Huntington's disease: update and review of neuropsychiatric aspects.
OBJECTIVE
This article presents a general update on Huntington's disease (HD) and reviews the psychiatric and cognitive features of this disorder.


METHODS
HD is discussed in five sections: an introduction and update, the psychiatric aspects, the cognitive aspects, brain-behavior relationships, and the differential diagnosis and management.


RESULTS
Recent advancements in HD include the identification of presymptomatic testing methods and HD gene defect, structural and metabolic neuroimaging findings, and a neuropsychological profile. HD is associated with mood disorders, personality changes, irritable and explosive behavior, a schizophrenia-like illness, suicidal behavior, sexuality changes, and specific cognitive deficits.


CONCLUSIONS
HD results in organic mental disorders from dysfunction of prefrontal-subcortical circuits coursing through the caudate nuclei. The diagnosis of HD is aided by genetic testing, neuroimaging, and neuropsychological testing. Management involves education, genetic counseling and psychotropic medications. Finally, the future of HD holds promise for the development of rational, neurobiologically-based treatments and genetically engineered therapies.
Mendez
M F
MF
Department of Neurology St. Paul-Ramsey Medical Center, MN 55101.
D000328:Adult; D000368:Aged; D003704:Dementia / Q000175:diagnosis* / Q000523:psychology; D005260:Female; D006801:Humans; D006816:Huntington Disease / Q000175:diagnosis* / Q000523:psychology; D008297:Male; D008875:Middle Aged; D019965:Neurocognitive Disorders / Q000175:diagnosis* / Q000523:psychology; D009483:Neuropsychological Tests