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論文
タイトル
タイトル(英)
Long-standing preservation of levodopa response in progressive supranuclear palsy.
参照URL
https://researchmap.jp/masatohasegawa/published_papers/48213064
著者
著者(英)
Terunori Sano,Masashi Mizutani,Tasuku Ishihara,Norikazu Hara,Akinori Miyashita,Takeshi Ikeuchi,Masato Hasegawa,Yuji Takahashi,Masaki Takao
担当区分
概要
概要(英)
The clinical and neuropathological characteristics of progressive supranuclear palsy (PSP) with preservation of levodopa (L-dopa) response are described in this report. We present the case of a 73-year-old Japanese man with a 13-year history of dopa-responsive Parkinsonism and abnormalities observed in metaiodobenzylguanidine (MIBG) myocardial scintigraphy, suggesting Parkinson's disease. However, autopsy results revealed PSP pathology, including tuft-shaped astrocytes and globose-type neurofibrillary tangles, without Lewy body pathology. The degeneration was moderately to severely distributed in the globus pallidus, subthalamic nucleus, and substantia nigra, whereas striatal degeneration was mild. These findings suggest an intact response to L-dopa therapy throughout the patient's lifetime. Pathological examination of cardiac sympathetic nerves revealed intact nerves, suggesting functional involvement in the MIBG abnormality. This study provides further evidence of the clinical and pathological heterogeneity of PSP. Homozygosity for both the rs564309-C allele at TRIM11 and the rs2242367-G allele at SLC2A13 might have played a protective role. This case indicates a protracted course-PSP, which may hold promise for future treatments.
出版者・発行元
出版者・発行元(英)
誌名
誌名(英)
Journal of the neurological sciences
466
開始ページ
123203
終了ページ
123203
出版年月
2024年8月29日
査読の有無
招待の有無
掲載種別
研究論文(学術雑誌)
ISSN
DOI URL
https://doi.org/10.1016/j.jns.2024.123203
共同研究・競争的資金等の研究課題
研究者