Sensory nerve pathology in multifocal motor neuropathy.
The nosological status of multifocal motor neuropathy remains controversial. The clinical and electrodiagnostic hallmarks suggest selective motor fiber involvement. In this study, we asked to what extent sensory nerves might be involved pathologically in multifocal motor neuropathy. Examination of sensory nerve biopsy specimens from 11 patients did reveal pathological findings in all, but they were very mild. An increased number of thinly myelinated, large-caliber fibers was the unifying feature common to each specimen. By electron microscopy, each biopsy specimen had thinly myelinated fibers surrounded by minor onion bulbs. Active demyelination, though scant, was seen in 3 nerves. Myelinated fiber density was normal. Subperineurial edema and inflammation were not present. We conclude that multifocal motor neuropathy is not an exclusively motor abnormality, although it appears to be so clinically and electrophysiologically. The frequent, albeit mild, pathological abnormalities in sensory fibers suggest that the demyelinating pathophysiology also affects sensory fibers, but to a lesser degree than motor fibers. Some investigators maintain that multifocal motor neuropathy is within the spectrum of chronic inflammatory demyelinating polyneuropathy. The very mild degree of sensory fiber involvement, the absence of inflammation or edema, and the distinctive clinical features support the concept of multifocal motor neuropathy as distinct from chronic inflammatory demyelinating polyneuropathy.
Corse
A M
AM
Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD 21287-7519, USA.
Chaudhry
V
V
Crawford
T O
TO
Cornblath
D R
DR
Kuncl
R W
RW
Griffin
J W
JW
D000328:Adult; D001369:Axons / Q000648:ultrastructure; D003711:Demyelinating Diseases / Q000473:pathology; D005260:Female; D015417:Hereditary Sensory and Motor Neuropathy / Q000134:cerebrospinal fluid / Q000473:pathology* / Q000503:physiopathology; D006801:Humans; D007075:Immunoglobulin M / Q000097:blood; D008297:Male; D008854:Microscopy, Electron; D008875:Middle Aged; D009434:Neural Pathways; D009475:Neurons, Afferent / Q000473:pathology*; D012583:Schwann Cells / Q000648:ultrastructure; D012720:Severity of Illness Index; D013497:Sural Nerve / Q000473:pathology* / Q000648:ultrastructure
D007075:Immunoglobulin M