupils, and sweating.

Anonymous Current p. 1059.

-clouding of consciousness

-rapid onset

-fluctuating mental status (with least impairment in the morning)

-varying inability to concentrate, maintain attention, sustain purposeful behavior

-marked deficit of short term memory and recall

-anxiety & irritability

-retrograde & anterograde amnesia

-psychomotor restlessness

-insomnia

-autonomic dysfunction (tachycardia, pupil dilation, sweating)

Delerium can co-exist with dementia.  Sundowning – mild to moderate delerium at night – is more common in patients with preexisting dementia.

Delerium alone lasts about a week on average.  Full recovery occurs in most cases.

Anonymous  Goroll p956      

Initially

ü      Slight forgetfulness

ü      Attention and concentration deficits

ü      Increasing repetitiousness or inconsistencies in usual behavior

Later

ü      Increasingly impaired judgment

ü      Inability to abstract or generalize

ü      Personality changes: rigidity, irritability, confusion to minor changes

ü      Affective disorders or Aggressive behavior

ü      Loss of original personality

ü      Unable to maintain personal hygiene, nutrition and are helpless

Anonymous

Attention deficits: Disordered attention, difficulty in maintaining or attaining attention.

Thought disorder: "Unable to think straight," Unable to "get it together".

Perceptual disturbances: Perhaps the most dramatic. Misinterpretation of stimuli, ex: crawling bugs, seeing snakes or wild

animals, sounds – Hallucinations.

Language disturbances: Word-finding difficulty, Misspellings and perseverations.

Disturbance in Sleep-Wake Cycle: Some may be hypersomnolent; others awake for days at a time.

Fluctuations in symptomology: Fluctuations in cognitive function daily or over a 24-hour period.

Arousal: Quiet or withdrawn or just the opposite; hyperaroused with minimal sleep, may thrash about in bed, pacing.

8. Describe the initial laboratory work-up for a patient suffering from delirium.

Note: There are many causes of delirium.  Our main thrust here is that you know the major categories of etiologies. (e.g., injury by physical agents, infections, intoxications, etc.)

Brent CMDT 1061

Labs include serum electrolytes, glucose, BUN, creatinine, LFT’s, thryoid function tests, ABG’s, CBC, Ca, phosphorus, Mg, Vit B₁₂, folate, blood cultures, UA, and CSF analysis.

Jam, CMDT 51

CBC, lytes, BUN, creatinine, glucose, calcium, albumin, liver function studies, UA, and ECG. In some cases, may want magnesium, serum drug levels, ABGs, blood cultures, CXR, and urinary toxin screens.

Anonymous Current p. 1060, Table 25-11

Etiology of delerium and other cognitive disorders

Anonymous  Goroll p 961

The lab test recommended should be individualized based on the pts history and physical and mental status exam results.

1)      CBC w/sed rate

2)      Chem panel (lytes, CA, Albumin, BUN Creatine, Transaminase levels)

3)      Glucose

4)      TSH

5)      VDRL for syphilis

6)      Serum B12 and folate

7)      UA

8)      Chest xray

9)      EKC

10)  CT of head

11)  LFT

Table 169.2 p959 Systemic Conditions Associated with Intellectual Impairment

            1) Infectious

                        Syphilis with CNS involvement

                        HIV infection with CNS involvement

                        Cryptococcal infection of the CNS

            2) Endocrine

                        Hypothyroidism and hyperthyroidism

                        Panhypopituitarism

                        High dose glucocorticoids therapy

            3) Metabolic

                        Vtm B12 deficiency

                        Thiamine deficiency

                        Niacin deficiency (pellagra)

            4) Chemical Poisons

                        Alcohol

                        Metals (lead, mercury)

                        Aniline dyes

            5) Drug Intoxications

                        Barbiturates

                        Opiates

                        Anticholinergic

                        Lithium

                        Bromides

                        Haloperidol

                        Antihypertensives

Anonymous

The lab work-up will be focused according to possible etiology.

Rule out physical injury: X-rays, CTs, etc.

Infections: Lumbar puncture, blood cultures, other

Intoxication: Tox screens, blood alcohol

Metabolic: ABGs, blood glucose, LFTs, BUN, T4, TSH

Vascular disorders: CBC, CTs, EKG

Cerebral degenerative disorders: Test for things like MS

Extracranial lesions or cancers: X-ray, CT

Hypersensitivity and autoimmune disorders: Work up for food allergies or serum sickness.

