HEADACHE


Definitions and classification
History relevant to diagnosis
Physical exam
Diagnostic evaluation
Treatment

I. Definitions and classification

  1. Traditional dichotomy between "vascular" (migraine, cluster) and "muscular" (tension) may not have a pathophysiologic basis
  1. Many pts with migraine lack classic features (unilaterality, throbbing HA with aura, nausea, and pos. family hx)
  2. Many pts with tension HA have migrainous features, e.g. nausea
  3. Muscle contraction is no greater in pts with "tension" HA than nl controls (Handbook of Clinical Neurology, 1986, cited in Bowen handout)
  4. As many as 85% of pts have sx c/w both types of HA (Bowen handout)
  5. No clear relation between change in blood vessel diameter and headache
  1. International Headache Society classification scheme (see references below)
    1. Migraine
  1. Slightly more common in women than men
  2. 75% of women with migraine improve with onset of menopause
  3. Pregnancy is usually associated with improvement and only very rarely ass'd with onset of migraine, so apparent migraine presenting during pregnancy should prompt a fairly thorough evaluation for other causes
  4. Reported precipitants: air pollution, perfumes, hypoglycemia, oversleeping, fatigue, chocolate, cheese, alcohol, MSG, missing meals, meds (nifedipine, nitrates, estrogens, H2 blockers)
  5. Autonomic symptoms may occur with migraine, e.g. tearing, redness of eyes, eyelid edema, nasal congestion, and/or rhinorrhe
  6. Aura-See below; Gradual and/or evolving presentation of aura symptoms help to distinguish them from those related to an ischemic event, which are typically sudden-onset and not evolving.
  7. Associated with increased risk of CVA in women, more so fore women with migraine with aura (RR 2.27 compared to non-migraineurs) than migraine without aura (RR 2.27 compared to non-migraineurs)
  8. "Complicated" migraine (prodromal neurologic sx last for entire HA and may even leave permanent residuae); must exclude serious nerulogic disorders, e.g. tumors, before making diagnosis
    1. Ophthalmoplegic--rare, most common in children & adolescents; CN III most commonly involved; may get permanent ophthalmoplegia
    2. Hemiplegic--unilateral motor and/or sensory sx that outlast hte HA; us recover over days-weeks; 4-18% have a family Hx; OCP's may exacerbate
    3. Basilar artery--most common in young women & kids; sx include vertigo, diplopia, MS changes, tinnitus, and ataxia.
International Headache Society (IHS)* diagnostic criteria for "Migraine without aura"
(previously referred to as "common migraine"):
  • Duration 4-72h
  • Has at least 2 of the following characteristics:
    1. Unilateral location
    2. Pulsating quality
    3. Moderate or severe intensity
    4. Aggravation by routine physical activity
  • During HA, at least one of the following occurs:
    1. Nausea and/or vomiting
    2. Photophobia and phonophobia
  • At least 5 attacks occur fulfilling above criteria
  • Hx/Px (including neurologic Px) do not suggest any other cause

 

