Sporadic motor neuron disease with severe sensory neuronopathy

被引：0

作者：

K. Wakabayashi

Y. Horikawa

Mutsuo Oyake

Shoji Suzuki

Takashi Morita

Hitoshi Takahashi

机构：

[1] Brain Disease Reserach Center,

[2] Brain Research Institute,undefined

[3] Niigata University,undefined

[4] 1 Asahimachi,undefined

[5] Niigata 951-8585,undefined

[6] Japan Tel.: 81-25-223-6161,undefined

[7] ext. 5221; Fax: 81-25-223-7503; e-mail: koichi@bri.niigata-u.ac.jp,undefined

[8] Department of Neurology,undefined

[9] Shinrakuen Hospital,undefined

[10] Niigata,undefined

[11] Japan,undefined

[12] Department of Pathology,undefined

[13] Shinrakuen Hospital,undefined

[14] Niigata,undefined

[15] Japan,undefined

[16] Department of Pathology,undefined

[17] Brain Research Institute,undefined

[18] Niigata University,undefined

[19] 1 Asahimachi,undefined

[20] Niigata 951-8585,undefined

[21] Japan,undefined

来源：

Acta Neuropathologica | 1998年 / 95卷

关键词：

Key words Motor neuron disease; Amyotrophic lateral sclerosis; Sensory neuronopathy; Bunina body; Skein-like inclusion;

D O I：

暂无

中图分类号：

学科分类号：

摘要：

We report a 62-year-old man with sporadic motor neuron disease (MND) of 52 months’ duration with progressive sensory disturbance and high cerebrospinal fluid protein content. Neuropathologically, both the upper and lower motor neuron systems were severely affected, and light and electron microscopy revealed Bunina bodies and skein-like inclusions, which are characteristic of amyotrophic lateral sclerosis, in the remaining anterior horn cells. Moreover, there was severe degeneration without inflammatory infiltrates in the spinal posterior columns, spinal ganglia, and peripheral sensory nerves. These findings suggest that this case may be an unusual variant of sporadic MND with severe somatic sensory system involvement.

引用

页码：426 / 430

页数：4