Allen D DeSena1,2, Daniel K Noland3,4, Karen Matevosyan3, Kathryn King5, Lauren Phillips1, Sara S Qureshi1, Benjamin M Greenberg1,2, Donna Graves1,2. 1. Department of Neurology and Neurotherapeutics, University of Texas Southwestern Medical Center, Dallas, Texas. 2. Department of Neurology, Children's Medical Center, Dallas, Texas. 3. Department of Pathology, University of Texas Southwestern Medical Center, Dallas, Texas. 4. Department of Pathology, Children's Medical Center, Dallas, Texas. 5. Department of Pediatrics, Children's Medical Center, Dallas, Texas.
Abstract
INTRODUCTION: Anti-N-methyl-d-aspartate (NMDA) receptor antibody encephalitis is an increasingly recognized form of autoimmune encephalitis. Conventional treatments include therapies such as corticosteroids, intravenous immunoglobulin (IVIg), and/or therapeutic plasma exchange (TPE). Although TPE is regularly used for treatment of anti-NMDA receptor antibody encephalitis, the American Society for Apheresis has given it a category III recommendation only. Earlier administered immunotherapies in tumor-negative patients may facilitate faster recoveries, but it remains unclear whether or not TPE is superior to steroids and/or IVIG. METHODS: We retrospectively evaluated 10 of 14 patients that received steroids and TPE with modified Rankin scores and subjectively assessed the point of largest sustained improvement in all 14 patients. RESULTS: In the patients that received both steroids and TPE at our institution during the same hospitalization (only 10 of 14 patients), 7/10 patients after TPE had improved with the modified Rankin score versus 3/10 patients after steroids. The average modified Rankin score improvement after steroids in this group was -0.1 as compared with 0.4 after TPE. Based on subjective chart review analysis during which all 14 patients were assessed, the largest sustained improvement occurred immediately following the third-fifth exchange in 9/14 patients, whereas only 2/14 patients appeared to have had significant benefit immediately following steroids. CONCLUSIONS: This is compelling preliminary data that suggests that corticosteroids may not be as effective compared to steroids followed by TPE. Given the importance of time-sensitive treatment, more formal studies may illuminate the ideal first-line treatment for anti-NMDA receptor antibody encephalitis.
INTRODUCTION: Anti-N-methyl-d-aspartate (NMDA) receptor antibody encephalitis is an increasingly recognized form of autoimmune encephalitis. Conventional treatments include therapies such as corticosteroids, intravenous immunoglobulin (IVIg), and/or therapeutic plasma exchange (TPE). Although TPE is regularly used for treatment of anti-NMDA receptor antibody encephalitis, the American Society for Apheresis has given it a category III recommendation only. Earlier administered immunotherapies in tumor-negative patients may facilitate faster recoveries, but it remains unclear whether or not TPE is superior to steroids and/or IVIG. METHODS: We retrospectively evaluated 10 of 14 patients that received steroids and TPE with modified Rankin scores and subjectively assessed the point of largest sustained improvement in all 14 patients. RESULTS: In the patients that received both steroids and TPE at our institution during the same hospitalization (only 10 of 14 patients), 7/10 patients after TPE had improved with the modified Rankin score versus 3/10 patients after steroids. The average modified Rankin score improvement after steroids in this group was -0.1 as compared with 0.4 after TPE. Based on subjective chart review analysis during which all 14 patients were assessed, the largest sustained improvement occurred immediately following the third-fifth exchange in 9/14 patients, whereas only 2/14 patients appeared to have had significant benefit immediately following steroids. CONCLUSIONS: This is compelling preliminary data that suggests that corticosteroids may not be as effective compared to steroids followed by TPE. Given the importance of time-sensitive treatment, more formal studies may illuminate the ideal first-line treatment for anti-NMDA receptor antibody encephalitis.
Authors: Kenneth E Remy; Jason W Custer; Joshua Cappell; Cortney B Foster; Nan A Garber; L Kyle Walker; Liliana Simon; Dayanand Bagdure Journal: Front Pediatr Date: 2017-11-24 Impact factor: 3.418