Late onset levodopa responsive Huntington's disease with minimal chorea masquerading as Parkinson plus syndrome.
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.
Reuter
I
I
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.
Hu
M T
MT
Andrews
T C
TC
Brooks
D J
DJ
Clough
C
C
Chaudhuri
K R
KR
D000368:Aged; D000978:Antiparkinson Agents / Q000627:therapeutic use*; D002819:Chorea / Q000175:diagnosis*; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D006816:Huntington Disease / Q000175:diagnosis* / Q000188:drug therapy* / Q000235:genetics; D007980:Levodopa / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D010300:Parkinson Disease / Q000175:diagnosis*; D014055:Tomography, Emission-Computed; D016896:Treatment Outcome; D018911:Trinucleotide Repeats
D000978:Antiparkinson Agents; D007980:Levodopa