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Hypertrophic perineurial dysplasia in multifocal and generalized peripheral neuropathies
Author(s) -
Thomas P. K.,
King R. H. M.,
Workman J. M.,
Schröder J. M.
Publication year - 2000
Publication title -
neuropathology and applied neurobiology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.538
H-Index - 95
eISSN - 1365-2990
pISSN - 0305-1846
DOI - 10.1046/j.0305-1846.2000.00289.x
Subject(s) - medicine , nerve biopsy , endoneurium , peripheral neuropathy , pathology , mononeuropathy , paresis , plasma cell dyscrasia , optic neuropathy , anatomy , optic nerve , peripheral nerve , surgery , diabetes mellitus , endocrinology , immunoglobulin light chain , antibody , immunology
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.