Survey of Fukuyama type congenital muscular dystrophy in Tokyo

: On the 1st of April in 1988, we identified 26 children with Fukuyama type congenital muscular dystrophy (FCMD) among 1,227,000 children in Tokyo whose ages ranged from 6 to 14 years. The prevalence rate of FCMD was 2.1 per 100,000. All affected children attended special school for crippled children. Mean absence from school on account of illness was 33.9 days in a year. Fifteen among 26 affected children had ability of verbal communication. The loss of gross motor function started at ages 6-7 years but muscle weakness might have occurred earlier. Ten affected children were treated with antiepileptic drugs. Six affected children needed to be hospitalized for the treatment of vomiting with dehydration, acute bronchitis, or pertussis infection during one year until the 1st of April in 1989. Two cases among 26 with FCMD died of respiratory complications shortly after admission.