A new type of non-progressive sensory neuropathy in children with atypical dysautonomia.
Three cases of non-progressive, sensory neuropathy with dysautonomia are presented. Light and electron microscopy on whole sural nerve biopsies revealed an almost total lack of myelinated nerve fibres. The total fibre count was also reduced as was the total number of Schwann cell nuclei. No degenerative phenomena were seen within the nerve fibres. The aberrations are probably caused by a maldevelopment of the neural crest implying a stunted proliferation and growth of sensory and autonomous neurones as well as a reduced proliferation of Schwann cells. Since the morphology and clinical features differ from that in other cases of sensory neuropathy with dysautonomia the three present cases are considered to represent a new type of the disease.
D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D004402:Dysautonomia, Familial / Q000150:complications* / Q000473:pathology; D005260:Female; D006801:Humans; D008854:Microscopy, Electron; D009413:Nerve Fibers, Myelinated / Q000648:ultrastructure; D010523:Peripheral Nervous System Diseases / Q000150:complications* / Q000473:pathology; D012583:Schwann Cells / Q000648:ultrastructure; D013497:Sural Nerve / Q000648:ultrastructure