Abstract Details

A 27 year-old woman with a 10-year history of SLE treated with mycophenolate mofetil developed rapidly progressive quadriparesis with arreflexia, a thoracic sensory level, and urinary retention over 36 hours. Her symptoms started six days earlier with fever, headaches, pruritic skin eruptions, nausea, vomiting and back spasms. Spinal MRI showed extensive T2-weighted signal abnormality involving the entire cord from the conus to the lower medulla. Cerebrospinal fluid analysis revealed lymphocytic predominant pleocytosis (152 WBC/mcl, 85% lymphocytes). Laboratory testing revealed low serum complement levels and positive titers for ANA, SSA, and dsDNA; AQP4-IgG was positive at a titer of > 1:100,000. Myelin oligodendrocyte glycoprotein (MOG) and antiphospholipid antibodies were negative. Skin biopsy demonstrated leukocytoclastic vasculitis. MRI brain demonstrated scattered, nonenhancing T2 hyperintensities in the deep subcortical white matter, brainstem and cerebellum and a punctate periventricular infarct. Brain MR angiography (MRA) showed segmental narrowing and dilation involving medium and small sized vessels. Treatment was initiated with intravenous methylprednisolone and cyclophosphamide followed by rituximab. Repeat brain MRA on hospital day 5 showed significant improvement in the appearance of the intracranial vasculature; her rash and headache resolved but minimal motor recovery was noted.