9. Define dementia.

Brent Taber’s, CMDT 1060

A broad (global) impairment of intellectual function (cognition) that usually is progressive and that interferes with normal social and occupational activities.

Jam, CMDT 48-9, 1060

An acquired persistent and progressive impairment in intellectual function, with compromise in multiple cognitive domains (language impairment, apraxia, agnosia, impaired executive functioning), at least one of which is memory. Deficits must represent a significant decline in function and be severe enough to interfere with work or social life. Onset is insidious over months to years and is rarely reversible.

Anonymous Current p. 1060.

Dementia is characterized by chronicity and deterioration of selective mental functions.  Onset is insidious over months to years (usually).  Dementia is rarely reversible.  Dementia is classified as cortical or subcortical.  Loss of impulse control is common.

Goroll p. 956, Noble p. 1475, Bates p. 121.

Dementia is a syndrome characterized by a generalized and sustained decline in intellectual functioning.  It is progressive, measurable in months to years vs. days or weeks (delerium).  Decline in mental abilities involves memory, cognition & adaptive behavior.

Anonymous  Goroll p956,Noble1475, Bate 121

            Dementia is a syndrome characterized by a generalized and sustained decline in intellectual functioning from a previously attained level. It is characteristically a progressive disorder, measurable in months or years rather than days or weeks. The decline from a previously attained level of mental ability is broad based usually involving memory, cognitive capacities and adaptive behavior without alteration of consciousness.

Anonymous

A syndrome resulting from many etiologies, slow loss of previously acquired intellectual or behavioral function without

alteration in level of awareness. Affects independent daily living.

(Noble pp. 1473 – 1483) –

10. Describe the most common causes of dementia.

Brent CMDT 1060

The most common cause of dementia are neurodegenerative disorders.  Examples include Alzheimer’s dementia (most common) and Pick, Creutzfeldt Jakob, and Huntington dementia (less common).

The 2^nd most common cause is vascular dementia or atherosclerotic (multi-infarct) dementia.  Results from cerebrovascular disease with multiple infarctions.

Jam, CMDT 1060

Primary degenerative dementia (Alzheimer’s most common; also Pick, Creutzfeldt-Jakob, and Huntington) accounts for 50-60% of cases, atherosclerotic (multi-infarct) dementia for 15-20%, mixture of the first 2 types or due to miscellaneous causes 15-20%

Anonymous Current p. 1060.

Cortical Dementia:

-Primary degenerative dementia (eg, Alzheimer’s) accounts for 50-60% of cases.

-Atherosclerotic (multi-infarct) dementia accounts for 15-20% of cases.

-Mixtures of the 2 above & misc. dementias account for 15-20% of cases.

Subcortical Dementia: not detailed in Current.

Goroll says that in patients over 85, vascular dementia and Alzheimer’s disease account for the vast majority of cases.

Anonymous  Goroll p959

Among pts presenting with dementia:

v     Alzheimer’s Dz 65%                

v     Vascular Dz with multiple infarcts-10%-20%

v     Brain tumors-5%

v     Unknown-10%-15%

Among pt >age 85, vascular dementia and Alzheimer’s disease account for the vast majority of cases.

Anonymous

Most causes of dementia lead to the death or metabolic dysfunction of neurons, producing losses in cognitive function. The

location of the cell loss is a critical factor. In Alzheimerπs Disease, the most common cause of devastating progressive

dementia, cell loss is most prominent in the cortex, hippocampus, and amygdala. In multiinfarct dementia, small strokes placed

in strategic locations can lead to the loss of memory, visuospatial abilities, language, and personality. Dementia can also occur

because of a change in the overall chemical milieu of the brain (ie. hypothyroidism and toxic encephalopathies from meds).

These "reversible" dementias are less common but critically important to diagnose.

Diseases associated with dementia: Alzheimerπs Dz, Down Syndrome, Pickπs Dz, Dementia with Lewy bodies, Huntingtonπs

Dz, cerebrovascular dz, Binswangerπs encephalopathy, Vasulitis (ie. Systemic Lupus Erythmatosus), Depression, chronic

schizophrenia, normal-pressure hydrocephalus, nonconvulsive status seizures, infections (ie. syphilis, AIDS), Creutzfeldt-Jakob

Dz, HIV encephalopathy, metabolic disorders (ie. hypo/hyperthyroidism), Vitamin B12 deficiency, Thiamine deficiency (ie.

