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

被引:16
作者
Ohta, Yasuyuki [1 ]
Hayashi, Takeshi [1 ]
Nagai, Makiko [1 ]
Okamoto, Miyuki [1 ]
Nagotani, Shoko [1 ]
Nagano, Isao [1 ]
Ohmori, Nobuhiko [1 ]
Takehisa, Yasushi [1 ]
Murakami, Tetsuro [1 ]
Shoji, Mikio [1 ]
Kamiya, Tatsushi [1 ]
Abe, Koji [1 ]
机构
[1] Okayama Univ, Grad Sch Med Dent & Pharm, Dept Neurol, Okayama, Japan
关键词
spinocerebellar ataxia; motor neuron disease; bulbar palsy; tongue atrophy; MRI;
D O I
10.2169/internalmedicine.46.6261
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
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.
引用
收藏
页码:751 / 755
页数:5
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