2021 Volume 53 Issue 2 Pages 118-123
Objective: It is imperative to sufficiently manage the daily life of pediatric patients with immune-mediated neuromuscular diseases, such as myasthenia gravis (MG) and chronic inflammatory demyelinating polyneuropathy (CIDP), for proper growth and development. However, current evidence on management practice is unclear and many pediatric neurologists have unresolved questions. We therefore conducted a multi-center questionnaire survey to establish a more standardized management plan. Methods: In February 2017, we distributed a written questionnaire to members of the Pediatric Immune-mediated Neuromuscular Diseases Study Group (82 members in 67 facilities at the time of survey). Results: Completed questionnaires were obtained from 15 respondents in 15 facilities. Each facility treated an average of 5.5 patients with MG and 0.3 patients with CIDP. We observed differences among the facilities in terms of immunization schedules, ophthalmological examinations, and prophylactic medication for the adverse effects of corticosteroids, which included infections, peptic ulcers, and osteoporosis. It was particularly evident that pediatricians were uncertain about the usage of anticonvulsants and sedatives, with prescribed benzodiazepine agents in fact being contraindicated for MG patients in Japan. Conclusions: The daily life management plan of pediatric patients with immune-mediated neuromuscular diseases varied considerably among institutions, possibly due to the limited number of patients with MG and CIDP treated at each facility. We therefore propose a guide for the daily life management of children with such diseases. Further investigation of cases and institutions is needed for more useful and effective guidelines.