Wernicke-Korsakoff syndrome), frontal or temporal lobe tumors, trauma, alcohol-related dementia, and toxic causes (ie. Rx

and OTC meds).

11. Formulate a work-up for a patient presenting with dementia.

Greg.  CMDT pp. 48-50.  

Hx:

Historical questions in an evaluation for dementia include those directed at the rate of progression of the deficits, their nature (including any personality or behavioral change), motor problems, risk factors for HIV or syphilis, family history medication list , fxnl disabilities, and degree of social support.

PE:

Neuro exam emphasizes assessment of mental status.  The PE should also focus on identifying aggravating comorbid conditions.

Labs:

Intended to uncover treatable causes of cognitive impairment & include  a CBC, electrolytes, Ca, Creatinine, Glucose, TSH, & Vit B-12 levels.  HIV testing, RPR, and LFT’s may be helpful.

If focal neuro s/s, seizures, gait abnormalities, w/ acute or subacute onset may have positive findings w/ head CT or MRI.

Referral:

Neuropsychologic testing may be helpful in the following circumstances: to distinguish dementia from depression, to dx dementia in persons of very poor education or very high premorbid intellect, & to aid dx when impairment is mild.

Tx:

Alzheimer’s is a common cause of dementia.  So a make the pt/family aware of Alzheimer’s Association for coping and support.  Educate caregivers; be sure to cover burn out/depression and elder abuse.

Tx for cognitive decline:  Acetylcholinesterase inhibitors (may or may not be effective).  A non pharmacologic approach (keep things simple and non confrontational for the pts) often works best.

Tx for depression:  Antidepressants if non pharmacologic tx not helpful.

Tx for hallucinations/delusions:  ranges from low potency anti psychotics to anticholinergics.

Paul, CDMT pg 48-50

History:  Cognitive and behavioral changes and rate of progression, family history, risk factors for HIV or syphilis, medication list, functional disabilities, and depress of social support.

PE:  Neurologic examination emphasizes assessment of mental status.  The remainder should focus on identifying comorbid conditions that may aggravate the individual’s disability.

Laboratory studies:  For most patients, intended to uncover treatable causes of cognitive impairment and include a CBC, electrolytes, calcium, creatinine, glucose, TSH and vitamin B12.  HIV testing, RPR, liver function studies may be informative in selected patients.  

Radiological studies:  Area of controversy.  Head CT or MRI may benefit those with focal neurologic signs or symptoms, seizures, gait abnormalities, and an acute or subacute onset.

Referral:  neuropsychologic testing may be helpful in the following: to distinguish dementia from depression, to diagnose dementia in persons of very poor education or very high premorbid intellect, and to aid diagnosis when impairment is mild.

Treatment:  Alzheimer’s association and other helpful publications.  Education.  Acetylcholinesterase inhibitors produce statistically significant but clinically modest improvements in cognitive function (still undetermined in Alzheimer’s dementia).  Teach caregivers to use simple commands and language.  Pharmacologic approach to depression, anxiety, or psychosis may be helpful.

Anonymous Bates p. 122, Noble p. 1473.

Currently there are no reliable screening tests to detect dementia early on.  Watch for changes in cognition, ADLs and family complaints about new/unusual behaviors.  Use the MMSE.  Once cognitive change is identified, look into meds, depression, metabolic abnormalities.  Counsel families.  Warn of potential for disruptive behavior, falls/accidents, impaired driving.  Advise re: power of attorney & advanced directives.

Anonymous  Bates, p. 122 & Noble, p. 1473-74

Dementia often has a slow, insidious onset and may escape detection by both families and clinicians, especially in its initial stages.  Currently, there are no reliable screening tests to help you detect dementia early in its course.  Clinicians should be alert to evidence of change in cognitive function or activities of daily living, and to family complaints about new and unusual patient behaviors.  Use of the Mini-Mental Status Examination is helpful for assessing cognitive impairment.  Once cognitive change is identified , be sure to address the possible role of medications, depression, or metabolic abnormalities.  For demented patients and affected families, counseling about the potential for disruptive behavior, accidents and falls, and termination of driving privileges is warranted.  Clinicians can foster discussion of legal matters such as power of attorney and advanced directives while the patient is still able to contribute to decision-making.

