ナカムラ　マサタカ   NAKAMURA M.   中村　正孝    所属   関西医科大学  神経内科学講座    職種   講師
言語種別	日本語
発表タイトル	長期の臨床経過を示した弧発性筋萎縮性側索硬化症の一剖検例
会議名	第50回日本神経病理学会総会学術研究会
学会区分	全国規模の学会
発表形式	ポスター掲示
講演区分	一般
発表者・共同発表者	◎西井誠, 伊東秀文, 木下芳美, 中村聖香, 朝山知子, 朝山真哉, 中村正孝, 藤田賢吾, 和手麗香, 新出明代, 中野智, 日下博文
発表年月日	2009/06
開催地 （都市, 国名）	高松市
概要	A 61-year-old man presented with progressive dysarthria, dysphagia and disturbed gait. Neurologically, tendon reflexes and extensor plantar responses were exaggerated, but lower motor neuron signs were absent until he reached the age of 71, when active denervation potential was detected in his right hand. Muscle wasting progressed very slowly thereafter, but fasciculation did not arise throughout the course of the disease. He developed prostate cancer at age 80, which caused compression myelopathy due to metastasis into the Th3 vertebra, and he died without artificial ventilation 20 years after onset. Gene analysis revealed no mutations in the SOD1 gene. The brain weighed 1,020 g after fixation and no atrophy was evident in the precentral gyrus. Microscopic observation revealed a degenerated pyramidal tract, depleted Betz cells and a slight decrease in the number of anterior horn cells. Bunina bodies or round hyaline inclusions were not evident. However, a few skein-like inclusions were identified only by TDP-43 immunostaining. Compared with conventional SALS, the neuropathology of the present case was very mild, and TDP-43 immunohistochemistry helped to detect subtle findings.