Confirmation of virtual unmyelinated fiber absence in hereditary sensory neuropathy type IV

Hans H. Goebel, Stefan Veit, Peter James Dyck

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64 Scopus citations

Abstract

Sural nerves from two unrelated young boys were virtually without unmyelinated fibers (UFs). Small myelinated fibers (MFs) may also have been slightly reduced in number. Since no degeneration or regeneration is observed, UF absence is assumed to be congenital, due to either lack of neuron formation or to premature degeneration. The main clinical features of this inherited sensory neuropathy (previously identified by us as type IV) are the inherited nature and abnormality of nociception, of sweating, and of thermal regulation associated with mild mental retardation. Our findings confirm the congenital absence of UFs of cutaneous nerves in cases such as these and provide further evidence that this disorder has a different natural history and pathology than do the other three types of hereditary sensory neuropathy.

Original languageEnglish (US)
Pages (from-to)670-675
Number of pages6
JournalJournal of Neuropathology and Experimental Neurology
Volume39
Issue number6
DOIs
StatePublished - Nov 1980

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Neurology
  • Clinical Neurology
  • Cellular and Molecular Neuroscience

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