Focal upper limb demyelinating neuropathy

• Published in: Brain (London, England : 1878) Vol. 119; no. 3; pp. 765 - 774
• Main Authors: Thomas, P. K., Claus, D., Jaspert, A., Workman, J. M., King, R. H. M., Larner, A. J., Anderson, Milne, Emerson, J. A., Ferguson, I. T.
• Format: Journal Article
• Language: English
• Published: England Oxford University Press 01-06-1996; Oxford Publishing Limited (England)
• Subjects: Adolescent; Adult; Aged; Arm - innervation; Arm - physiopathology; Biopsy; Demyelinating Diseases - diagnosis; Demyelinating Diseases - physiopathology; Enzyme-Linked Immunosorbent Assay; Female; G(M1) Ganglioside - blood; G(M1) Ganglioside - immunology; Humans; inflammatory demyelination; Male; Microscopy, Electron; Middle Aged; Motor Neuron Disease - pathology; Motor Neuron Disease - physiopathology; Muscle Contraction - physiology; Neural Conduction - physiology; neuropathy; Ulnar Nerve - pathology; Ulnar Nerve - physiopathology; Ulnar Nerve - ultrastructure
• ISSN: 0006-8950; 1460-2156
• DOI: 10.1093/brain/119.3.765

Summary Observations are presented on nine selected patients with chronic upper limb demyelinating neuropathy to illustrate the range of manifestations that may be observed. In three, the involvement was purely motor, in five, mixed motor and sensory and, in one, virtually purely sensory; in seven the symptoms were unilateral and in two bilateral. The presence of reduced nerve conduction velocity and conduction block and the response to treatment in seven of the cases indicate that they represented examples of chronic inflammatory demyelinating polyneuropathy (CIDP) with focal involvement. This was confirmed by nerve biopsy in two cases. The presentation in one patient was accompanied by forearm swelling initially suspected of being a tumour but shown to be due to muscle hypertrophy. This was probably the consequence of recurrent muscle cramps and fasciculation and possibly neuromyotonia. The patient with predominant sensory involvement restricted to the upper limbs demonstrates that sensory CIDP can present focally. In one patient with monomelic motor and sensory involvement, nerve biopsy showed multifical areas of hypertrophic demyelinating neuropathy distally in the ulnar nerve without inflammatory infiltration. This patient failed to respond to therapy. Response in the others was satisfactory, although one patient with a monomelic motor neuropathy showed a severe deterioration after being given corticosteroids; he subsequently improved with intravenous human immunoglobulin therapy.