IHS* diagnostic criteria for "Migraine with aura" (previously referred to as "classic migraine"):
  • Aura (fully reversible neurologic symptom) meeting description below and without any motor weakness:
    • Visual symptoms (scintillomata, scotomata, spots/lines, and/or loss of vision)
    • Somatosensory symtpoms (e.g. paresthesias, numbness)
    • Dysphasia
  • 2 or more of the following:
    • Homonymous visual symptoms and/or unilateral sensory symptoms
    • Development of aura symptom(s) gradually or in sequence over at least 5min
    • Duration of aura symptom(s) from 5-60min
  • At least 2 attacks occur fulfilling these criteria
  • Hx/Px (including neurologic Px) do not suggest any other cause
  • Note that headache is not mandatory for the diagnosis, but in most cases a headache begins as the aura resolves.
  1. Cluster headache, a.k.a. "histamine headache" & "migrainous neuralgia"
  1. Named because they "cluster" in time, e.g. occurring daily for weeks-months followed by remission for months-years; in some pts they occur chronically without remission
  2. However, in a cross-sectional study of 230 pts meeting IHS diagnostic criteria for cluster HA, 21% had chronic headaches, i.e. NOT clustered in time (Neurol. 58:354, 2002--JW)
  3. Pain is usually unilateral; on right side in 60% of patients; can shift sides but not common
  4. Male:Female prevalence 5:1
  5. Tend to occur at same time each day
  6. Risk factors
    1. Alcohol use
    2. Tobacco use
    3. Sleep apnea
    4. Use of vasodilatory medications
    5. Family history of headaches
    6. History of head injury
  7. Typical precipitants: sleep deprivation, EtOH, altitude, overexercise, NTG, nifedipine
  8. May have history of migraine and some migraine-like symptoms with cluster headaches (aura, photophobia, etc.)
IHS* diagnostic criteria for "Cluster Headache":
  • Severe unilateral orbital, supraorbital, and/or temporal pain lasting 15-180min if untreated
  • Frequency of occurrence from QOD to 8/d
  • Ass'd w/ 1 or more of the following signs on the pain side:
    1. Conjunctival injection
    2. Lacrimation
    3. Nasal congestion
    4. Rhinorrhea
    5. Forehead and facial sweating
    6. Miosis
    7. Ptosis
    8. Eyelid edema
    9. A sense of restlessness or agitation
  • At least 5 attacks fulfilling these criteria
  • Hx/Px (including neurologic Px) do not suggest any other cause
  • "Episodic cluster headache": at least 2 periods of HA lasting from 7d-1y, separated by remissions of at least 1 month
  • "Chronic cluster headache": attacks for > 1y with no remission for 1mo or longer
  1. Tension-type headache
  1. Unilateral in 10-20% of pts
  2. Can be accompanied by jaw discomfort, neck pain, tender spots, or sharply localized nodules in cervical or pericranial mm.
IHS* diagnostic criteria for "Episodic Tension-Type Headache"
  • Headache lasting 30min-7d
  • At least 2 of the following:
    1. Pressing/tightening quality
    2. Mild-mod intensity
    3. Bilateral location
    4. No aggravation by routine physical activity
  • No nausea or vomiting
  • Can have either but not both of photophobia or phonophobia
  • At least 10 HA's meeting above criteria
  • # of headache days < 180/yr, < 15/mo
  • Hx/Px (including neurologic Px) do not suggest any other cause

 

IHS* diagnostic criteria for "Chronic Tension-Type Headache"
  • Average headache frequency 15 or more days/mo for 3mos or more
  • Lasts hours or may be continuous
  • At least 2 of the following pain characteristics:
    1. Pressing/tightening quality
    2. Mild-mod intensity
    3. Bilateral location
    4. No aggravation by routine physical activity
  • No vomiting
  • No more than one of the following: nausea, photophobia, or phonophobia
  • Hx/Px (including neurologic Px) do not suggest any other cause
  1.  Medication overuse headache
    1. aka "Analgesic rebound headache"
    2. Characterized by a pattern of frequent use of abortive headache medications with gradually worsening overall headache condition.
    3. Click here for information on treatment
IHS* diagnostic criteria for "Medication-Overuse Headache"
  • Regular overuse** of one or more headache treatments for over three months
  • Onset or worsening of headache during medication overuse
  • Reversion of headache to prior pattern within 2mo after discontinuation of overuse medications
  • Headache as above present on 15 or more days/month

**-Medication-specific overuse thresholds:
  • Triptans: > 9 days/month
  • Ergotamines: > 9 days/month
  • Opioids: > 9 days/month
  • Simple analgesics: > 14 days/month
  • Or...oversue of any combination of the above > 15 days/month

 

