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August 1994

Lupus Erythematosus and Miller-Fisher Syndrome

Author Affiliations

From the Departments of Internal Medicine (Drs Bingisser, Speich, Fontana, and Gmür) and Neurology (Drs Vogel and Landis), University Hospital, Zürich, Switzerland.

Arch Neurol. 1994;51(8):828-830. doi:10.1001/archneur.1994.00540200108024

Objective:  To compare the clinical course of an unusual case of Miller-Fisher syndrome in systemic lupus erythematosus with therapeutic interventions, in particular with plasma exchanges.

Design:  The clinical state and laboratory and electrophysiologic parameters were controlled for over a year and related to therapeutic attempts with immunoglobulins, steroids, and plasma exchanges.

Setting:  Medical intensive care unit of a university hospital.

Patient:  A 17-year-old black female student with known systemic lupus erythematosus who developed ataxia, areflexia, and ophthalmoplegia (Miller-Fisher syndrome) and later became tetraplegic and required full mechanical ventilatory support.

Results:  High-dose immunoglobulin treatment combined with corticosteroid pulse therapy was not beneficial. However, plasma exchange (performed five times over a period of 4 months) was followed by a striking clinical improvement within hours after each plasma exchange.

Conclusions:  Plasma exchange appears to remove a yet unknown agent producing a distal motor nerve conduction block and is efficacious in severe neuropathy associated with Miller-Fisher syndrome in lupus erythematosus.

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