Peripheral autonomic nervous system lesions in congenital or familial dysautonomia

Abstract

Neuro-pathologic observations are presented in a case of congenital dysautonomia in a male infant, 10 months of age. The predominant finding was one of vacuolar, cytoplasmic changes in the peripheral autonomic nervous system ganglia and plexuses. There were no lesions in the central nervous system. The findings are compared with those of previously autopsied patients. The possibility of varying lesions in the nervous system producing a similar clinical picture is considered. The use of rectal biopsies for investigating cases of dysautonomia is suggested.