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Sporadic ataxias in Japan – a population-based epidemiological study

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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.

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Authors and Affiliations

  1. Department of Neurology, The University of Tokyo, Tokyo, Japan

    Shoji Tsuji & Jun Goto

  2. Department of Neurology, Brain Research Institute, Niigata University, Niigata, Japan

    Osamu Onodera & Masatoyo Nishizawa

  3. Department of Neurology, University of Tokyo Hospital, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan

    Shoji Tsuji

Authors
  1. Shoji Tsuji
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  2. Osamu Onodera
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  3. Jun Goto
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  4. Masatoyo Nishizawa
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On Behalf of the Study Group on Ataxic Diseases*

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Correspondence to Shoji Tsuji.

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*Shoji Tsuji, Osamu Onodera, Ichiro Kanazawa, Hidehiro Mizusawa, Takamichi Hattori, Gen Sobue, Mitsunori Yamada, Yoshiyuki Kuroiwa, Akira Kakizuka, Atsushi Takeda, Kazuko Hasegawa, Tatsuhiko Yuasa, Takemasa Kanda, Kunihiro Yoshida, Teruhiko Kachi, Takashi Nakajima, Mitsuhiro Osame, Sadako Kuno, Kenji Nakajima, Hideshi Kawakami, Yoshitaka Nagai, and Masatoyo Nishizawa.

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Tsuji, S., Onodera, O., Goto, J. et al. Sporadic ataxias in Japan – a population-based epidemiological study. Cerebellum 7, 189–197 (2008). https://doi.org/10.1007/s12311-008-0028-x

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  • DOI: https://doi.org/10.1007/s12311-008-0028-x

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