Congenital hypo- and hypermyelination neuropathy. Two cases.
Two young patients were referred recently to the authors for investigation of a peroneal atrophy syndrome. Since the first symptoms were observed in infancy, a congenital hypomyelination neuropathy was suspected, and superficial peroneal nerve biopsies were taken. Signs of severe and widespread demyelination/remyelination were observed. These features appeared morphologically similar to those observed in the globular or tomaculous neuropathies. The mechanism of the hypermyelination is discussed.
Vallat
J M
JM
Department of Neurology, University Hospital, Limoges, France.
Gil
R
R
Leboutet
M J
MJ
Hugon
J
J
Moulies
D
D
D002648:Child; D002675:Child, Preschool; D003711:Demyelinating Diseases / Q000151:congenital* / Q000473:pathology; D005260:Female; D006801:Humans; D008297:Male; D008854:Microscopy, Electron; D009186:Myelin Sheath / Q000473:pathology* / Q000648:ultrastructure; D009422:Nervous System Diseases / Q000151:congenital* / Q000473:pathology