LUPUS-ERYTHEMATOSUS AND MILLER-FISHER SYNDROME

Objective: To compare the clinical course of an unusual case of Miller -Fisher syndrome in systemic lupus erythematosus with therapeutic inte rventions, in particular with plasma exchanges. Design: The clinical s tate and Laboratory and electrophysiologic parameters were controlled for over a year and related to therapeutic attempts with immunoglobuli ns, steroids, and plasma exchanges. Setting: Medical intensive care un it of a university hospital. Patient: A 17-year-old black female stude nt with known systemic lupus erythematosus who developed ataxia, arefl exia, and ophthalmoplegia (Miller-Fisher syndrome) and later became te traplegic and required full mechanical ventilatory support. Results: H igh-dose immunoglobulin treatment combined with corticosteroid pulse t herapy was not beneficial. However, plasma exchange (performed five ti mes over a period of 4 months) was followed by a striking clinical imp rovement within hours after each plasma exchange. Conclusions: Plasma exchange appears to remove a yet unknown agent producing a distal moto r nerve conduction block and is efficacious in severe neuropathy assoc iated with Miller-Fisher syndrome in lupus erythematosus.