FOCAL UPPER-LIMB DEMYELINATING NEUROPATHY

Observations are presented on nine selected patients with chronic uppe r limb demyelinating neuropathy to illustrate the range of manifestati ons 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 pres ence of reduced nerve conduction velocity and conduction block and the response to treatment in seven of the cases indicate that they repres ented examples of chronic inflammatory demyelinating polyneuropathy (C IDP) with focal involvement. This was confirmed by nerve biopsy in two cases. The presentation in one patient was accompanied by forearm swe lling initially suspected of being a tumour but shown to be due to mus cle 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 demonst rates that sensory CIDP can present focally. In one patient with monom elic motor and sensory involvement, nerve biopsy showed multifocal are as of hypertrophic demyelinating neuropathy distally in the ulnar nerv e without inflammatory infiltration. This patient failed to respond to therapy. Response in the others was satisfactory, although one patien t with a monomelic motor neuropathy showed a severe deterioration afte r being given corticosteroids; he subsequently improved with intraveno us human immunoglobulin therapy.