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* Huntington's Disease
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J Neuropsychiatry Clin Neurosci 10:249-266, August 1998
© 1998 American Psychiatric Press, Inc.


Special Article

Neuropsychiatric Correlates and Treatment of Lenticulostriatal Diseases

A Review of the Literature and Overview of Research Opportunities in Huntington's, Wilson's, and Fahr's Diseases

A Report of the ANPA Committee on Research , Edward C. Lauterbach, M.D.; , Jeffrey L. Cummings, M.D.; , James Duffy, M.B., Ch.B.; , C. Edward Coffey, M.D.; , Daniel Kaufer, M.D.; , Mark Lovell, Ph.D.; , Paul Malloy, Ph.D.; , Alison Reeve, M.D.; , Donald R. Royall, M.D.; , Teresa A. Rummans, M.D.; and Stephen P. Salloway, M.D.

Accepted April 3, 1998. From the American Neuropsychiatric Association (ANPA) Committee on Research. Address correspondence to Dr. Lauterbach, Division of Adult and Geriatric Psychiatry, Department of Psychiatry and Behavioral Sciences, Mercer University School of Medicine, 1550 College Street, Macon, GA 31207.

This report reviews clinical neuropsychiatric findings and opportunities for research in Huntington's, Wilson's, and Fahr's diseases. Consistent, systematic methodology is lacking among neuropsychiatric studies in these lenticulostriatal diseases. Systematic cross-sectional and longitudinal assessments are needed to ascertain the prevalence of psychiatric disorders as a function of disease course. Preliminary synthesis of existing data suggests the following heuristic relationships in these diseases: depression with parkinsonian states; personality changes with caudate or putamen disease; psychosis, impulsivity, and sexual disorders with caudate disease; dementia and mania with caudate and pallidal diseases; and compulsions with pallidal disease. Correlation of neuropsychiatric findings with disease stage, clinical signs, and radiologic, metabolic, physiologic, and pathologic markers of disease will add to our understanding of these conditions.

Key Words: Huntington's Disease • Wilson's Disease • Fahr's Disease




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