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Neuropathy with Onion Bulb Formations and Pure Motor Manifestations

Published online by Cambridge University Press:  18 September 2015

Roland N. Auer ,
Robert B. Bell  and
Mary Anne Lee
Roland N. Auer*
Affiliation:
Departments of Pathology and Clinical Neurosciences, University of Calgary, Calgary
Robert B. Bell
Affiliation:
Departments of Pathology and Clinical Neurosciences, University of Calgary, Calgary
Mary Anne Lee
Affiliation:
Departments of Pathology and Clinical Neurosciences, University of Calgary, Calgary
*
Neuropathology, Health Sciences Center, 3330 Hospital Dr. N.W., Calgary, Alberta, Canada T2N 4N1
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Abstract:

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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.

Type
Original Articles
Information
Canadian Journal of Neurological Sciences , Volume 16 , Issue 2 , May 1989 , pp. 194 - 197
Copyright
Copyright © Canadian Neurological Sciences Federation 1989

References

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