Hostname: page-component-8448b6f56d-cfpbc Total loading time: 0 Render date: 2024-04-19T05:30:26.963Z Has data issue: false hasContentIssue false
  • English
  • Français

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
Rights & Permissions [Opens in a new window]

Abstract:

Core share and HTML view are not available for this content. However, as you have access to this content, a full PDF is available via the ‘Save PDF’ action button.

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

REFERENCES

1.Lewis, RA, Summer, AJ, Brown, MJ, et al. Multifocal demyelinating neuropathy with persistent conduction block. Neurology (NY) 1982; 32: 958–964.CrossRefGoogle ScholarPubMed
2.Adams, RD, Asbury, AK, Micklesen, JJ, Multifocal pseudohypertrophic neuropathy. Trans Amer Neurol Assoc 1965; 90: 30–34.Google ScholarPubMed
3.Bradley, WG, Bennett, RK, Good, P, et al. Proximal chronic inflammatory polyneuropathy with multifocal conduction block. Arch Neurol 1988; 45: 451–455.CrossRefGoogle ScholarPubMed
4.Chad, DA, Hammer, K, Sargent, J, Slow resolution of multifocal weakness and fasciculation: a reversible motor neuron syndrome. Neurology 1986; 36: 1260–1263.CrossRefGoogle ScholarPubMed
5.Parry, GJ, Clarke, S, Multifocal acquired demyelinating neuropathy masquerading as motor neuron disease. Muscle Nerve 1988; 11: 103–107.CrossRefGoogle ScholarPubMed
6.Roth, G, Rohr, J, Magistris, MR, et al. Motor neuropathy with proximal multifocal persistent conduction block, fasciculations and myokymia. Evolution to tetraplegia. Eur Neurol 1986; 25: 416–423.CrossRefGoogle ScholarPubMed
7.van den Bergh, P, Logigian, EL, Kelly, JJ Jr,. Motor neuropathy with multifocal conduction blocks. Muscle Nerve 1989; 11: 26–31.CrossRefGoogle Scholar
8.Dyck, PJ, Experimental hypertrophic neuropathy: pathogenesis of onion-bulb formations produced by repeated tourniquet applications. Arch Neurol 1969; 21: 73–95.CrossRefGoogle ScholarPubMed
9.Lampert, PW, Schochet, SS Jr. Demyelination and remyelination in lead neuropathy: Electron microscopic studies. J Neuropathol Exp Neurol 1968; 27: 527–545.Google ScholarPubMed
10.Wellers, RO, Das Gupta, TK, Experimental hypertrophic neuropathy: an electron microscope study. J Neurol Neurosurg Psychiatry 1968; 31: 34–42.CrossRefGoogle Scholar
11.Cusimanos, MD, Bilbao, JM, Cohen, SM, Hypertrophic brachial plexus neuritis: a pathological study of two cases. Ann Neurol 1988; 24: 615–622.CrossRefGoogle Scholar
12.Bilbao, JM, Khoury, NJS, Hudson, AR, et al. Perineurioma (Localized hypertrophic neuropathy). Arch Pathol Lab Med 1984; 108: 557–560.Google ScholarPubMed
13.Boker, D-K, Schonberg, F, Gullotta, F, Localized hypertrophic neuropathy ¯ a rare, clinically almost unknown syndrome. Clin Neuropathol 1984; 3: 228–230.Google ScholarPubMed
14.Tranmer, BI, Bilbao, JM, Hudson, AR, Perineurioma: a benign peripheral nerve tumour. Neurosurgery 1986; 19: 134–137.CrossRefGoogle Scholar
15.Ohno, T, Park, P, Akai, M, et al. Ultrastructural study of a perineurioma. Ultrastruct Pathol 1988; 12: 495–504.CrossRefGoogle ScholarPubMed
16.Gullotta, F. Localized hypertrophic neuropathy: a perineurialoma?. Clin Neuropathol 1985; 4: 92.Google ScholarPubMed
17.Vallat, JM, Vital, C, Localized hypertrophic neuropathy - a rare and clinically almost unknown syndrome. Clin Neuropathol 1986; 5: 230.Google ScholarPubMed