Author:
Auer Roland N.,Bell Robert B.,Lee Mary Anne
Abstract
ABSTRACT:A thirty-eight-year-old man presented with a six year history of symptoms resembling an anterior horn cell disorder. There was progressive upper extremity wasting and weakness in the absence of sensory complaints. Electrophysiologic abnormalities were confined to motor nerve conduction and indicated a demyelinating process involving the brachial plexus and major proximal upper extremity nerve trucks bilaterally. Biopsy of the proximal right ulnar nerve revealed changes suggesting a chronic demyelinating process, and onion-bulb formations were present. Immunohistochemical staining for S-100 protein was positive in the cells comprising the onion-bulbs, indicating a Schwann cell, not a perineurial origin of these cells. After 8 years, symptoms have failed to appear in the lower limbs. Recent reports in the literature have begun to delineate the syndrome, which appears to represent an unusual, localized or multifocal, sometimes inflammatory, clinically benign neuropathy that can mimic motor neuron disease in its earlier stages. We report the first such case with underlying pathology.
Publisher
Cambridge University Press (CUP)
Subject
Clinical Neurology,Neurology,General Medicine
Reference17 articles.
1. Localized hypertrophic neuropathy - a rare and clinically almost unknown syndrome;Vallat;Clin Neuropathol,1986
2. Localized hypertrophic neuropathy: a perineurialoma?;Gullotta;Clin Neuropathol,1985
3. Perineurioma: A Benign Peripheral Nerve Tumor
4. Experimental hypertrophic neuropathy: an electron microscope study.
5. Demyelination and remyelination in lead neuropathy: Electron microscopic studies;Lampert;J Neuropathol Exp Neurol,1968
Cited by
78 articles.
订阅此论文施引文献
订阅此论文施引文献,注册后可以免费订阅5篇论文的施引文献,订阅后可以查看论文全部施引文献