A Case of Systemic Lupus Erythematosus Presenting as Guillain-Barré Syndrome.

Helen Chioma Okoh,Spencer Langevin,Adriana Abrudescu,Sandeep Singh Lubana,Susan Sanelli-Russo

doi:10.1155/2015/528026

Abstract

Systemic lupus erythematosus (SLE) is an autoimmune systemic disease with multiple organ involvement with high morbidity and mortality rate. Among the severe potential fatal complications are those of the central and peripheral nervous system which usually develop during the course of the disease and very rarely from the outset of the disease. We are reporting a rare case of Miller-Fisher (MFS) variant of Guillain-Barré syndrome (GBS) as the first manifestation of SLE in a 41-year-old female who progressed to flaccid paralysis with no neurological improvement with initial immunosuppressive therapy, plasmapheresis, and first cycle of intravenous immunoglobulin (IVIG) but with remarkable and complete recovery after the second 5-day course of IVIG.

Highlights

Neuropsychiatric systemic lupus erythematosus (NPSLE) as described by the American College of Rheumatology (ACR) research committee includes 19 neuropsychiatric syndromes divided into neurologic syndromes of the central, peripheral, and autonomic nervous system and the psychiatric syndromes observed in patients with Systemic lupus erythematosus (SLE) in which other causes have been excluded
We report a case of Miller-Fisher variant of Guillain-Barresyndrome presenting as the initial presentation of SLE and while the nonneurological manifestations, renal function, and SLE serology resolved with immunosuppressive therapy and plasmapheresis, the MFS symptoms did not improve and the patient remained dependent on mechanical ventilation and endogastric tube feeding
In view of SLE with positive antiribosomal P protein and lack of improvement in the neurological/GBS symptoms and with patient still being dependent on mechanical ventilation and PEG tube feeding, a 2nd course of intravenous immunoglobulin (IVIG) was started 2 g/kg divided over 5 days

Summary

Introduction

Neuropsychiatric systemic lupus erythematosus (NPSLE) as described by the American College of Rheumatology (ACR) research committee includes 19 neuropsychiatric syndromes divided into neurologic syndromes of the central, peripheral, and autonomic nervous system and the psychiatric syndromes observed in patients with SLE in which other causes have been excluded. These symptoms may precede the onset of SLE or can occur at any time during the course of SLE [1]. Guillain-Barresyndrome which is classified under the peripheral involvement has been rarely associated with SLE. Complete recovery was only achieved after the 2nd cycle of IVIG which was given approximately 2 months after the 1st cycle while only on prednisone maintenance daily dose

Case Report

Findings

Discussion