Hypertrophic perineurial dysplasia in multifocal and generalized peripheral neuropathies.

Abstract:

:Two cases are described, one with a multifocal cranial and limb neuropathy of adult onset associated with optic neuropathy, and the other with a diffuse demyelinating neuropathy characterized by congenital cataract, mental retardation and progressive lower limb paresis with an onset in childhood. Extensive investigation in both failed to establish the causation. No family history of similar disorder was obtained in either case. Nerve biopsy in both showed similar perineurial abnormalities, the endoneurium being compartmentalized by hypertrophic perineurial cells that exhibited dysplastic features. The appearances resemble those described in a previously reported case of multifocal neuropathy and probably represent an unusual but non-specific response to a peripheral neuropathy.

authors

Thomas PK,King RH,Workman JM,Schröder JM

doi

10.1046/j.0305-1846.2000.00289.x

keywords:

subject

Has Abstract

pub_date

2000-12-01 00:00:00

pages

536-43

issue

6

eissn

0305-1846

issn

1365-2990

pii

nan289

journal_volume

26

pub_type

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