Objectives. To characterize the dementing and neuropsychiatric disorders specific to Huntington 's disease (HD), and illustrate them by a case report of a 34-year-old woman with an early onset HD, hospitalized three times in the Psychiatry Department.
Review. Huntington 's disease is a progressive degenerative disease of the CNS associated with motor impairment, dementia and mental disorders. The diagnosis is based on genetic tests, neuroimaging and neuropsychiatric examinations. HD is related to a number of abnormalities: mood and personality disorders, irritability and explosive behaviors, schizophreniform psychotic disorders, suicidal tendencies, and specific cognitive dysfunctions.
Conclusions. Dementia in HD differs from that present in other degenerative diseases of the CNS. Mental disorders may be a first signal of HD onset.