Brief communication participation in social activities of six individuals with advanced-stage hereditary motor sensory neuropathy with proximal dominant involvement (HMSN-P)

The intractable neurological disease HMSN-P (hereditary motor and sensory neuropathy with proximal dominant involvement) includes four-limb paralysis and resembles amyotrophic lateral sclerosis (ALS). Although the long-term employment and social activities of individuals with severe neurological diseases have increased with improvements in medical/welfare services, those of individuals with HMSN-P have not been reported. We analyzed the long-term outcomes of six individuals with advanced-stage HMSN-P treated in 2018–2023; one exhibited proximal dominant quadriplegia in 2018 and was using an electric wheelchair with full assistance. He had continued to attend Sanshin concerts for 5 years and organized a music festival for the local handicapped population. The other patients similarly exhibited quadriplegia; three were using electric wheelchairs with full assistance, and two were using pushchairs. Four attended music festivals for the handicapped, and all have completed 20–35 years' continuous employment, attended public meetings about HMSN-P, and actively participated in the meetings' discussions.