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

Abstract

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.

• Authors: L Teot, F Arnal, C Humeau, J G Pous, A Dimeglio
• Journal: Journal of orthopaedic research : official publication of the Orthopaedic Research Society (J Orthop Res) Vol. 3 Issue 2 Pg. 226-35 ( 1985) ISSN: 0736-0266 [Print] United States
• PMID: 3858491 (Publication Type: Case Reports, Journal Article)

Chemical References

• Collagen

Topics

• Arterioles (ultrastructure)
• Bone Resorption
• Bone and Bones (blood supply, ultrastructure)
• Child
• Collagen (biosynthesis)
• Electromyography
• Female
• Femoral Nerve (ultrastructure)
• Hereditary Sensory and Autonomic Neuropathies (pathology)
• Humans
• Male
• Osteocytes (ultrastructure)
• Pedigree