Two cases of spinocerebellar ataxia accompanied by involvement of the skeletal motor neuron system and bulbar palsy

Yasuyuki Ohta; Takeshi Hayashi; Makiko Nagai; Miyuki Okamoto; Shoko Nagotani; Isao Nagano; Nobuhiko Ohmori; Yasushi Takehisa; Tetsuro Murakami; Mikio Shoji; Tatsushi Kamiya; Koji Abe

doi:10.2169/internalmedicine.46.6261

Two cases of spinocerebellar ataxia accompanied by involvement of the skeletal motor neuron system and bulbar palsy

Yasuyuki Ohta, Takeshi Hayashi, Makiko Nagai, Miyuki Okamoto, Shoko Nagotani, Isao Nagano, Nobuhiko Ohmori, Yasushi Takehisa, Tetsuro Murakami, Mikio Shoji, Tatsushi Kamiya, Koji Abe

Research output: Contribution to journal › Article › peer-review

18 Citations (Scopus)

Abstract

We report two patients with spinocerebellar ataxia (SCA) with cranial and spinal motor neuron involvement. They initially presented with cerebellar ataxia, followed by bulbar palsy and limb motor neuron sign. One of the patients had a brother with allied disorder. SCA type 1 (SCA1), SCA3 and SCA6 have been reported to involve the motor neuron system, but they were excluded by DNA analyses in the present two patients. These two patients may form a distinct disease entity among SCAs.

Original language	English
Pages (from-to)	751-756
Number of pages	6
Journal	Internal Medicine
Volume	46
Issue number	11
DOIs	https://doi.org/10.2169/internalmedicine.46.6261
Publication status	Published - Jun 1 2007

Keywords

Bulbar palsy
MRI
Motor neuron disease
Spinocerebellar ataxia
Tongue atrophy

ASJC Scopus subject areas

Internal Medicine

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10.2169/internalmedicine.46.6261

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abstract = "We report two patients with spinocerebellar ataxia (SCA) with cranial and spinal motor neuron involvement. They initially presented with cerebellar ataxia, followed by bulbar palsy and limb motor neuron sign. One of the patients had a brother with allied disorder. SCA type 1 (SCA1), SCA3 and SCA6 have been reported to involve the motor neuron system, but they were excluded by DNA analyses in the present two patients. These two patients may form a distinct disease entity among SCAs.",

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AU - Ohta, Yasuyuki

AU - Hayashi, Takeshi

AU - Nagai, Makiko

AU - Okamoto, Miyuki

AU - Nagotani, Shoko

AU - Nagano, Isao

AU - Ohmori, Nobuhiko

AU - Takehisa, Yasushi

AU - Murakami, Tetsuro

AU - Shoji, Mikio

AU - Kamiya, Tatsushi

AU - Abe, Koji

PY - 2007/6/1

Y1 - 2007/6/1

N2 - We report two patients with spinocerebellar ataxia (SCA) with cranial and spinal motor neuron involvement. They initially presented with cerebellar ataxia, followed by bulbar palsy and limb motor neuron sign. One of the patients had a brother with allied disorder. SCA type 1 (SCA1), SCA3 and SCA6 have been reported to involve the motor neuron system, but they were excluded by DNA analyses in the present two patients. These two patients may form a distinct disease entity among SCAs.

AB - We report two patients with spinocerebellar ataxia (SCA) with cranial and spinal motor neuron involvement. They initially presented with cerebellar ataxia, followed by bulbar palsy and limb motor neuron sign. One of the patients had a brother with allied disorder. SCA type 1 (SCA1), SCA3 and SCA6 have been reported to involve the motor neuron system, but they were excluded by DNA analyses in the present two patients. These two patients may form a distinct disease entity among SCAs.

KW - Bulbar palsy

KW - MRI

KW - Motor neuron disease

KW - Spinocerebellar ataxia

KW - Tongue atrophy

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Two cases of spinocerebellar ataxia accompanied by involvement of the skeletal motor neuron system and bulbar palsy

Abstract

Keywords

ASJC Scopus subject areas

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