E R Hammond1, A I Kaplin, D A Kerr. 1. Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD 21287-5371, USA.
Abstract
STUDY DESIGN: Case report. CLINICAL SETTING: Johns Hopkins University School of Medicine, Baltimore, MD, USA. CASE REPORT: Sarcoidosis is a multi-system granulomatous disease of unknown etiology with worldwide distribution. The involvement of the nervous system is common-neurosarcoidosis. Immune responses play an important role in the inflammatory process and granuloma formation. We report a case of neurosarcoidosis that was refractory to two courses of intravenous steroids. Upon initiation of oral thalidomide, the patient showed dramatic improvement clinically and on magnetic resonance imaging. CONCLUSION: Thalidomide is an immunomodulatory agent that acts to inhibit production of tumor-necrosis factor-alpha (TNF-alpha), an important mediator in CNS inflammation, by enhancing TNF-alpha mRNA degradation. Corticosteroids have been the mainstay of treatment of neurosarcoidosis with success at halting progression of the immune process in 50% cases. Thalidomide offers unique opportunities at managing CNS inflammation due to neurosarcoidosis. DISCLOSURES: None.
STUDY DESIGN: Case report. CLINICAL SETTING: Johns Hopkins University School of Medicine, Baltimore, MD, USA. CASE REPORT: Sarcoidosis is a multi-system granulomatous disease of unknown etiology with worldwide distribution. The involvement of the nervous system is common-neurosarcoidosis. Immune responses play an important role in the inflammatory process and granuloma formation. We report a case of neurosarcoidosis that was refractory to two courses of intravenous steroids. Upon initiation of oral thalidomide, the patient showed dramatic improvement clinically and on magnetic resonance imaging. CONCLUSION: Thalidomide is an immunomodulatory agent that acts to inhibit production of tumor-necrosis factor-alpha (TNF-alpha), an important mediator in CNS inflammation, by enhancing TNF-alpha mRNA degradation. Corticosteroids have been the mainstay of treatment of neurosarcoidosis with success at halting progression of the immune process in 50% cases. Thalidomide offers unique opportunities at managing CNS inflammation due to neurosarcoidosis. DISCLOSURES: None.
Authors: Jeffrey M Gelfand; Michael J Bradshaw; Barney J Stern; David B Clifford; Yunxia Wang; Tracey A Cho; Laura L Koth; Stephen L Hauser; Jason Dierkhising; NgocHanh Vu; Subramaniam Sriram; Harold Moses; Francesca Bagnato; Jeffrey A Kaufmann; Deidre J Ammah; Tsion H Yohannes; Mark J Hamblin; Nagagopal Venna; Ari J Green; Siddharama Pawate Journal: Neurology Date: 2017-10-13 Impact factor: 9.910