It is critically important to obtain a history from family members or the caregiver.  Included should be questions about ADL’s (eating, dressing, toileting, etc.).  There are standardized questions to assist in the history-taking (The Blessed dementia scale, p. 1474 in Noble).  All medications including over-the-counter meds., folk remedies, alcohol, and street drugs should be reviewed.  Any change in consumption of alcohol noted, because it could be self-treatment for depression that goes unnoticed by the family.   The medical review of systems should focus on a history of hypertension, diabetes, thyroid disease, Vit. B12 deficiency (gastric surgery??),  syphilis, and  AIDS (for the acquired dementia assoc. with AIDS).  A family history is vital for certain diagnoses such as Huntington’s disease and familial AD (alzheimers).    Any history or family history of psychiatric illnesses in the patient or other family members should be recorded.  The patient’s occupational and educational history allows an estimation of premorbid cognitive functioning.

Included with a general exam are cardiac and thyroid exams, plus evaluation for carotid bruits, orthostatic blood pressures.  Because of incorrect answers, “covering” of memory lapses, and overreliance on empty social comments, open-ended discussion with the patient often suggests a diagnosis of dementia..  The Folstein minimental status exam and the Blessed Dementia scale include scales of information, memory, concentration, language, and visuospatial abilities.  Testing for apraxia, vision, and hearing are also important.  The neurologic exam should then focus on finding focal abnormalities, hemiparesis, reflex symmetries, and sensory changes could be indicative of strokes or mass lesions.

Differential Diagnosis:  Alzheimer’s Disease (including familial); Neurodegenerative diseases (MS, Pick’s disease, Parkinson’s, & MS); Cerbrovascular Disease (diabetes and HTN can be causal agents for strokes that impair cognitive function);  Vasculitis (SLE, sarcoidosis, AIDS); Depression and Psychiatric disorders (pseudodementia, depression assoc. w/ AD, and chronic schizophrenia); Normal-Pressure Hydorcephalus, Nonconvulsive Seizures; Infections (Syphylis, AIDS, Creutzfeldt-Jakob disease, temporal lobe abscesses, chronic meningitides-cryptococcal, HIV encephalopathy); metabolic diseases (thyroid, Vit. B12 deficiency, thiamine deficiency); tumors, trauma, alcohol-related dementia; toxic causes (OTC or Rx’d meds., herbal or folk remedies); and sensory deprivations (hearing and visual acuity losses).

Labs:CBC w/ diff.;  TSH; B12 level; VDRL; LFT’s;, and UA.  Possibly:  ESR, other hormone levels, ABG’s, antinuclear antibody testing, LP with appearance, cell count, protein, glucose levels, a VDRL, cryptococcal antigen,a dn cultures.  A CT or an MRI scan of the head should be done at the initial diagnosis and then repeated with any future deterioration.  An EEG could be considered.  Neuropshchologic testing as indicated or for ruling out.

Anonymous

Hx: Obtain pt history from the caregiver and the pt. Ask questions about: changes in behavior, difficulties with activities of

daily living (eating, dressing), meds, alcohol/drug use, PMH (of HTN, DM, thyroid dz, vit B12 deficiency, syphilis, HIV,

psych illnesses), FH (of dementia, psych illnesses), ptπs educational and occupational hx.

PE and Neuro exam: general med exam including cardiac and thyroid exams, check for carotid bruits and orthostatic BPs for

vascular risk factors, mental status exam, Blessed Dementia Scale (example Noble p. 1474), test for apraxia (inability to perform

purposeful movements without sensory or motor impairment), test vision and hearing, check for focal abnormalities (ie.

hemiparesis, reflex asymmetry, sensory changes) using DTRs, sensory perception, and motor strength. Further neuro consult

if needed.

12. Compare and contrast the common presentations of delirium and dementia.

Greg  Noble p. 1473

Dementia is a slow loss of previously acquired intellectual or behavioral function w/o alteration in level of awareness.  At least two areas of cognitive function, such as memory, language, personality, visuospatial abilities, or judgment, are impaired in dementia, and the disabilities affect independent daily living.  This is not to be confused w/ memory loss that naturally occurs w/ aging.

Delirium (an acute confusional state) is an acute or subacute onset of disorientation w/ alterations in levels of awareness, including hyperalert and drowsy states.  Note that delirium may coexist w/ dementia.

