Infantile chronic peripheral neuropathy with giant axons. Report of a case

An 8-year-old boy with a slowly progressive motor neuropathy is described. The first signs appeared at the age of 3 years. Histological examination of the sural nerve showed the presence of numerous segmental axonal swellings and features of demyelination as well as remyelination. These enlargements...

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Bibliographic Details
Published inActa neuropathologica Vol. 37; no. 3; p. 213
Main Authors Jedrzejowska, H, Drac, H
Format Journal Article
LanguageEnglish
Published Germany 01.01.1977
Subjects
Axons - ultrastructure
Child
Demyelinating Diseases
Humans
Male
Microscopy, Electron
Neurofibrils - ultrastructure
Peripheral Nervous System Diseases - pathology
Spinal Nerves - pathology
Sural Nerve - pathology
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Summary:An 8-year-old boy with a slowly progressive motor neuropathy is described. The first signs appeared at the age of 3 years. Histological examination of the sural nerve showed the presence of numerous segmental axonal swellings and features of demyelination as well as remyelination. These enlargements were filled with irregularly orientated 10 nm filaments. The case resembled the previously described cases of giant axonal neuropathy but differed from them in absence of kinky hair.
ISSN:0001-6322
DOI:10.1007/BF00686881