Rinsho Shinkeigaku (Clinical Neurology)

Original Article

Study of care practices for patients with myotonic dystrophy in Japan
-Nationwide patient survey

Masanori P. Takahashi, M.D., Ph.D.1), Risa Yamamoto, B.S.1), Tomoya Kubota, M.D., Ph.D.1), Tohru Matsuura, M.D.2), Keiko Ishigaki, M.D., Ph.D.3), Yoshihide Sunada, M.D., Ph.D.4), Hirofumi Komaki, M.D., Ph.D.5), Hiroto Takada, M.D., Ph.D.6), Satoshi Kuru, M.D., Ph.D.7) and Tsuyoshi Matsumura, M.D., Ph.D.8)

1)Department of Functional Diagnostic Science, Osaka University Graduate School of Medicine
2)Division of Neurology, Department of Medicine, Jichi Medical University School of Medicine
3)Department of Pediatrics, Tokyo Women's Medical University, School of Medicine
4)Department of Neurology, Kawasaki Medical School, Kurashiki
5)Translational Medical Center, National Center of Neurology and Psychiatry
6)Department of Neurology, National Hospital Organization Aomori National Hospital
7)Department of Neurology, National Hospital Organization Suzuka National Hospital
8)Department of Neurology, National Hospital Organization Osaka Toneyama Medical Center

We conducted a comprehensive anonymous questionnaire survey on medical care and treatment for patients with myotonic dystrophy, who registered in the Japanese national registry (Remudy) or were undergoing care in seven hospitals specializing neuromuscular diseases. The questionnaire consisted of 49 questions were distributed to 813 patients, and 342 valid responses were collected. Most prevalent symptoms or complaints were dysfunction of fingers and fatigue. One-third of the adult patients left the job, half of which was due to the disease. Twelve percent of the patients did not visit the specialist regularly, the main reason being distance. The most common reason that the patients did not follow the advice of using a ventilator by medical professionals was lack of feeling the need. One-fourth of the adult female patients had infertility treatment, 80% of which was before a diagnosis of this disorder. This first-time nationwide survey revealed the actual condition of Japanese patients with myotonic dystrophy and raised various carerelated issues.
Original Questionnaire (English)
Full Text of this Article in PDF (1189K)

(CLINICA NEUROL, 60: 130|136, 2020)
key words: myotonic dystrophy, patient registry, questionnaire, survey, patient care

(Received: 20-Jul-19)