Late onset levodopa responsive Huntington's disease with minimal chorea masquerading as Parkinson plus syndrome. Issue 2 (1st February 2000)

• Record Type: Journal Article
• Authors: Reuter, I; Hu, M T M; Andrews, T C; Brooks, D J; Clough, C; Chaudhuri, K Ray
• Is Part Of: Journal of neurology, neurosurgery and psychiatry. Volume 68:Issue 2(2000)
• Page Start: 238
• Page End: 241
• Publication Date: 2000-02-01
• Subjects: Huntington's disease -- chorea -- dopa-responsive -- multiple system atrophy -- postural hypotension; Neurology -- Periodicals; Nervous system -- Surgery -- Periodicals; Psychiatry -- Periodicals; 616.8
• DOI: 10.1136/jnnp.68.2.238
• Languages: English
• ISSNs: 0022-3050

Abstract

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.