  1. Alternative, practical classification scheme (handout from James Bowen, MD circa 1995)-focus on ruling out non-idiopathic causes
  1. Idiopathic (tension, migraine, cluster)
  2. Medication Overuse Headache
  3. Neck: DJD, C2-3 neuralgia (occipital nerve)
  4. Intracranial
  1. Brain
    1. Hemorrhage
    2. Infection
    3. Tumors
      1. About 50-60% will have HA, but HA will be presenting sx in only about 20%
      2. Early dx generally does not influence outcome of primary brain tumors
      3. us. tension-type but migraine may occur; us. progressive, worst in am though us. does not awaken from sleep; in about 25% is worse with maneuvers that increase ICP (sexual activity, stooping, coughing, sneezing, laughing, straining at stool)
  2. Vascular
  1. Venous occlusion--can occur w/o focal neurologic findings, esp. if the affected vessel is the superior sagittal sinus
  2. Aneurysm--note that unruptured aneurysms don't usually cause pain
  3. HA is common in CVA, particularly when intracerebral hemorrhage is present, often ipsilateral to the CVA
  1. "Benign intracranial hypertension," aka "pseudotumor cerebri"
    1. Presents with headache, often with tinnitus and nausea/vomiting
    2. Usually young, overweight female
    3. Increased ICP of unknown cause
    4. Get progressive HA's often accompanied by blurred vision, horizontal diplopia, and (uncommonly), visual loss
    5. Papilledema and/or loss of venous pulsation is seen in 90%
    6. Head CT usually normal or can show small ventricles
    7. LP (after screening CT) shows high opening pressure (>200mm H2O in nonobese pt or 250mm H2O in obese pt); CSF protein levels may be low
    8. Associated w/certain meds (OCPs, tetracycline, vitamin A)
    9. Management options include corticosteroids, therapeutic LP, and lumboperitoneal shunt
  1. Meningeal
    1. Tumor
    2. Infection
    3. Subdural hemorrhage
    4. Subarachnoid hemorrhage
      1. Presents with sudden-onset severe, continuous HA
      2. Sometimes preceded by a severe but very brief "sentinel headache"
      3. Often precipitated by activities such as sexual intercourse, exertion, coughing, or sneezing, can be localized or generalized
      4. Often accompanied by decreased LOC and/or neck stiffness, ophthalmoplegia or other focal neurologic signs, or papilledema
      5. Dx is by CT w/o contrast (sensitivity decreases w/time after onset) or LP
  1. Extracranial
  1. Trauma, abscesses
  2. Bone (Pagets, tumors)
  3. Giant cell arteritis
  4. Trigeminal or glossopharyngeal neuralgia
  5. Glaucoma
  6. Sinusitis
  7. Teeth
  8. TMJ (causes unilateral pain in TMJ, not headaches)
  9. Carotid artery dissection
  10. Ears
  1. Toxin: CO, MSG, nitrates ("hot dog headaches"), medications
  2. Ophthalmologic (eyestrain, possible secondary to cervical mm. tension; refractive errors not thought to cause HA)
  3. Post-traumatic (30-50% of pts with head injury will develop chronic HA; also dizziness, irritability, fatigue, insomnia, impaired concentration; us. all goes away in a matter of 6-18 months)

II. History relevant to diagnosis

  1. Temporal pattern
    1. Onset: sudden (suggests infection, SAH, tumor) vs. gradual
    2. Frequency/duration (clustering?)
    3. Progression in freq/severity suggests increased ICP
    4. Seasonal pattern?
  2. Precipitants:
    1. Certain foods (chocolate, coffee, cheese, nitrites, MSG)
    2. Odors
    3. Caffeine withdrawal
    4. Trauma
    5. Change in sleep pattern
    6. Menses
    7. Stress
  1. Consistency of Presentation: us. is consistent
  2. Location: unilateral? Upper face (cluster) vs. lower face (trigeminal neuralgia)
  3. Character of pain: "throbbing/pulsating" for migraine, "stabbing/burning" for cluster, "pressing/dull" for tension (to a point)
  4. Accompanying sx: throbbing, nau/vom, photophobia, sonophobia are us. considered signs of migraine
  5. Prodromal sx: scotomata (blind spots), photopsia (flashing lights), vertigo, malaise, pallor, perioral numbness, confusion, aphasia, ataxia, paresis, loss of consciousness
  6. Motion Sickness in general? : found in 45% of migraine cases
  7. Recent trauma
  8. Head/neck surgery
  9. Appropriate ROS
  10. Family Hx: Thought to be typical for migraine
  11. Current use of abortive/prophylactic tx--using them appropriately?
  12. Use of other meds, e.g. exogenous estrogens, nitrates and antihypertensive, that may cause/worsen HA's
  13. Psychosocial hx, inc. stressors & substance use, including caffeine
  14. Occupational hx & exposure to fumes, e.g. Carbon Monoxide; time-relation of sx to work/hobbies

