A Rare Presentation of Acute Rhomboencephalitis and Cervical Myelitis Secondary to Systemic Lupus Erythematosus

Abstract

SESSION TITLE: Nervous System Disorders in the ICU 2 SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 31, 2017 at 01:30 PM - 02:30 PM INTRODUCTION: Neuroligic manifestations of Lupus requires a prompt diagnosis in initiation of therapy. We present a rare case of Lupus associated with rhomboencephalitis and cervical myelitis. In literature review there has not been a documented case of similar presentation as well as recommendations regarding appropriate treatment protocol. CASE PRESENTATION: This is a case of a 45 year old female with a history of Rheumatoid Arthritis managed on chronic steroids. She was diagnosed four years prior to admission and had abruptly stopped taking her prednisone 6-8 weeks prior to her admission. She was admitted for progressive dizziness, weakness, decreased appetite along with a weight loss of 20 lbs. She reported night sweats, cough with sputum, and episodes of diarrhea. Her initial laboratory workup revealed pancytopenia, mild transaminitis, and UTI. She was started on antibiotics to cover for the UTI and Clostridium difficile colitis. CT imaging only revealed splenomegaly. Bone marrow biopsy was performed due to concern of underlying malignancy which was negative. Over the next few days, the patient developed an unsteady gait and had two falls, along with extremity weakness. MRI brain was emergently done which shows increased signal Flair and T2 sequences involving the posterior right and left pons, midbrain, cerebellar peduncles, and the cervicomedullary cord. She later developed respiratory failure and was intubated for airway protection.The patient was then started on cyclophosphamide and pulse dose methylprednisolone. Amphotericin B was empirically started due to concern of neuro-histoplasmosis. All cultures were negative, autoimmune work up showed hypocomplementemia, positive antinuclear antibody 1:1280, and positive dsDNA. GM-1Ab was also negative. Repeat urinalysis was positive for 8.5 grams proteinuria with + RBC and blood. Due to lack of clinical response, daily plasmapheresis was implemented for a course of 5 days after which the patient clinically improved and was successfully weaned from mechanical ventilation. Repeat MRI imaging showed dramatic reduction of signal abnormality although some involvement was present. DISCUSSION: Although rhomboencephalitis is common in infectious and autoimmune conditions, involvement of brainstem and cervical spinal cord is quite rare, especially with a confirmed diagnosis of Lupus. CONCLUSIONS: Prompt diagnosis and initiation of immunosupressive therapy and plasmapheresis is effective in treating even rare neurologic manifestations of lupus. Reference #1: None DISCLOSURE: The following authors have nothing to disclose: Kunal Patel, Lavanya Srinivasan No Product/Research Disclosure Information