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molecular pathological study of pripheral- type Lewy body disease

外周型路易体病的分子病理学研究

基本信息

批准号：
22K07550
负责人：
齊藤祐子
金额：
$ 2.66万
依托单位：
Tokyo Metropolitan Geriatric Hospital and Institute of Gerontology
依托单位国家：
日本
项目类别：
Grant-in-Aid for Scientific Research (C)
财政年份：
2022
资助国家：
日本
起止时间：
2022-04-01 至 2025-03-31
项目状态：
未结题

来源：
https://kaken.nii.ac.jp/grant/KAKENHI-PROJECT-22K07550/
关键词：
黒質レビー小体認知症 Lewy body alpha- synuclein pure autonomic failure Parkinson disease dementia with Lewy body

项目摘要

【目的】純粋自律神経不全症 (pure autonomic failure: PAF)は，レビー小体病理を背景とする，孤発性，成人発症，緩徐進行性の自律神経系の変性疾患であり，臨床的には起立性低血圧，膀胱・性機能障害などの自律神経症状を示すが，ほかの神経症状は伴わない．当施設で長期経過を観察し，剖検をえられた2例の特徴を検討する．【方法】症例1は死亡時88歳男性である．60代から頻回なめまいとふらつき，失神があり，便秘，発汗障害，嗅覚低下も出現した．途中，軽度認知機能障害を指摘された．その後，心原性脳塞栓症を発症，嚥下性肺炎で死亡した．症例2は死亡時85歳男性である．73歳時に高度の便秘と動揺性血圧が出現，81歳頃から，起立性低血圧や尿失禁，夜間徘徊が出現した．85歳時には認知機能低下が指摘され，同年，尿路感染症により死亡した．【結果】両症例ともに，肉眼所見では青斑核に高度の脱色素を認めたが，中脳黒質の脱色素は比較的軽度であった．組織学的には副腎周囲脂肪織内神経束や交感神経節，中間外側核や後根神経節，迷走神経背側核，延髄網様体，背側縫線核，青斑核，黒質，視床下部，島回，マイネルト基底核，移行嗅内野，海馬CA2，前帯状回，前頭・側頭・頭頂葉にレビー小体病理を認め，DLBコンセンサスガイドラインで辺縁系ないしは新皮質型に該当する広がりであった．【結論】両症例は，我々が提唱しているレビー小体病理の進展仮説であるbrain-first versus body-first仮説においては後者に該当するが，黒質が比較的保たれていたことが特徴である．また，海馬CA2のレビー小体病理が相対的に強い点は，PAFの晩期には認知機能にも留意が必要であることを支持する所見である．

【Purpose】Pure autonomic failure: PAF), the background of corpuscular pathology, autistic disorder, adult dysplasia, slowly progressive autonomic nervous system disorder, etc. , clinical orthostatic hypotension, bladder and sexual dysfunction, no autonomic symptoms, no symptoms, no symptoms of no autonomic symptoms, no symptoms. When the facility has been inspected for a long period of time, it is necessary to analyze the special cases of 2 cases. [Method] Case 1 was an 88-year-old male at the time of death. In the 60s, the symptoms of frequent return of narcosis, absentmindedness, constipation, dyshidrosis, and low olfactory symptoms appeared. On the way, he was diagnosed with cognitive impairment. Later, cardiogenic thromboembolic syndrome, dysphagia, and death from swallowing pneumonia. Case 2 was an 85-year-old male at the time of death. At the age of 73, high degree of constipation and irritable blood pressure appeared, and at the age of 81, orthostatic hypotension and urinary incontinence, as well as wandering at night, appeared. When he was 85 years old, he was accused of having low cognitive function. In the same year, he died of a urinary tract infection. [Results] In the case of the disease, the degree of depigmentation of the livedo core and the degree of depigmentation of the medium-dark matter were relatively high as seen with the naked eye. Histology: Sympathetic ganglia, mediolateral nucleus, posterior root ganglion, dorsal nucleus of vagus ganglion, corpus reticularis, dorsal suture nucleus, livedo nucleus, substantia nigra, inferior part of the optic bed, insular gyrus, basal nucleus of the meridian , transitional olfactory infield, hippocampus CA2, anterior gyrus, anterior, lateral, and parietal lobes, small body pathological recognition, DLB コンセンサスガイドラインで辺縁system ないしは neocortical type にshould be する広がりであった. [Conclusion] The symptoms are as follows, and the progress of corpuscular pathology is discussed in the brain-first versus The body-first仮说においてはlater should be the same, and the black one should be the same as the black one.また, hippocampus CA2 corpuscle pathology is a strong point い, PAF is a cognitive function, it is necessary to pay attention to, support what you see, and support.