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Brain, Vol. 119, No. 3, 765-774, 1996
© 1996 Guarantors of Brain

research-article

Focal upper limb demyelinating neuropathy

P. K. Thomas1,1, D. Claus4, A. Jaspert4, J. M. Workman1, R. H. M. King1, A. J. Larner2, Milne Anderson2, J. A. Emerson3 and I. T. Ferguson3

1Department of Clinical Neurosciences, Royal Free Hospital School of Medicine London 2Midland Centre for Neurology and Neurosurgery Bristol, UK 3Southmead Hospital Bristol, UK 4Neurologische Klinik der Universität Erlangen-Nurnberg Germany

Correspondence to: Correspondence to: Professor P. K. Thomas, Clinical Neurosciences, Royal Free Hospital School of Medicine, Rowland Hill Street, London NW3 2PF, UK

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.

neuropathy; inflammatory demyelination


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