HUNTINGTON'S DISEASE


Tx of Huntington's:

Neuropsychiatric features of Huntington's

  1. Depression
    1. Prevalence of major mood disturbances 30-40%; 6% die by suicide, usually early during the course of illness
    2. Depression may precede overt movement abnormalities
    3. Generally responsive to conventional antidepressant tx
  1. Psychoses
    1. Prevalence 10-30%
    2. Almost exclusively "positive" psychoses, i.e. delusions & hallucinations
    3. May appear before overt movement abnormalities
    4. Generally responsive to conventional antipsychotic tx
  1. Personality changes--2 overlapping syndromes
    1. Apathetic: slowness, disinterest in usual activities, hygienic neglect, social avoidance
    2. Disinhibited: Irritability, angry outbursts, occasionally, violent behavior
  1. Neuropsychological deficits
    1. Progressive cognitive decline is almost universal, with onset often preceding overt movement abnormalities
    2. "As the dementia advances, patients reach the point at which they are nearly mute, akinetic, globally disinterested, unable to recognize family members and friends, and incapable of managing their activities of daily living."

(Source: Comprehensive Textbook of Psychiatry, 6th Ed., 1995)