III. Physical exam

  1. Sinuses & nares for evidence of sinusitis
  2. Eyes for evidence of cluster (see below)
  3. Palpate temporal aa.
  4. Palpation of face for hyperesthesia
  5. Palpation of masseters and TMJ for TMJ dysfunction
  6. Fundoscopy for papilledema, optic atrophy, venous pulsations (absence suggests increased ICP_
  7. Head & neck for bruits
  8. Neck ROM for spinal or meningeal disorders
  9. Complete neurologic exam inc. cerebellar function
  10. Mood & affect
  11. Degree of functional impairment--unable to do tasks at home/work b/c of HA's?

IV. Diagnostic evaluation

  1. Neuro exam
  2. Listen for cranial bruits, a sign of tumor
  3. Examine head for focal tenderness
  4. Eyes for glaucoma, papilledema
  5. ENT and neck exam
  6. Endocrine exam for pituitary adenoma (galactorrhea, Cushing's)
  7. Diagnostic studies
  1. ESR if over 55
  2. CBC if infection suspected
  3. Indicators of potential abnormal findings on neuro-imaging (per Kumar & Cooney and other sources):
  1. Recent, substantial change in frequency/severity of HA's
  2. Progressive worsening HA despite appropriate therapy
  3. HA during sleep or on waking (except in cluster HA where that is common)
  4. Sx/signs suggesting focal neurologic process, increased ICP, or meningitis
  5. Onset of HA w/exertion, cough, or sexual activity
  6. Onset of HA at > 40yo
  7. Orbital or cranial bruit
  8. Unusually prolonged or persistent aura
  9. Migraine associated with confusion
  10. Aura without headache
  11. Migraine onset after trauma
  12. Chronic daily headache
  13. Cognitive or personality changes
  1. American Academy of Neurology practice parameters for imaging in patients with migraine (Neurology 55:754, 2000):
    1. Factors associated with significantly increased likelihood of a significant abnormality on neuroimaging:
      1. Rapid increase in headache frequency
      2. History of lack of motor coordination
      3. History of localized neurologic signs
      4. History of headache causing awakening from sleep
    2. Consider neuroimaging if:
      1. Unexplained abnormal neurologic exam finding
      2. Headache features that are atypical or do not fulfill strict definition of a primary headache disorder
    3. Neuroimaging is "not usually warranted" with migraine and a normal neurologic examination
  2. US Headache Consortium (14-member group that includes Am. Acad. Neurol., AAFP, ACP-ASIM, etc.) reviewed published data and recommend neuroimaging studies in the following instances for patients with nonacute headache:
    1. Unexplained abnormal finding on neuro exam
    2. Neurologic sx--no recommendation for or against neuroimaging based on presence of neurologic sx, though they acknowledge that sig. intracranial pathology is more likely in:
      1. New-onset HA in older pts
      2. HA worse w/valsalva
      3. HA that awakens the pt
      4. Progressively worsening HA (intensity or frequency
      5. Dizziness or lack of coordination
      6. Numbness/paresthesias
      7. History of HA awakening pt from sleep
    3. Neuroimaging is not recommended in pts with migraine or tension-type HA and no abnormalities on neuro exam
  1. Consider MRI if suspect posterior fossa lesions or pts w/new onset of cough & exertional HA raising suspicion of craniospinal abnormalities like Arnold-Chiari malformation
  2. Consider lumbar puncture if suspect infection or SAH (if there is meningismus, fever, or pain is related to exertion)
  3. Consider carbon monoxide level in very cold weather headaches or epidemic headaches in people who live together

*--Headache Classification Subcommittee of the International Headache Society. The International Classification of Headache Disorders, 2nd ed. Cephalalgia 24 suppl. 1:1, 2004.

(Sources include Core Content Review of Family Medicine, 2012)