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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.

Original publication

DOI

10.1136/jnnp.68.2.238

Type

Journal article

Journal

Journal of neurology, neurosurgery, and psychiatry

Publication Date

02/2000

Volume

68

Pages

238 - 241

Addresses

The Regional Movement Disorders Unit, Department of Neurology, Regional Neurosciences Centre, King's College Hospital, Guy's, King's, and St Thomas School of Medicine, London, UK.

Keywords

Humans, Huntington Disease, Parkinson Disease, Chorea, Levodopa, Antiparkinson Agents, Diagnosis, Differential, Tomography, Emission-Computed, Treatment Outcome, Trinucleotide Repeats, Aged, Middle Aged, Female, Male