title: Sporadic ataxias in Japan--a population-based epidemiological study.
abstract: Sporadic spinocerebellar ataxias (SCAs) comprise heterogeneous diseases with poorly understood epidemiologies and etiologies. A population-based epidemiological analysis of sporadic ataxias in the Japanese population was described. The prevalence rate of SCAs in the Japanese population is estimated to be 18.5/100,000. Sporadic SCAs account for 67.2% of total SCAs including hereditary SCAs, with olivopontocerebellar atrophy (OPCA) being the most common form sporadic ataxia (64.7%). The natural history analysis conducted on the basis of International Cooperative Ataxia Rating Scale (ICARS) showed that only 33% of patients with OPCA were able to walk at least with one stick 4-5 years after the onset of OPCA, which is much less than that of patients with cortical cerebellar atrophy (CCA). Similarly, 43% of patients with OPCA were able to stand alone 4-5 years after the onset, while 76% of patients with CCA were able to stand alone at the same disease duration. A population-based epidemiological analysis should provide essential information on the natural history of SCAs.
authors.affiliation: Department of Neurology, The University of Tokyo, Bunkyo-ku, Tokyo, Japan. tsuji@m.u-tokyo.ac.jp
authors.forename: Shoji
authors.initials: S
authors.lastname: Tsuji
authors.forename: Osamu
authors.initials: O
authors.lastname: Onodera
authors.forename: Jun
authors.initials: J
authors.lastname: Goto
authors.forename: Masatoyo
authors.initials: M
authors.lastname: Nishizawa
mesh_terms: D002524:Cerebellar Ataxia / Q000453:epidemiology* / Q000235:genetics / Q000503:physiopathology; D006801:Humans; D007564:Japan / Q000453:epidemiology; D007719:Knee Joint / Q000503:physiopathology; D009483:Neuropsychological Tests; D009849:Olivopontocerebellar Atrophies / Q000453:epidemiology* / Q000235:genetics / Q000503:physiopathology; D015995:Prevalence; D013977:Tibia / Q000503:physiopathology; D014202:Tremor / Q000453:epidemiology