MSA-C is the predominant clinical phenotype of MSA in Japan: analysis of 142 patients with probable MSA.
We investigated the clinical features and mode of disease progression in 142 patients with probable multiple system atrophy (MSA) according to the Consensus Criteria. The subjects included 84 men and 58 women with a mean age at onset of 58.2+/-7.1 years (range: 38-79 years). Cerebellar signs were detected in 87.3% of these patients at the time of initial examination, and were found in 95.1% of them at latest follow-up. MSA-C was diagnosed in 83.8% of the patients at their first examination. Parkinsonism was initially detected in 28.9% of the patients, increasing to 51.4% at the latest follow-up. Among all of the subjects, only 16.2% were classified as having MSA-P on initial examination. At the latest follow-up, parkinsonian features had become predominant over cerebellar features in 24.6% of the 65 patients with MSA-C who were followed for more than 3 years. Although parkinsonism usually masked the signs of cerebellar involvement in MSA-C patients, none of the patients with MSA-P at an early stage showed predominance of cerebellar features at the latest follow-up. Parkinsonism is the predominant feature of MSA among Western patients, even at an early stage, but this study showed that cerebellar deficits are the main feature in Japanese patients. This difference of disease manifestations between ethnic groups suggests that genetic factors may influence the clinical phenotype of MSA.
Yabe
Ichiro
I
Department of Neurology, Hokkaido University, Graduate School of Medicine, Sapporo, Hokkaido 060-8368, Japan. yabe@med.hokudai.ac.jp
Soma
Hiroyuki
H
Takei
Asako
A
Fujiki
Naoto
N
Yanagihara
Tetsuro
T
Sasaki
Hidenao
H
D000328:Adult; D000368:Aged; D000978:Antiparkinson Agents / Q000627:therapeutic use; D001341:Autonomic Nervous System / Q000503:physiopathology; D002524:Cerebellar Ataxia / Q000209:etiology / Q000503:physiopathology; D003645:Death, Sudden / Q000209:etiology; D003952:Diagnostic Imaging; D004351:Drug Resistance; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007564:Japan / Q000453:epidemiology; D007980:Levodopa / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009069:Movement Disorders / Q000209:etiology / Q000503:physiopathology; D019578:Multiple System Atrophy / Q000145:classification / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology* / Q000503:physiopathology; D020734:Parkinsonian Disorders / Q000188:drug therapy / Q000209:etiology / Q000503:physiopathology; D010641:Phenotype; D012189:Retrospective Studies; D014826:Vocal Cord Paralysis / Q000209:etiology
D000978:Antiparkinson Agents; D007980:Levodopa