Lupus erythematosus and Miller-Fisher syndrome.

Abstract

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, are flexia, 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.

• Authors: R Bingisser, R Speich, A Fontana, J Gmür, B Vogel, T Landis
• Journal: Archives of neurology (Arch Neurol) Vol. 51 Issue 8 Pg. 828-30 (Aug 1994) ISSN: 0003-9942 [Print] United States
• PMID: 8042933 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Immunoglobulins, Intravenous
• Cyclophosphamide
• Methylprednisolone

Topics

• Adolescent
• Ataxia (complications, therapy)
• Cyclophosphamide (therapeutic use)
• Female
• Humans
• Immunoglobulins, Intravenous
• Lupus Erythematosus, Systemic (complications, therapy)
• Methylprednisolone (therapeutic use)
• Ophthalmoplegia (complications, therapy)
• Plasma Exchange
• Quadriplegia (complications)
• Reflex, Abnormal
• Syndrome