• Monami Tarisawa, Masaaki Matsushima, Akihiko Kudo, Ken Sakushima, Yasuhiro Kanatani, Naoki Nishimoto, Jun Sawada, Takeshi Matsuoka, Shin Hisahara, Haruo Uesugi, Naoya Minami, Kazuya Sako, Asako Takei, Akiko Tamakoshi, Norihiro Sato, Hidenao Sasaki, Ichiro Yabe, and HoRC-MSA Study Group.
• Department of Neurology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Japan.
• Intern. Med. 2024 Nov 1; 63 (21): 290329122903-2912.

AbstractObjective In 2022, Wenning et al. proposed the Movement Disorder Society Criteria (MDS criteria) for the Diagnosis of Multiple System Atrophy (MSA). These criteria were expected to provide useful alternatives to the second consensus statement. We examined trends in these diagnostic criteria. Methods We used patient data registered with the Hokkaido Rare Disease Consortium for Multiple System Atrophy, which has been recruiting patients with MSA through medical facilities in Hokkaido since November 2014. Patients were evaluated according to the MDS criteria based on neurological examinations and imaging findings at three separate times: the first evaluation, the time of enrollment (diagnosis), and the most recent evaluation (final evaluation). Results The MDS criteria were examined in 68 of 244 patients enrolled between November 2014 and July 2022. At the initial evaluation, the classifications were as follows: clinically established (n=27; 39.7%); clinically probable (n=13; 19.1%); possible prodromal (n=12; 17.6%); and negative [did not meet criteria (n=16; 23.5%)]. At the time of diagnosis, the classifications were as follows: clinically established (n=45; 66.2%); clinically probable (n=12; 17.6%); possible prodromal (n=4; 5.9%); and negative (n=7; 10.3%). At the final evaluation, the classifications were as follows: clinically established (n=52; 76.5%); clinically probable (n=9; 13.2%); possible prodromal (n=2; 2.9%); and negative (n=5; 7.4%). Conclusion We were able to clarify the changes in the criteria values and transition of patients due to the clarification of imaging and supportive findings in the MDS criteria.

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