Sporadic ataxias in Japan--a population-based epidemiological study

Cerebellum. 2008;7(2):189-97. doi: 10.1007/s12311-008-0028-x.


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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Cerebellar Ataxia / epidemiology*
  • Cerebellar Ataxia / genetics
  • Cerebellar Ataxia / physiopathology
  • Humans
  • Japan / epidemiology
  • Knee Joint / physiopathology
  • Neuropsychological Tests
  • Olivopontocerebellar Atrophies / epidemiology*
  • Olivopontocerebellar Atrophies / genetics
  • Olivopontocerebellar Atrophies / physiopathology
  • Prevalence
  • Tibia / physiopathology
  • Tremor / epidemiology