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March 1978

Huntington's DementiaClinical and Neuropsychological Features

Author Affiliations

From the Section on Experimental Therapeutics, Laboratory of Clinical Science (Drs Caine and Ebert), and the Laboratory of Psychology and Psychopathology (Dr Weingartner), National Institute of Mental Health, Bethesda, Md; and the Department of Child Psychiatry, University of California, Los Angeles (Dr Hunt).

Arch Gen Psychiatry. 1978;35(3):377-384. 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.