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Brain and Nerve No to Shinkei Volume 56, Issue 9 （September 2004）

Brain and Nerve 脳と神経 56巻9号（2004年9月発行）

Japanese English

症例報告

痴呆を伴うパーキンソン病の臨床像を呈した嗜銀性顆粒痴呆（argyrophilic grain disease）の1剖検例 Argyrophilic Grain Disease Clinically Mimicking Parkinson's Disease with Dementia : Report of an Autopsy Case 内門大丈 ^1,2 , 土谷邦秋 ³ , 富永格 ⁴ , 都甲崇 ^1,2 , 大島健一 ⁵ , 秋山治彦 ² , 池田研二 ² , 織田辰郎 ⁴ , 平安良雄 ¹ Hirotake Uchikado ^1,2 , Kuniaki Tsuchiya ³ , Itaru Tominaga ⁴ , Takashi Togo ^1,2 , Kenichi Oshima ⁵ , Haruhiko Akiyama ² , Kenji Ikeda ² , Tatsuro Oda ⁴ , Yoshio Hirayasu ¹ ¹横浜市立大学医学部精神医学教室 ²東京都精神医学総合研究所 ³東京都立松沢病院検査科 ⁴国立下総療養所神経精神科 ⁵東京都立松沢病院精神科 ¹Department of Psychiatry, Yokohama City University School of Medicine ²Department of Psychogeriatrics, Tokyo Institute of Psychiatry ³Department of Laboratory Medicine and Pathology, Tokyo Metropolitan Matsuzawa Hospital ⁴Department of Neuropsychiatry, National Shimousa Mental Hospital ⁵Department of Psychiatry, Tokyo Metropolitan Matsuzawa Hospital キーワード： argyrophilic grain disease , Parkinson's disease , dementia , neurodegenerative diseases Keyword: argyrophilic grain disease , Parkinson's disease , dementia , neurodegenerative diseases pp.785-788

発行日 2004年9月1日 Published Date 2004/9/1

DOI https://doi.org/10.11477/mf.1406100338

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Abstract 文献概要
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　要旨　嗜銀性顆粒痴呆（argyrophilic grain disease：AGD）は，グレインと呼ばれる嗜銀性の顆粒状構造物が脳内に出現することを病理学的特徴とする痴呆性変性疾患であるが，現時点ではその臨床症状に関する報告は極めて少ない。今回われわれは，上肢の振戦で発症し，寡動や筋固縮も含むパーキンソニズムが出現し，発症8年後に記憶障害や失見当識，易怒性や易刺激性が出現した1症例を経験した。本症例の臨床診断は，痴呆を伴うパーキンソン病（Parkinson's disease with dementia）であった。しかし，病理学的には，桃核に神経細胞脱落とballooned neuronsとグレインの出現，さらに海馬・海馬傍回に多数のグレインとcoiled bodyの出現を認め，AGDと診断した。黒質・青斑核などに神経細胞脱落やレビー小体の出現は見い出されなかった。以上の臨床病理像より，AGDの臨床亜型として，痴呆を中心とする臨床像以外に，“痴呆を伴うパーキンソニズム”が存在しうることが示唆された。

　Argyrophilic grain disease（AGD）is a neurodegenerative dementia, which is neuropathologically characterized by the spindle-or comma-shaped argyrophilic grains scattered in the neuropil of hippocampal area. Several research reports have disclosed the pathological, biochemical and genetic characteristics of AGD, whereas the clinical aspects have not been fully investigated. Here we report an autopsy case of AGD. She developed tremor at age 63, and then developed dyskinesia, rigidity and gait disturbance. Thereafter, she had cognitive impairment and emotional disturbance at age 71, and died of pneumonia at age 76. She was clinically diagnosed as Parkinson's disease with dementia due to the presence of parkinsonism and dementia. Macroscopically, the brain demonstrated mild atrophy, and the weight was 1,240g. Many argyrophilic grains were found in the hippocampus and amygdala. Coiled bodies and ballooned neurons were also present, while Alzheimer-type neurofibrillary changes were mild, con-sistent with stage 2 of Braak's classification. This case was neuropathologically diagnosed as AGD. In contrast, no remarkable pathological changes, including neuronal loss and Lewy bodies, were found in the nigra, locus ceruleus and basal nuclei. On the basis of the above-mentioned clinicopathological findings, parkinsonism with dementia is considered to be one of the clinical manifestations of AGD.

（Received : April 28, 2004）