Rinsho Shinkeigaku (Clinical Neurology)

Original Article

Inpatients with facioscapulohumeral muscular dystrophy in specialized institutions in Japan from 1999 to 2013-Clinical condition changes and causes of death

Toshio Saito, M.D.1), Satoshi Kuru, M.D.2), Toshiaki Takahashi, M.D.3), Mikiya Suzuki, M.D.4) and Katsuhisa Ogata, M.D.4)

1)Division of Child Neurology, Department of Neurology, National Hospital Organization Toneyama National Hospital
2)Department of Neurology, National Hospital Organization Suzuka National Hospital
3)Department of Neurology, National Hospital Organization Sendai-Nishitaga National Hospital
4)Department of Neurology, National Hospital Organization Higashisaitama Hospital

We analyzed the registration data of inpatients with facioscapulohumeral muscular dystrophy (FSHD) receiving care at 27 specialized institutions for muscular dystrophy in Japan from 1999 to 2013 using data from October 1 of each year. The number of inpatients of each year ranged from 63 to 72 (67.1 ± 3.3) throughout the study period. Those aged over 50 years gradually increased during the study period, while the oldest inpatient was 82.8 years old. Most could not walk. The rate of respirator dependency increased from 21.0% in 1999 to 71.0% in 2013, while the rate of patients receiving oral nutrition was 98.4% in 1999 and then reduced to 75.4% in 2013. There were 36 death cases reported in the database, including 15 patients with respiratory failure and 4 with heart failure. Our findings indicate that FSHD patients in a severe condition are impacted by respiratory and nutritional problems and their prognosis for survival is related to respiratory failure.
Full Text of this Article in Japanese PDF (557K)

(CLINICA NEUROL, 59: 716|722, 2019)
key words: database of wards for inpatients with muscular dystrophy, facioscapulohumeral muscular dystrophy (FSHD), respiratory failure, nutritional support, cause of death

(Received: 23-Sep-18)