Acute motor-sensory axonal neuropathy associated with active systemic lupus erythematosus and anticardiolipin antibodies

Acute motor-sensory axonal neuropathy (AMSAN) is an axonal variant of Guill ian-Barre syndrome (GBS) that presents with acute ascending quadriparesis. This has generally been described in association with Campylobacter jejuni infections or with antiganglioside antibodies. Known cases have shown a slo w recovery and a poor prognosis. We report a case with clinical and electro physiological evidence of AMSAN in association with active systemic lupus e rythematosus (SLE) and anticardiolipin antibodies but not the other associa tions, with a rapid response to combination immunosuppressant and intraveno us immunoglobulin (Mg) therapy. The association between AMSAN and SLE has n ot been previously described. This case illustrates that early recognition and the utilization of electrophysiologic techniques may be beneficial in t he diagnosis and management of GBS associated with SLE. Fulminant or rapidl y progressive cases should be managed in specialized intensive care units. Combination therapy of immunosuppressants, and IVIg may be beneficial in no nvasculitic axonal radiculoneuropathies associated with SLE, resulting in g ood outcomes.