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Brain and Nerve No to Shinkei Volume 39, Issue 8 （August 1987）

Brain and Nerve 脳と神経 39巻8号（1987年8月発行）

Japanese English

原著

著明な脳幹萎縮を呈したdentatorubropallidoluysian atrophy （DRPLA）の1剖検例 AN AUTOPSY CASE OF DENTATORUBROPALLIDOLUYSIAN ATROPHY SHOWING MARKED ATROPHY OF THE BRAIN STEM 森岡英五 ¹ , 中津武志 ² , 黒田重利 ² , 山本光利 ³ , 細川清 ¹ , 大月三郎 ² Eigo Morioka ¹ , Takeshi Nakatsu ² , Shigetoshi Kuroda ² , Mitsutoshi Yamamoto ³ , Kiyoshi Hosokawa ¹ , Sabro Otsuki ² ¹香川医科大学精神神経医学教室 ²岡山大学医学部神経精神医学教室 ³香川県立中央病院神経内科 ¹Department of Neuropsychiatry, Kagawa Medical School ²Department of Neuropsychiatry, Okayama University Medical School ³Department of Neurology, Kagawa Central Hospital pp.769-773

発行日 1987年8月1日 Published Date 1987/8/1

DOI https://doi.org/10.11477/mf.1406205957

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Abstract 文献概要
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　抄録　症例は58歳頃より，精神症状，痴呆，小脳症状，舞踏病様の不随意運動が，しだいに進行し，66歳時敗血症にて死亡した。全経過約8年。剖検では淡蒼球には病変はごく軽度であったが，淡蒼球・ルイ体系と歯状核・赤核系に変性がみられ，平山らのいうpseudo-Huntington formのdentatorubropallidoluy-sian atrophyと思われる。本例に特徴的なことは脳幹部特に被蓋部の萎縮が，高度であることと，家系内に5世代にわたり，18人におよぶ発病者がいることである。その発病年齢は，10歳から60歳に及び，若年発症者では，ミオクロヌス・けいれん発作で発症し，壮年発症者は失調歩行，痴呆，舞踏病様の不随意運動を呈する。そして，それらの症例のなかには，NMR-CTにより，脳幹部が小さいものが認められている。本症例における脳幹部の萎縮を中心に考察した。

An autopsy case of a 66 year-old woman is re-ported. She developed personality change and psy-chotic symptoms at the age of 58. She began to show gait disturbance and forgetfullness at the age of 60. She was admitted to Okayama Univer-sity Hospital at the age of 61, when she showed personality change, dementia, cerebellar sings and chorea like involuntary movement. The illness progressed slowly and she died of septicemia at the age of 66.

At autopsy brain weighed 990g. Macroscopically, the atrophy of the brain stem was severe, and the cerebellum was slightly atrophic. Microscopically, the globus pallidus was almost intact, but the de-generation involved dentate nuclei, their projec-tions, red nucleus and the subthalamic nuclei, so this case was considered to be a case of pseudo-Huntington form of dentatorubropallidoluysian atrophy, proposed by Hirayama.

The most striking feature of this case was mark-ed atrophy of the brain stem and her intense familial history. Investigation of her familial his-tory revealed that there were 18 affected cases in 5 successive generations. Their onset of the dis-ease varied from the age of 10 to 60 years old. Cases of juvenile onset showed myoclonus and convulsion as the intial symptoms, and convulsion as the ini-tial symptoms, and those of presenile onset showed dementia, cerebellar ataxia and chorea like in-voluntary movement. And in some of these cases it was proved by NMR-CT that their brain stem were small.

We dicussed the meaning of the atrophy of the brain stem in these cases and the difference of the symptoms between the cases of juvenile onset and the cases of presenile onset.