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Brain and Nerve No to Shinkei Volume 44, Issue 2 （February 1992）

Brain and Nerve 脳と神経 44巻2号（1992年2月発行）

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症例報告

失調型Creutzfeldt-Jakob病の1剖検例 An Autopsy Case of Ataxic Form of Creutzfeldt-Jakob Disease 妹尾晴夫 ¹ , 石野博志 ¹ , 稲垣卓司 ¹ , 山森周子 ¹ , 飯島正明 ¹ , 田中新一 ¹ , 井手下久登 ² , 好永順二 ² , 引地明義 ² Haruo Seno ¹ , Hiroshi Ishino ¹ , Takuji Inagaki ¹ , Chikako Yamamori ¹ , Masaaki Iijima ¹ , Shinichi Tanaka ¹ , Hisato Ideshita ² , Junji Yoshinaga ² , Akiyoshi Hikiji ² ¹島根医科大学精神医学教室 ²広島市民病院神経科 ¹Department of Psychiatry, Shimane Medical University ²Department of Neuroiogy, Hiroshima City Hospital キーワード： ataxic fom , Creutzfeldt-Jakob disease , severe degeneration of granule cell type in the cerebeilar cortex Keyword: ataxic fom , Creutzfeldt-Jakob disease , severe degeneration of granule cell type in the cerebeilar cortex pp.177-181

発行日 1992年2月1日 Published Date 1992/2/1

DOI https://doi.org/10.11477/mf.1406900304

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Abstract 文献概要
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　Brownell，Oppenheimerの提唱した失調型Creutzfeldt-Jakob病の1例を報告した。症例は死亡時71歳の男子で，臨床的に初期には歩行時ふらつきに代表される小脳性失調症状および複視を示し，その後意識レベルの変動，四肢のミオクローヌス，Gegenhalten，脳波上のPSDを呈しながら失外套状態となり全経過5カ月で死亡した。神経病理学的所見としては，肉眼的に大脳皮質の萎縮と脳室の軽度拡大を認め，組織学的には，大脳皮質の層構築は不明瞭となり，基質のふるい状の微細海綿状態，神経細胞の残存，視床病変，線条体病変および，それに比べより重篤な小脳病変（顆粒細胞の脱落と皮質のグリオーゼ）を認めた点が特徴的であった。本邦では非常に稀な症例と思われた。

An autopsy case of ataxic form of Creutzfeldt-Jakob disease (Brownell and Oppenheimer, 1965) was reported. The patient, a 71-year-old male, noticed ataxic gait at the beginning of June in 1988, and was admitted to the Hiroshima City Hospital for the neurological examination at the end of June. He showed ataxia of the left arm and legs and diplopia. Gradually he became delirious at night. On July 16, tremor-like involuntary movement of the left hand was noticed. On July 20, he became somno-lent and doubly incontinent. Myoclonus and par-atonic rigidity were also observed. The EEG showed periodic synchronous discharge on July 25. The brain CT and MRI were normal. He became apallic gradually and died on October 28. The duration of illness was 5 months.

At autopsy, brain weighed 1000gr. Cerebral atro-phy and slight enlargement of the ventricles were observed. The cerebellum was also slightly atro-phic. Histologically, the destruction of the cerebral cortical layer, slight sieve-like spongy state of the neuropil, slight neuronal loss of the thalamus and sieve-like spongy state of the striatum were obser-ved. The cerebellar lesion was the most severe, where granular cell loss and gliosis of the cortex were observed.