Objective: To investigate epidemiological information of multiple system atrophy (MSA) patients in Hokkaido by using the data from the Hokkaido Rare-disease Consortium for MSA Extended (HoRC-MSA EX).
Background: MSA is an intractable neurodegenerative disease whose main symptoms are autonomic dysfunction, cerebellar ataxia, and parkinsonism. There is a lack of long-term, large-scale epidemiological data on MSA, so data collection and analysis are important. On the other hand, since its development in the 1800s, Hokkaido has had many residents who were immigrants from all over Japan, making it a microcosm of Japan as a whole. Hokkaido also has a relatively low population turnover, making it well suited to conducting epidemiological studies of MSA.
Method: We started recruiting MSA patients in November 2014. Postal surveys were sent to medical facilities and MSA patients. Clinical information is collected from attending doctors and patients at the time of registration and annually.
Results: As of February 28, 2026, informed consent to this study was obtained from 288 MSA patients. Diagnostic criteria and their activity of daily living (ADL) were examined for 273 patients. MSA-C was 62% at the time of registration and decreased over time. The group considered ambulatory (UMSARS part 4 ≤ 3, good ADL group) comprised 57% at the initial assessment, with MSA-C accounting for 74% of this group. In contrast, within the group corresponding to walking difficulties (UMSARS part 4 ≥ 4, poor ADL group), 44% were diagnosed with MSA-C. Furthermore, at the initial assessment, the rates of indwelling urinary catheter use, rigidity, and levodopa usage were 5%, 43%, and 32%, respectively, in the good ADL group, while they were 44%, 65%, and 50%, respectively, in the poor ADL group. Notably, nearly all cases in the good ADL group transitioned to the poor ADL group during the five-year observation period.
Conclusion: HoRC-MSA EX has accumulated extensive long-term epidemiological data on MSA patients. This analysis suggests that parkinsonism influence declines in ADL. Management of parkinsonism is considered important in the care of MSA patients.
Further detailed consideration is required in the future.
To cite this abstract in AMA style:
M. Matsushima, K. Sakushima, N. Nishimoto, J. Sawada, T. Matsuoka, H. Uesugi, N. Minami, K. Sako, A. Takei, S. Hisahara, Y. Kanatani, A. Tamakoshi, N. Sato, I. Yabe. The epidemiological study of multiple system atrophy beginning in Hokkaido, Japan 2014-2026 [abstract]. Mov Disord. 2026; 41 (suppl 1). https://www.mdsabstracts.org/abstract/the-epidemiological-study-of-multiple-system-atrophy-beginning-in-hokkaido-japan-2014-2026/. Accessed October 1, 2026.« Back to 2026 International Congress
MDS Abstracts - https://www.mdsabstracts.org/abstract/the-epidemiological-study-of-multiple-system-atrophy-beginning-in-hokkaido-japan-2014-2026/