Paul, Noble pg 1743, CDMT pg 51

Dementia:  A syndrome resulting from many etiologies is a slow loss of previously acquired intellectual or behavioral function without alteration in level of awareness.  At least two areas of cognitive function, such as memory, language, personality, visuospatial abilities, or judgment are impaired in dementia, and the disabilities affect independent daily living.  A common disorder in elderly persons, occurring in all age groups.

Delirium:  An acute confusional state is an acute or subacute onset of disorientation with alterations in levels of awareness including hyperalert and drowsy states.  It often disrupts day/night cycles and can be accompanied by delusions.  Delirium may coexist with dementia.  It is the pathophysiologic consequence of an underlying general medical condition such as infection, coronary ischemia, hypoxemia, or metabolic derangement.  Most episodes of delirium clear in a amatter of days after correction of the precipitant, but some patients suffer episodes of longer duration and need to have follow-up for the development of dementia.

Anonymous Current p. 48.

Please read Answers to #6, 7, 9.

Noble p. 1468.

The two conditions are similar in that they both involve a diffuse impairment of intellectual functioning.  The delirious patient has what appears to be a specific disturbance of consciousness but dementia occurs in the context of a clear sensorium.  The nature of onset and course of illness are the most valuable in differentiating delerium and dementia.  Dementia is usually insidious in onset with progressive deterioration.  Delerium usually begins abruptly and shows little progression.  Delerium is relatively short lived.  Most cases of dementia are not; they usually do not improve but worsen over time.

Anonymous  Noble 1468 & 1472 .

Acute confusion, also known as acute encephalopathy, acute toxic psychosis, or delirium, can be thought of as an acquired incapacity to think with customary speed and clarity. The major feature of this syndrome is the failure to maintain normal, sequential thought, reflecting an inability to rank the priority of stimuli. Patients thus are unable to maintain a coherent stream of thought. The consequent failure in the designation of behavioral priorities causes an immediate and drastic disintegration of the adaptational interaction with the environment. Patients' general behavior and speech reflect an inappropriate sequencing of ideas, and patients exhibit a wide range of abnormal behaviors, including assaultiveness, motor hyperactivity, hallucinations, somnolence, and extreme states of panic or fear.

Dementia is an important consideration in the differential diagnosis of confusional states (delirium). The two conditions are similar in that they both involve a diffuse impairment of intellectual functioning. The confused patient, however, has what appears to be a specific disturbance of consciousness, whereas dementia occurs in the context of a "clear sensorium." The nature of onset and course of the illness are most valuable in separating these two conditions. Dementia is usually insidious in onset with a progressive deterioration, whereas confusion usually begins abruptly and shows little progression. Confusional states are relatively short-lived. If treated appropriately, and in some patients even if left untreated appropriately, spontaneous clearing may occur. Most cases of dementia, on the other hand, do not improve unless a specific treatable cause exists. Other features that help differentiate the two conditions are (1) fluctuation in symptoms, (2) disruption of the sleep-wake cycle, and (3) autonomic overactivity. These features, if present, usually favor a diagnosis of acute confusion. Periods of acute confusion, however, may be noted in the course of dementia. Dementia sometimes becomes much worse, and a stable dementia may exhibit a sudden deterioration. Frequently, this deterioration in the mental status is the result of a superimposed confusional state for which a reversible cause may be identified and corrected.

Anonymous

Dementia is a slow loss of previously acquired intellectual or behavioral function without alteration in level of awareness. At

least two of the following are impaired and affect independent daily living in dementia: memory, language, personality,

visuospatial abilities, or judgment. Common in elderly.

Delirium, or an acute confusional state, is an acute or subacute onset of disorientation with alterations in levels of awareness,

including hyperalert and drowsy states. It often disrupts day/night cycles and can be accompanied by delusions. Delirium

may coexist with dementia.

13. Given a patient presentation be able to differentiate between delirium and dementia.

Greg See Above

Anonymous  Noble 1473

Delirium , an acute confusional state, is defined as an acute or subacute onset of disorientation with alterations in levels of awareness, including hyperalert and drowsy states.  It often distrupts day/night cycles and can be accompanied by delusions.  Delirium may coexist with dementia.

Dementia is a syndrome with multiple causes. It is characterized by a slow loss of previously acquired intellectual or behavioral function without alteration in level of awareness. At least two areas of cognitive function ( memory, language, personality, visuospatial abilities, judgement) are impaired and the disabilities affect independent daily living.

Anonymous

Use Obj #12 as a guide.