Abstract
We analyzed a sural nerve biopsy of a child with congenital hypomyelinating neuropathy.
A lack of normally myelinated fibres, abnormal architecture of premyelin fibres and
basal lamina onion bulbs were identified. The most prominent pathological finding
was the appearance of significant death of Schwann cells with apoptotic morphology.
This new finding may suggest that abnormal premyelin fibres are susceptible to death
and that their disappearance is responsible for empty basal lamina onion bulb formation.
Keywords
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Article info
Publication history
Accepted:
May 29,
2002
Received in revised form:
May 7,
2002
Received:
July 4,
2001
Identification
Copyright
© 2002 Elsevier Science B.V. Published by Elsevier Inc. All rights reserved.
