筋萎縮性側索硬化症，痴呆，頸の後屈を示した1剖検例

AN AUTOPSY CASE OF AMYOTROPHIC LATERAL SCLEROSIS WITH DEMENTIA AND NECK EXTENSION

　抄録　筋萎縮，痴呆，頸部後屈を呈した1剖検例を報告した。症例は60歳，男性で，58歳嗄声，構音障害の球症状ではじまり，痴呆が加わり，筋萎縮は全身へと進行し，末期に頸部固縮，後屈を示し，呼吸麻痺で死亡した。経過1年5カ月。神経病理学的には，大脳皮質では前頭葉，側頭葉で，限局性の表層の海綿状態と極めてわずかの原線維変化，老人斑を認めるのみでPick球はなかった。中心前回，脳幹運動神経核，脊髄，筋肉に運動ニューロン疾患の所見を得た。側頭葉白質，淡蒼球外節，扁桃核，脊髄前角にグリオーシスがあり，Neumannの進行性皮質下グリオーシスに類似しているが，軽度であった。中脳黒質に変性をみた。最近，痴呆，筋萎縮，パーキンソン病類似の錐体外路症状を呈した報告例がある。本例はそれらと同一であるが，臨床，病理像とともに筋萎縮性側索硬化症の所見が明らかであったことが注目される。

A 58-year-old man recognized hoarseness and dysarthria followed by weakness of the left upper extremity. Examination five months later disclosed dementia which was manifested dominantly by changes in personality and behavior, but also by symptoms of amyotrophic lateral sclerosis. He became progressively weak, and his neck became rigid and extended for two months. He expired due to respiratory failure at the age of 60.

At autopsy, the brain weighed 1120g with atrophy of the temporal lobes. Microscopic exami-nation revealed marked diminution of Betz cells with astrocytic proliferation in the motor cortex. There was a mild, localized spongy state in the upper layer in the frontal and temporal cortices. There were very few neurofibrillary changes and senile plaques, and no Pick's argentophilic bodies. Fibrillary gliosis was found in the white matter of the temporal lobes, external segment of the globus pallidus and the amygdaloid nucleus. The substantia nigra showed depletion of pigmented cells, free melanin pigment and reactive astrocy-tosis. A diminished number of motor neurons in the brain stem and spinal cord accompanied astro-cytic gliosis, while the remaining cell contained many Bunina bodies. The pyramidal tracts show-ed mild degeneration bilaterally below the pyramis in the meddula.

There are a number of cases in the literature whose pictures consist of amyotrophic lateral scle-rosis, dementia and/or exptapyramidal symptoms. This case is identical to those cases. But in this case, the clinical and pathological features of amyo-trophic lateral sclerosis are more dominant than in other cases.