STABBED IN THE BACK! A CASE OF NEUROSARCOIDOSIS WITH SPINAL CORD INVOLVEMENT

Abstract

SESSION TITLE: Tuesday Fellows Case Report Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: 10/22/2019 01:00 PM - 02:00 PM INTRODUCTION: Sarcoidosis is a systemic disease characterized by the presence of non-caseating granulomas. While the lungs are the primary site of involvement, multiple other organ systems can be affected1. Sarcoidosis involving the central nervous system (CNS) is a rare manifestation seen in roughly 5% of sarcoid cases2. Clinical manifestations of neurosarcoidosis depend on the CNS site affected and can be quite variable leading to diagnostic challenges and delays. Neurosarcoidosis with spinal cord involvement (NSSI) is a rarely encountered variant of CNS involvement and can pose serious diagnostic challenges due to the high morbidity and potential mortality associated with direct tissue biopsy from spinal cord structures. However, a majority of cases also have pulmonary involvement3. In such cases; a thorough evaluation for concurrent pulmonary involvement may provide diagnostic confirmation despite absence of overt pulmonary symptoms. CASE PRESENTATION: A 66-year-old nonsmoker with a history of rheumatoid arthritis and hypertension was found to have bilateral FDG avid mediastinal lymphadenopathy in the setting of a recent working diagnosis of neuromyelitis optica (Figure 1). Aside from bilateral lower extremity ascending paresthesias, the patient was otherwise asymptomatic with normal pulmonary function tests within the past five years. On physical exam, there was evidence of bilateral lower extremity spasticity with decreased proprioception and temperature sensation. Cardio-pulmonary exam was otherwise normal. Neuromyelitis optica (NMO) autoantibodies were negative and neuroimaging showed extensive longitudinal central T2 cord signal with subpial enhancement,‘Trident sign’ (Figure 2). Bronchoscopy with endobronchial ultrasound guided transbronchial biopsy (EBUS TBNA) found non-caseating granulomatous inflammation. Diagnosis of NSSI was made and patient was started on high dose prednisone with transition the infliximab. DISCUSSION: NSSI is a rarely encountered variant of sarcoidosis. Patients with this form of disease commonly present with clinical findings of abnormal proprioception, spasticity, paresthesias and urinary dysfunction2. Additionally the ‘trident sign’ comprised of linear dorsal subpial enhancement within the spinal cord is a well described pattern on MRI associated with NSSI3. Recognizing the common symptoms and imaging findings associated with NSSI is important as up to eighty percent of patients lack pulmonary symptoms or impairment4. Once diagnosis is suspected, a thorough workup to identify pulmonary and other non-CNS sites of sarcoid involvement should be undertaken. It is important to remember that up to seventy percent of patients with neurosarcoidosis have some pulmonary manifestations of sarcoidosis. CONCLUSIONS: This case underscores the importance of pulmonary evaluation when NSSI is suspected, as EBUS TBNA can aid in clinching the diagnosis as demonstrated in this case. Reference #1: Baughman RP, Culver DA, Judson MA. A concise review of pulmonary sarcoidosis. Am J Respir Crit Care Med. 2011;183: 573-581 Reference #2: Zalewski NL, Krecke KN, Weinshenker BG, Aksamit AJ, Conway BL, McKeon A, Flanagan EP. Central canal enhancement and the trident sign in spinal cord sarcoidosis. Neurology. 2016;87(7): 743-744 Reference #3: Kingah P, Alam M, Chugh K, Kamholz J, Samavati L. Role of pulmonary evaluation in diagnosis of neurosarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis. 2016;33: 209-215 DISCLOSURES: No relevant relationships by Vivek Iyer, source=Web Response No relevant relationships by Mark Norton, source=Web Response