Abstract Details

Title Acute Motor-Sensory Axonal Neuropathy Associated with Systemic Lupus Erythematosus: A Challenge in Treatment

Topic Autoimmune Neurology

Presentation(s) P1 - Poster Session 1 (5:30 PM-6:30 PM)

Poster/Presentation
Number 15-047

Objective To present an unusual case with complex management of acute motor-sensory axonal neuropathy (AMSAN) associated with systemic lupus erythematosus (SLE).

Background AMSAN is a variant of Guillian-Barré syndrome. The standard treatment is intravenous immunoglobulin (IVIg) or plasmapheresis. SLE is a multisystem autoimmune disease which is generally treated by immunosuppressant. The association of AMSAN and SLE is rarely reported. Herein, we described the clinical presentation and complex management in the case of AMSAN with SLE.

Design/Methods NA

Results A previously healthy 72-year-old Caucasian man presented with rapidly progressive paraparesis and dysesthesia and bilateral knees and finger pain with morning stiffness. He had no history of recent fever, diarrhea or vaccination. Neurological examination showed complete quadriplegia and areflexia. Hyperesthesia was noted in all extremities. Cranial nerves were intact. Pain with passive movement and tenderness were noted in knees and joints of the fingers. Other systemic examination was normal. Blood work showed elevated erythrocyte sedimentation rate, antinuclear antibody, and anti-dsDNA titer. Antineutrophil cytoplasmic antibody and paraneoplastic antibodies were negative. Cerebrospinal fluid showed albuminocytologic dissociation. Nerve conduction study was suggestive for acute diffuse axonal sensorimotor polyneuropathy (motor predominant). Initially, the patient was treated by IVIg without significant response. Because the patient met the systemic lupus international collaborating clinic diagnostic criteria for SLE, intravenous methylprednisolone and cyclophosphamide were given. Joint pain was resolved. The motor strength was slowly improving. Ganglioside antibody panel reported positive GD1b antibody with negative GM1, GD1a and GQ1b. Ten days later, the weakness started worsening. After plasmapheresis, the patient was stabilized, then slowly improving. The outcome at 3 and 6 months will be presented at the conference.

Conclusions

The association of AMSAN and SLE is poorly understood. Combination of IVIg and/or plasmapheresis with immunosuppression may benefit in AMSAN associated with SLE.

Authors/Disclosures
Smathorn Thakolwiboon, MD (Mayo Clinic Health System) PRESENTER	Dr. Thakolwiboon has nothing to disclose.
Amputch Karukote, MD	Dr. Karukote has nothing to disclose.

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