Multiple system atrophy in Hokkaido, Japan: a prospective registry study of natural history and symptom assessment scales followed for 5 years
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 Pa...
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BMJ Publishing Group
2021-02-01
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| Series: | BMJ Open |
| Online Access: | https://bmjopen.bmj.com/content/11/2/e045100.full |
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| author | Norihiro Sato Akiko Tamakoshi Ichiro Yabe Hidenao Sasaki Asako Takei Masaaki Matsushima Ken Sakushima Yasuhiro Kanatani Naoki Nishimoto Takeshi Matsuoka Jun Sawada Haruo Uesugi Kazuya Sako Shun Shimohama Seiji Kikuchi |
| author_facet | Norihiro Sato Akiko Tamakoshi Ichiro Yabe Hidenao Sasaki Asako Takei Masaaki Matsushima Ken Sakushima Yasuhiro Kanatani Naoki Nishimoto Takeshi Matsuoka Jun Sawada Haruo Uesugi Kazuya Sako Shun Shimohama Seiji Kikuchi |
| author_sort | Norihiro Sato |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-aec6b9d1912147dfb19ddf3fbb9c81dc |
| institution | Kabale University |
| issn | 2044-6055 |
| language | English |
| publishDate | 2021-02-01 |
| publisher | BMJ Publishing Group |
| record_format | Article |
| series | BMJ Open |
| spelling | doaj-art-aec6b9d1912147dfb19ddf3fbb9c81dc2024-11-19T06:00:09ZengBMJ Publishing GroupBMJ Open2044-60552021-02-0111210.1136/bmjopen-2020-045100Multiple system atrophy in Hokkaido, Japan: a prospective registry study of natural history and symptom assessment scales followed for 5 yearsNorihiro Sato0Akiko Tamakoshi1Ichiro Yabe2Hidenao Sasaki3Asako Takei4Masaaki Matsushima5Ken Sakushima6Yasuhiro Kanatani7Naoki Nishimoto8Takeshi Matsuoka9Jun Sawada10Haruo Uesugi11Kazuya Sako12Shun Shimohama13Seiji Kikuchi142 Clinical Research and Medical Innovation Center, Hokkaido University Hospital, Sapporo, Hokkaido, JapanDepartment of Public Health, Hokkaido University, Sapporo, Hokkaido, Japan4 Department of Neurology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Sapporo, JapanDepartment of Neurology, Hokkaido University Graduate School of Medicine, Sapporo, Hokkaido, JapanHokuyukai Neurological Hospital, Sapporo, Hokkaido, Japan4 Department of Neurology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Sapporo, JapanDepartment of Neurology, Hokkaido University Graduate School of Medicine, Sapporo, Hokkaido, JapanDepartment of Health Crisis Management, National Institute of Public Health, Wako, Saitama, JapanDepartment of Biostatistics, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Sapporo, Hokkaido, JapanDepartment of Neurology, Date Red Cross Hospital, Date, Hokkaido, JapanCardiovascular, Respiratory and Neurology Division, Department of Internal Medicine, Asahikawa Medical University, Asahikawa, Hokkaido, JapanDepartment of Medical Service, Aizen Hospital, Sapporo, Hokkaido, JapanDepartment of Neurology, Nakamura Memorial Hospital, Sapporo, Hokkaido, JapanDepartment of Neurology, Sapporo Medical University School of Medicine, Sapporo, Hokkaido, JapanHokkaido Medical Center, Sapporo, Hokkaido, JapanObjectives 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.https://bmjopen.bmj.com/content/11/2/e045100.full |
| spellingShingle | Norihiro Sato Akiko Tamakoshi Ichiro Yabe Hidenao Sasaki Asako Takei Masaaki Matsushima Ken Sakushima Yasuhiro Kanatani Naoki Nishimoto Takeshi Matsuoka Jun Sawada Haruo Uesugi Kazuya Sako Shun Shimohama Seiji Kikuchi Multiple system atrophy in Hokkaido, Japan: a prospective registry study of natural history and symptom assessment scales followed for 5 years BMJ Open |
| title | Multiple system atrophy in Hokkaido, Japan: a prospective registry study of natural history and symptom assessment scales followed for 5 years |
| title_full | Multiple system atrophy in Hokkaido, Japan: a prospective registry study of natural history and symptom assessment scales followed for 5 years |
| title_fullStr | Multiple system atrophy in Hokkaido, Japan: a prospective registry study of natural history and symptom assessment scales followed for 5 years |
| title_full_unstemmed | Multiple system atrophy in Hokkaido, Japan: a prospective registry study of natural history and symptom assessment scales followed for 5 years |
| title_short | Multiple system atrophy in Hokkaido, Japan: a prospective registry study of natural history and symptom assessment scales followed for 5 years |
| title_sort | multiple system atrophy in hokkaido japan a prospective registry study of natural history and symptom assessment scales followed for 5 years |
| url | https://bmjopen.bmj.com/content/11/2/e045100.full |
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