Clinicopathologic investigation of a family with expanded SCA8 CTA/CTG repeats

H. Ito, H. Kawakami, R. Wate, S. Matsumoto, T. Imai, A. Hirano, H. Kusaka

Research output: Contribution to journalArticle

27 Citations (Scopus)

Abstract

We investigated a family manifesting progressive ataxia, with expanded SCA8 CTA/CTG repeats. Neuropathologically, degeneration of Purkinje, inferior olivary, and nigral neurons and periaqueductal gliosis were evident. The sites of Purkinje cell loss were occupied by fibrillary accumulations. The remaining Purkinje cells showed somatic sprouts, and intracytoplasmic 1C2-positive granular structures were recognizable. This characteristic distribution of neurodegeneration and Purkinje cell cytopathology were distinct from those of other hereditary spinocerebellar ataxias previously reported.

Original languageEnglish (US)
Pages (from-to)1479-1481
Number of pages3
JournalNeurology
Volume67
Issue number8
DOIs
StatePublished - Oct 2006

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Purkinje Cells
Spinocerebellar Degenerations
Spinocerebellar Ataxias
Gliosis
Substantia Nigra
Ataxia
Neurons

ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

Ito, H., Kawakami, H., Wate, R., Matsumoto, S., Imai, T., Hirano, A., & Kusaka, H. (2006). Clinicopathologic investigation of a family with expanded SCA8 CTA/CTG repeats. Neurology, 67(8), 1479-1481. https://doi.org/10.1212/01.wnl.0000240256.13633.7b

Clinicopathologic investigation of a family with expanded SCA8 CTA/CTG repeats. / Ito, H.; Kawakami, H.; Wate, R.; Matsumoto, S.; Imai, T.; Hirano, A.; Kusaka, H.

In: Neurology, Vol. 67, No. 8, 10.2006, p. 1479-1481.

Research output: Contribution to journalArticle

Ito, H, Kawakami, H, Wate, R, Matsumoto, S, Imai, T, Hirano, A & Kusaka, H 2006, 'Clinicopathologic investigation of a family with expanded SCA8 CTA/CTG repeats', Neurology, vol. 67, no. 8, pp. 1479-1481. https://doi.org/10.1212/01.wnl.0000240256.13633.7b
Ito H, Kawakami H, Wate R, Matsumoto S, Imai T, Hirano A et al. Clinicopathologic investigation of a family with expanded SCA8 CTA/CTG repeats. Neurology. 2006 Oct;67(8):1479-1481. https://doi.org/10.1212/01.wnl.0000240256.13633.7b
Ito, H. ; Kawakami, H. ; Wate, R. ; Matsumoto, S. ; Imai, T. ; Hirano, A. ; Kusaka, H. / Clinicopathologic investigation of a family with expanded SCA8 CTA/CTG repeats. In: Neurology. 2006 ; Vol. 67, No. 8. pp. 1479-1481.
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