小脳失調から除脳状態へ移行し，Kuru斑を認めた1剖検例

A NECROPSY CASE OF PROGRESSIVE ATAXIA ENDING IN APALLIC STATE WITH KURU PLAQUES

I．はじめに

　Kuru^3,7,12,14）はニユーギニア高地のFore族にみられる亜急性，進行性の神経疾患で，それがチンパンジーに移植できたことより現在ではCreutzfeldt-Jakob （C-J）病と同じく，transmissible sponglform encephalopathyの1群に入れられている。その病理所見としては脳幹，間脳，小脳皮質などを中心にニューロンの著しい消失，海綿状態が主要所見であるが，その他に1つの特微的所見としてKuru^3,12）斑がある。我々はこのたび死亡時61歳の男性で，脳内にKuru斑といつてさしつかえない老人斑に似た構造物を認めた1例を経験したので，その臨床・病理像を報告し，若干の考察をおこないたい。

The clinical and pathological findings of a maleaged 61 at death was reported. He first noticed a"wobbly sensation" in his left leg at age of 53and the gait deteriorated. It was accompanied bydysarthria and the clumsiness in upper limbs. Theneurological examination on admission disclosedcerebellar ataxia and loss of tendon reflexes in thelower limbs. He showed mild difficulty withmemory and calculation, too. The illness progressedsteadily and dementia was obvious at age 59 andhe was in apallic state at age 60. He died ofbronchopneumonia at age 61.

At autopsy the brain weight was 1170 gm. andthe atrophy was found in the frontal and temporalregions. The remarkable finding was numeroussenile plaques like structures. They were observedin the cerebellar cortex, cerebral cortex, hippo-campus, thalamus and basal ganglia. They are eo-sinophilic, strongly PAS positive, weakly argyrop-hilic and positive and anisotropic in congo-red sec-tions. The electron microscopic study showed theseplaques were composed of bundles of interwovenamyloid fibrils which radiated out from a centralcore. From above mentioned the plaques can beconsidered Kuru plaques. Other findings were mildspongy state in the cerebral cortex, the degenera-tion of the spinocerebellar tracts and posterior co-lumn and the neuronal loss in the hypothalamicnucleus, the red nucleus, substantia nigra, inferiorolive and vermal grey matter. Thesepathology weresimilar to "Austrian Kuru (Seitelberger)" but forluck of familial history in this case.

The authors compared the case with the casesof Kuru and similar cases previously reported andadded some comments.