Huntington's Disease

Francis O. Walker

doi:10.1055/s-2007-971176

Seminars in Neurology, Table of Contents

Semin Neurol 2007; 27(2): 143-150
DOI: 10.1055/s-2007-971176

Huntington's Disease

Francis O. Walker¹

¹Department of Neurology, Wake Forest University School of Medicine, Winston-Salem, North Carolina

Abstract

ABSTRACT

Huntington's disease may present at any age, but most typically manifests between the ages of 35 and 45 years as a slowly progressive neurodegenerative movement disorder with cognitive and behavioral impairment. It is an autosomal-dominant disorder that has a substantial impact on family structure and dynamics in terms of providing care for affected family members and, for the offspring of an affected parent, dealing with at-risk status. Therapy that slows the progressive neuronal dysfunction or degeneration is unavailable, so pharmacotherapy is currently aimed primarily at managing behavioral and psychiatric symptoms, and, in selected cases, controlling severe chorea. Effective intervention by clinicians is possible, however, in terms of providing patients and families with accurate information about the disease, counseling them about availability of genetic testing at specialized centers, and in giving them sound advice regarding work, driving, relationships, finances, research participation, and support groups.

KEYWORDS

Huntington's disease - genetic testing - CAG repeat disorders - chorea - movement disorders - subcortical dementia

Full Text

References

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Francis O WalkerM.D.

Department of Neurology, Wake Forest University School of Medicine, Medical Center Blvd.

Winston-Salem, NC 27157-1078