YoungKyu Shim1, Soo Yeon Kim1, Hunmin Kim2, Hee Hwang2, Jong-Hee Chae1, Jieun Choi3, Ki Joong Kim1, Mi-Sun Yum4, Tae Sung Ko4, Young Ok Kim5, Jung Hye Byeon6, Jiwon Lee7, Jeehun Lee7, Jon Soo Kim8, Byung Chan Lim9. 1. Department of Pediatrics, Pediatric Clinical Neuroscience Center, Seoul National University Children's Hospital, Seoul National University College of Medicine, Seoul, Republic of Korea. 2. Department of Pediatrics, Bundang Seoul National University Hospital, Bundang, Republic of Korea. 3. Department of Pediatrics, Seoul National University Boramae Medical Center, Seoul, Republic of Korea. 4. Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea. 5. Department of Pediatrics, Chonnam National University Medical School, Gwangju, Republic of Korea. 6. Department of Pediatrics, Korea University College of Medicine, Seoul, Republic of Korea. 7. Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University, School of Medicine, Seoul, Republic of Korea. 8. Department of Pediatrics, Chungbuk National University Hospital, Cheongju, Republic of Korea. 9. Department of Pediatrics, Pediatric Clinical Neuroscience Center, Seoul National University Children's Hospital, Seoul National University College of Medicine, Seoul, Republic of Korea. Electronic address: prabbit7@snu.ac.kr.
Abstract
OBJECTIVE: To investigate the clinical features and long-term outcomes of pediatric Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. METHODS: Thirty-two anti-NMDAR encephalitis patients with positive anti-NMDAR antibody test results were recruited. Clinical outcomes were evaluated using the Clinical Assessment Scale in Autoimmune Encephalitis (CASE) and the modified Rankin Scale (mRS). RESULTS: The median age of onset was 9.0 years (range, 0.7-17.2 years). Twenty-four patients (75.0%) were female. All patients received first-line immunotherapy including intravenous immunoglobulin and/or steroid therapy. The second-line immunotherapy was administered to 22 patients (68.8%). Clinical outcomes were evaluated in 27 patients who were followed for longer than 6 months after onset, among whom the median follow-up duration was 31.2 months (range, 6.3-82.9 months). The proportion of patients with ≤2 points on the mRS at their 12-month follow-up was 79.2% (19/24). The CASE scores of these 19 patients ranged from 0 to 5, with language and memory deficits accounting for most of these disabilities. When the outcome was assessed according to onset age (<12 years or 12-18 years), the younger group tended to show a slower recovery over their clinical course. CONCLUSIONS: Despite overall favorable clinical outcomes, mild cognitive problems, including language and memory, may persist in pediatric anti-NMDAR encephalitis patients. A specific outcome measure, such as CASE, should be adopted to delineate clinical outcomes and aid the development of individualized treatment plans.
OBJECTIVE: To investigate the clinical features and long-term outcomes of pediatric Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. METHODS: Thirty-two anti-NMDAR encephalitispatients with positive anti-NMDAR antibody test results were recruited. Clinical outcomes were evaluated using the Clinical Assessment Scale in Autoimmune Encephalitis (CASE) and the modified Rankin Scale (mRS). RESULTS: The median age of onset was 9.0 years (range, 0.7-17.2 years). Twenty-four patients (75.0%) were female. All patients received first-line immunotherapy including intravenous immunoglobulin and/or steroid therapy. The second-line immunotherapy was administered to 22 patients (68.8%). Clinical outcomes were evaluated in 27 patients who were followed for longer than 6 months after onset, among whom the median follow-up duration was 31.2 months (range, 6.3-82.9 months). The proportion of patients with ≤2 points on the mRS at their 12-month follow-up was 79.2% (19/24). The CASE scores of these 19 patients ranged from 0 to 5, with language and memory deficits accounting for most of these disabilities. When the outcome was assessed according to onset age (<12 years or 12-18 years), the younger group tended to show a slower recovery over their clinical course. CONCLUSIONS: Despite overall favorable clinical outcomes, mild cognitive problems, including language and memory, may persist in pediatric anti-NMDAR encephalitispatients. A specific outcome measure, such as CASE, should be adopted to delineate clinical outcomes and aid the development of individualized treatment plans.
Authors: Jonathan D Santoro; Rebecca Partridge; Runi Tanna; Dania Pagarkar; Mellad Khoshnood; Mustafa Rehmani; Ryan M Kammeyer; Grace Y Gombolay; Kristen Fisher; Allison Conravey; Jane El-Dahr; Alison L Christy; Lina Patel; Melanie A Manning; Heather Van Mater; Michael S Rafii; Eileen A Quinn Journal: J Neurodev Disord Date: 2022-06-03 Impact factor: 4.074