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

CASE REPORTS

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

Author information

Yasuyuki Ohta
Department of Neurology, Graduate School of Medicine, Dentistry and Pharmacy, Okayama University
Takeshi Hayashi
Department of Neurology, Graduate School of Medicine, Dentistry and Pharmacy, Okayama University
Makiko Nagai
Department of Neurology, Graduate School of Medicine, Dentistry and Pharmacy, Okayama University
Miyuki Okamoto
Department of Neurology, Graduate School of Medicine, Dentistry and Pharmacy, Okayama University
Shoko Nagotani
Department of Neurology, Graduate School of Medicine, Dentistry and Pharmacy, Okayama University
Isao Nagano
Department of Neurology, Graduate School of Medicine, Dentistry and Pharmacy, Okayama University
Nobuhiko Ohmori
Department of Neurology, Graduate School of Medicine, Dentistry and Pharmacy, Okayama University
Yasushi Takehisa
Department of Neurology, Graduate School of Medicine, Dentistry and Pharmacy, Okayama University
Tetsuro Murakami
Department of Neurology, Graduate School of Medicine, Dentistry and Pharmacy, Okayama University
Mikio Shoji
Department of Neurology, Graduate School of Medicine, Dentistry and Pharmacy, Okayama University
Tatsushi Kamiya
Department of Neurology, Graduate School of Medicine, Dentistry and Pharmacy, Okayama University
Koji Abe
Department of Neurology, Graduate School of Medicine, Dentistry and Pharmacy, Okayama University

Keywords: spinocerebellar ataxia, motor neuron disease, bulbar palsy, tongue atrophy, MRI

JOURNAL OPEN ACCESS

2007 Volume 46 Issue 11 Pages 751-756

DOI https://doi.org/10.2169/internalmedicine.46.6261

Details

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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