Intravenous human immunoglobulin for the treatment of severe longitudinal extensive transverse myelitis associated with systemic lupus erythematous

A 39-year-old female with systemic lupus erythematous (SLE) presented with fever, headaches and rash and was treated with empiric antibiotics for a presumable meningitis. However, cerebrospinal fluid turned out to be sterile and fever persisted with onset of hyporeflexive paraparesis with sensory loss below D10. Magnetic resonance imaging (MRI) demonstrated intradural-extramedullary hematoma (related to traumatic lumbar puncture), which was successfully drained. Subsequent surgical scar infection occurred and was treated accordingly. Due to persistent deficits and urinary retention development, she repeated MRI, which showed holocord abnormal T2 hyperintensity. After excluding other causes, SLE-associated longitudinal extensive transverse myelitis was diagnosed and treated with high-dose steroids and intravenous human immunoglobulin (IVIG), with reduction in intensity and extension of abnormal T2 signal within only one week. Monthly IVIG was kept for 6 months with progressive ability to walk and MRI findings resolution in 3 months. Four months after IVIG suspension no recurrence occurred.