Ultrastructural aspects of nerves, bones, and vessels in hereditary sensory neuropathy

This study concerns two children, from the same otherwise normal family, presenting progressive spontaneous amputations of the limbs, suggesting peripheral hereditary sensory neuropathy. Samples from the distal nerves and vessels and the distal tibial epiphyses were examined ultrastructurally. The n...

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Bibliographic Details
Published inJournal of orthopaedic research Vol. 3; no. 2; p. 226
Main Authors Teot, L, Arnal, F, Humeau, C, Pous, J G, Dimeglio, A
Format Journal Article
LanguageEnglish
Published United States 1985
Subjects
Arterioles - ultrastructure
Bone and Bones - blood supply
Bone and Bones - ultrastructure
Bone Resorption
Child
Collagen - biosynthesis
Electromyography
Female
Femoral Nerve - ultrastructure
Hereditary Sensory and Autonomic Neuropathies - pathology
Humans
Male
Osteocytes - ultrastructure
Pedigree
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Summary:This study concerns two children, from the same otherwise normal family, presenting progressive spontaneous amputations of the limbs, suggesting peripheral hereditary sensory neuropathy. Samples from the distal nerves and vessels and the distal tibial epiphyses were examined ultrastructurally. The nerves showed signs of degeneration, and a typical bone necrosis, without osteoclasts, was observed in the 2 cm margin of the samples. Simultaneous modifications of the vessels suggest a neurally initiated vascular reflex resulting in bone destruction. Theories that might explain the observations are discussed.
ISSN:0736-0266
DOI:10.1002/jor.1100030213