PT  - JOURNAL ARTICLE
AU  - I Reuter
AU  - M T M Hu
AU  - T C Andrews
AU  - D J Brooks
AU  - C Clough
AU  - K Ray Chaudhuri
TI  - Late onset levodopa responsive Huntington's disease with minimal chorea masquerading as Parkinson plus syndrome
AID  - 10.1136/jnnp.68.2.238
DP  - 2000 Feb 01
TA  - Journal of Neurology, Neurosurgery & Psychiatry
PG  - 238--241
VI  - 68
IP  - 2
4099  - http://jnnp.bmj.com/content/68/2/238.short
4100  - http://jnnp.bmj.com/content/68/2/238.full
SO  - J Neurol Neurosurg Psychiatry2000 Feb 01; 68
AB  - 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.