Late onset levodopa responsive Huntington's disease with minimal chorea masquerading as Parkinson plus syndrome
- aThe 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, bMRC Cyclotron Unit, Imperial College School of Medicine, Hammersmith Hospital, London, UK , cUniversity Hospital Lewisham, London, UK
- Dr K Ray Chaudhuri, Department of Clinical Neurosciences, Mapother House, King's College Hospital, Denmark Hill, London SE5 9RS, UK emailjudy.grimshaw{at}kcl.ac.uk
- Received 7 April 1999
- Revised 17 September 1999
- Accepted 21 October 1999
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.
