ABSTRACT
Introduction: Our study aimed to describe the clinical features of multiple system atrophy (MSA) in Singapore and verify its diagnosis using the consensus statement in the diagnosis of MSA. Materials and Methods: All patients suspected to have MSA between 1995 and March 2005 were identified from the Movement Disorders database and the autonomic function testing results. The medical records were reviewed using a standardised data collection form. The diagnosis of MSA was verified using the consensus statement. Disease progression was evaluated using 2 pre-determined events: aid-requiring walking and wheelchair use. Results: Seventy-two per cent (33/46) fulfilled the consensus statement. There were 85% Chinese, 9% Malays, and 6% Indians. The mean age at onset of the disease was 60 ± 10 years. We found a predominance of males (M:F = 1.5:1) as well as MSA-C cases (67%). The most common initial presenting features were parkinsonism and cerebellar signs (27% each). Abnormal neuroimaging was seen in 29 patients (91%). Autonomic function testing was abnormal in 58% (7/12). The risk for aid-requiring walking and wheelchair use at 3 years from onset of the disease was 31% and 17%, respectively. By 5 years, this had increased to 45% and 30%, respectively. There was no difference in the events rate between MSA subtypes. Conclusions: The clinical characteristics of MSA in Singapore are presented. Our study revealed a predominance of MSA-C patients as well as a later age at onset of disease and longer median time to aid-requiring walking and wheelchair use compared to Japanese patients.
Multiple system atrophy (MSA), a term introduced in 1969, is a progressive neurodegenerative disease which occurs sporadically and is characterised by parkinsonism, cerebellar dysfunction, and autonomic insufficiency in various combinations. 1-3 It usually begins in the fifth decade and affects both sexes. MSA now includes the disorders previously called Shy-Drager syndrome, olivopontocerebellar atrophy (OPCA), and striatonigral degeneration (SND). The discovery of glial cytoplasmic inclusions (GCI) in the brain of patients with these conditions confirmed that SND, OPCA, and Shy-Drager syndrome are the same disease with different clinical expressions. 4 Several diagnostic criteria have been proposed but no consistent guidelines have been developed.2,5,6 A consensus conference was held in 1998 and a new diagnostic criteria was adapted.3 It was then recommended that patients be designated as having MSA-C if cerebellar features predominate and MSA-P if parkinsonian features predominate, instead of using the old terms SND and sporadic OPCA, respectively.3 The term Shy-Drager syndrome was found to be no longer useful since autonomic failure is invariably present.
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