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Late onset levodopa responsive Huntington's disease with minimal chorea masquerading as Parkinson plus syndrome

Late onset levodopa responsive Huntington's disease with minimal chorea masquerading as Parkinson... Huntington's disease is characterised by hyperkinetic movements, mainly chorea, cognitive dysfunction, and psychiatric abnormalities. Non-dopa responsive parkinsonism occurs in the later stages of choreic disease or as the predominant feature of juvenile patients (Westphal variant). Late onset Huntington's disease presenting as levodopa responsive parkinsonism is rare. A series of four patients with late onset Huntington's disease presenting as levodopa responsive parkinsonism and cardiovascular dysautonomia, initially misdiagnosed as multiple system atrophy (MSA) in three patients, is reported. Levodopa treatment did not unmask significant chorea. These cases suggest the presence of a distinct phenotypic variant of Huntington's disease to be added to the differential diagnosis of other akinetic rigid syndromes. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Journal of Neurology Neurosurgery & Psychiatry British Medical Journal

Late onset levodopa responsive Huntington's disease with minimal chorea masquerading as Parkinson plus syndrome

Late onset levodopa responsive Huntington's disease with minimal chorea masquerading as Parkinson plus syndrome

Journal of Neurology Neurosurgery & Psychiatry , Volume 68 (2) – Feb 1, 2000

Abstract


Huntington's disease is characterised by hyperkinetic movements, mainly chorea, cognitive dysfunction, and psychiatric abnormalities. Non-dopa responsive parkinsonism occurs in the later stages of choreic disease or as the predominant feature of juvenile patients (Westphal variant). Late onset Huntington's disease presenting as levodopa responsive parkinsonism is rare. A series of four patients with late onset Huntington's disease presenting as levodopa responsive parkinsonism and cardiovascular dysautonomia, initially misdiagnosed as multiple system atrophy (MSA) in three patients, is reported. Levodopa treatment did not unmask significant chorea. These cases suggest the presence of a distinct phenotypic variant of Huntington's disease to be added to the differential diagnosis of other akinetic rigid syndromes.

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Publisher
British Medical Journal
Copyright
Journal of Neurology, Neurosurgery, and Psychiatry
ISSN
0022-3050
eISSN
1468-330X
DOI
10.1136/jnnp.68.2.238
Publisher site
See Article on Publisher Site

Abstract

Huntington's disease is characterised by hyperkinetic movements, mainly chorea, cognitive dysfunction, and psychiatric abnormalities. Non-dopa responsive parkinsonism occurs in the later stages of choreic disease or as the predominant feature of juvenile patients (Westphal variant). Late onset Huntington's disease presenting as levodopa responsive parkinsonism is rare. A series of four patients with late onset Huntington's disease presenting as levodopa responsive parkinsonism and cardiovascular dysautonomia, initially misdiagnosed as multiple system atrophy (MSA) in three patients, is reported. Levodopa treatment did not unmask significant chorea. These cases suggest the presence of a distinct phenotypic variant of Huntington's disease to be added to the differential diagnosis of other akinetic rigid syndromes.

Journal

Journal of Neurology Neurosurgery & PsychiatryBritish Medical Journal

Published: Feb 1, 2000

Keywords: Huntington's disease chorea dopa-responsive multiple system atrophy postural hypotension

References