Huntington's dementia. Clinical and neuropsychological features

Arch Gen Psychiatry. 1978 Mar;35(3):377-84. doi: 10.1001/archpsyc.1978.01770270127013.


The neuropsychiatric syndrome of Huntington's disease is outlined in this report with an emphasis on the cognitive deficits that lend themselves to future neurobehavioral research. Eighteen patients without disabling cognitive or psychiatric symptoms were evaluated for a period of 3 to 15 weeks, with assessment of their cognitive disorder, psychiatric, and neurological symptoms. Neuropsychological examination included repeated mental status examination, the Wechsler Adult Intelligence Scale (WAIS), and, for some, parietal lobe testing. In addition to suffering from a loss of finely detailed memories, patients demonstrated impaired organizing, sequencing, planning, and recalling of information on request. On the WAIS, mean verbal and performance scores were not significantly different. Neuropsychological findings suggested that the Huntington's disease pattern of cognitive impairment is not initially diffuse and homogeneous, but characterized by a relative sparing of several higher cortical functions. Many patients had increased irritability and labile affect. The similarity of Huntington's disease to frontal lobe syndromes is also discussed.

MeSH terms

  • Adult
  • Anger
  • Antidepressive Agents, Tricyclic / therapeutic use
  • Cognition Disorders / etiology
  • Decision Making
  • Dementia / etiology*
  • Female
  • Humans
  • Huntington Disease / complications*
  • Huntington Disease / drug therapy
  • Huntington Disease / physiopathology
  • Male
  • Memory Disorders / etiology
  • Middle Aged
  • Parietal Lobe / physiopathology
  • Psychiatric Status Rating Scales
  • Psychological Tests
  • Wechsler Scales


  • Antidepressive Agents, Tricyclic