Spinal Cord Neurosarcoidosis: A Clinical-Radiological Correlation of 39 Cases

Abstract

Objective Present radiological and clinical data of spinal cord neurosarcoidosis and response to treatment. Background The diagnosis of neurosarcoidosis is challenging. Stern et al. have used histopathological data, clinical scenarios, and response to treatment to propose diagnosis criteria for definite, probable, or possible neurosarcoidosis. There is no definitive confirmatory test except sample biopsy, which is not a preferred test for the central nervous system due to potential complications. MRI studies can help detect nervous system involvement; however, it is neither sensitive nor specific. Design/Methods Retrospective analysis with descriptive statistics. Results Our cohort consisted of 39 patients with spinal neurosarcoidosis. On MRI, 62% of the patients had a longitudinally extensive intramedullary lesion, 21% had one or multiple patchy intramedullary lesions, 31% had leptomeningeal involvement, and 18% had nerve roots enhancement. The cervical spine was most commonly affected (85%), followed by the thoracic (38%) and lumbar (15%). Thirty-seven patients were treated with oral or IV corticosteroids at first presentation, followed by maintenance with oral steroids and maintenance immunosuppressive agents. The three most used agents were Methotrexate (49%), Azathioprine (31%), and Mycophenolate mofetil (18%). Thirty-four patients had MRIs during follow-up, and twenty-nine patients had documented improvement during follow-up, with a median improvement time on MRI of 10.8 months (95% CI = 6.1 to 17 months). Thirty-one patients had enhancement on MRI at presentation, and 18 (58%) had complete enhancement resolution during follow-up, with a median time for resolution of enhancement of 51.8 months (95% CI = 24.9 to 83.4 months). Conclusions The diagnosis of spinal neurosarcoidosis can be challenging; however, we found that resolution of MRI enhancement can require a few years of immunosuppression, which is longer compared to other spinal neuroimmunological pathologies. The current knowledge about the treatment and prognosis of neurosarcoidosis is limited, and there is no FDA medication approved nor clinical trials data regarding the treatment of neurosarcoidosis.