Multiple system atrophy in Hokkaido, Japan : a prospective registry study of natural history and symptom assessment scales followed for 5 years.

Matsushima, Masaaki; Yabe, Ichiro; Sakushima, Ken; Kanatani, Yasuhiro; Nishimoto, Naoki; Matsuoka, Takeshi; Sawada, Jun; Uesugi, Haruo; Sako, Kazuya; Takei, Asako; Tamakoshi, Akiko; Shimohama, Shun; Sato, Norihiro; Kikuchi, Seiji; Sasaki, Hidenao

Multiple system atrophy in Hokkaido, Japan : a prospective registry study of natural history and symptom assessment scales followed for 5 years.

Matsushima, Masaaki; Yabe, Ichiro; Sakushima, Ken et al.

BMJ open, 2020, 11(2), e045100

Permalink : https://hdl.handle.net/2115/80473

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アクセス権	open access
URI	https://hdl.handle.net/2115/80473
タイトル	Multiple system atrophy in Hokkaido, Japan : a prospective registry study of natural history and symptom assessment scales followed for 5 years.
著者	Matsushima, Masaaki ; Yabe, Ichiro ; Sakushima, Ken ; Kanatani, Yasuhiro ; Nishimoto, Naoki ; Matsuoka, Takeshi ; Sawada, Jun ; Uesugi, Haruo ; Sako, Kazuya ; Takei, Asako ; Tamakoshi, Akiko ; Shimohama, Shun ; Sato, Norihiro ; Kikuchi, Seiji ; Sasaki, Hidenao
著者名 Matsushima, Masaaki
e-Rad_Researcher 60372273 著者名 Yabe, Ichiro
著者名 Sakushima, Ken
著者名 Kanatani, Yasuhiro
e-Rad_Researcher 90599630 著者名 Nishimoto, Naoki
著者名 Matsuoka, Takeshi
著者名 Sawada, Jun
著者名 Uesugi, Haruo
著者名 Sako, Kazuya
著者名 Takei, Asako
e-Rad_Researcher 90236737 著者名 Tamakoshi, Akiko
著者名 Shimohama, Shun
著者名 Sato, Norihiro
著者名 Kikuchi, Seiji
e-Rad_Researcher 80281806 著者名 Sasaki, Hidenao
言語	英語
発行日	2020-12
出版者	BMJ Publishing Group
収録物名	BMJ open
巻(号)	11(2)
ページ	e045100
抄録	Objectives Multiple system atrophy (MSA) is a refractory neurodegenerative disease, but novel treatments are anticipated. An accurate natural history of MSA is important for clinical trials, but is insufficient. This regional registry was launched to complement clinical information on MSA. Setting Patient recruitment started in November 2014 and is ongoing at the time of submission. The number of participating facilities was 66. Postal surveys were sent to medical facilities and patients with MSA in Hokkaido, Japan. Participants After obtaining written consent from 196 participants, 184 overview surveys and 115 detailed surveys were conducted. Primary and secondary outcome measures An overview survey evaluated conformity to diagnostic criteria and a detailed survey implemented an annual assessment based on the Unified Multiple System Atrophy Rating Scale (UMSARS). Results At the time of registration, 58.2% of patients were diagnosed with cerebellar symptoms predominant type MSA (MSA-C) and 29.9% were diagnosed with parkinsonism predominant type MSA (MSA-P). UMSARS Part Ⅳ score of 4 or 5 accounted for 53.8% of participants. The higher the UMSARS Part Ⅳ score, the higher the proportion of MSA-P. At baseline, levodopa was used by 69 patients (37.5%) and the average levodopa dose was 406.7?mg/day. The frequency of levodopa use increased over time. Eleven cases changed from MSA-C to MSA-P during the study, but the opposite was not observed. Information about survival and causes of death was collected on 54 cases. Half of deaths were respiratory-related. Sudden death was recorded even in the group with UMSARS Part Ⅳ score of 1. Conclusions This study is the first large-scale prospective MSA cohort study in Asia. MSA-C was dominant, but the use of antiparkinsonian drugs increased over the study period. Changes from MSA-C to MSA-P occurred, but not vice versa.
資源タイプ	学術雑誌論文
出版タイプ	Version of Record
権利情報	Creative Commons Attribution-NonCommercial 4.0 International
PISSN	2044-6055
関連情報	PMID 33558361
関連情報 (isIdenticalTo)	出版社版DOI https://doi.org/10.1136/bmjopen-2020-045100