018. A CASE OF SYSTEMIC LUPUS ERYTHEMATOSUS PRESENTING WITH EXTENSIVE LONGITUDINAL MYELITIS AND CEREBRAL VASCULOPATHY

Abstract

Background: Neuropsychiatric SLE (NPSLE) affects 60% of patients with lupus. It usually occurs in active disease. The ACR established 19 definitions for NPSLE. Myelopathy is a rare form of NPSLE. Here we describe a case of a young patient presenting with acute extensive longitudinal myelopathy (AELM) and cerebral vasculopathy as a first manifestation of SLE. Methods: A 35-year old female patient presented to our hospital with history of lethargy, weight loss and night sweats for 3 months. She noticed lumps in her neck with difficulty passing urine and leg weakness for 8 weeks. She was 4 months post-partum. She had previous cardiomyopathy with LVEF of 35%. On presentation, she was febrile, had cervical and axillary lymphadenopathy with urinary retention. The neurological examination showed a power of 4/5 on the lower limbs bilaterally with brisk tendon reflexes, extensor plantar responses and no sensory deficits. Her motor power deteriorated over 10 days to 0/5. Results: Her laboratory workup showed Hb 79, WBC 2.5 (Neut 1.6, Lymph 0.8), Plt 413 and an inflammatory acute phase response (ESR 134mm/hr, CRP<5mg/l). Initial MRI of the brain and spinal cord was unremarkable. Autoimmune Screen showed (ANA 1/640, positive anti- Ro, anti-RNP, anti-dsDNA) negative ANCA and low C3 and C4. Antiphospholipid screen was negative. She had no proteinuria. CSF was acellular with protein 4000 mg/l, low glucose and positive oligoclonal bands. Infective causes were ruled out. PET/CT showed avid nodal disease above and below the diaphragm with involvement of the spleen and bone marrow. Bone marrow and lymph node biopsies showed reactive changes. TTE showed low ejection fraction 35% with no valvular disease or vegetations. She was treated initially with IVMP 3 days, then 60mg prednisolone and IV Cyclophosphamide 500 mg. She deteriorated with new onset cognitive dysfunction, UMN facial paresis and upper limb weakness. Repeat MRI of the brain showed new ischaemic changes in the brain and spinal cord. Brain biopsy showed mild chronic inflammation with occasional parenchymal and perivascular T-cell infiltrates with gliosis. Patient was febrile and neutropenic and plasma exchange was attempted, this was followed by Rituximab treatment. Her neurological status deteriorated further, she was mechanically ventilated. Follow up MRI showed extensive cerebral ischemic changes. She was restarted on IV cyclophosphamide. Patient was extubated, the power on the lower limbs remained the same, however, upper limb function and cognition improved. Conclusion: Longitudinal extensive transverse myelitis is a catastrophic presentation of systemic lupus erythematosus and is typically associated with poor clinical outcomes despite aggressive immunosuppressive therapy. Rarely is myelopathy the initial presentation of lupus. What makes our case more intriguing is the association of LETM with cerebral vasculopathy. Rapid initiation of immunosuppressive therapy as well as rigorous physical rehabilitation can improve the outcome.