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Clinical manifestations,treatment outcomes,and prognostic factors of pediatric anti-NMDAR encephalitis in tertiary care hospitals: A multicenter retrospective/prospective cohort study
Affiliation:1. Department of Pediatrics, King Chulalongkorn Memorial Hospital, The Thai Red Cross Society, Thailand;2. Department of Pediatrics, Chonburi Hospital, Chonburi, Thailand;3. Department of Pediatrics, Prapokklao Hospital, Chanthaburi, Thailand;4. Division of Neurology, Department of Pediatrics, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand;1. Department of Neurology at Hospital Clínic-Institut d''Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain;2. Department of Neurology, Erasmus Medical Center, Rotterdam, Netherlands;3. Biostatistics and Data Management Platform, IDIBAPS, Hospital Clínic, Barcelona, Spain;4. Department of Neurology, University of California—Los Angeles, Los Angeles, CA, USA;5. Department of Neuroscience, University of Pennsylvania, Philadelphia, PA, USA;6. Department of Neurology, University of Pennsylvania, Philadelphia, PA, USA;7. Department of Neurology, Children''s Hospital of Philadelphia (CHOP), Philadelphia, PA, USA;8. Institució Catalana de Recerca i Estudis Avançats (ICREA), Barcelona, Spain;1. Paediatric Neurology Unit, Department of Woman''s and Child''s Health, University Hospital of Padua, Padua, Italy;2. Child Neuropsychiatry Unit, Polytechnic University of the Marche, Ancona, Italy;3. Paediatric Acute and Emergency Operative Unit and Department, Policlinico Vittorio Emanuele University Hospital, University of Catania, Italy;4. Department of Child Neuropsychiatry, Epilepsy Center, C. Poma Hospital, Mantua, Italy;5. Unit of Child Neuropsychiatry, Head-Neck and Neuroscience Department, Giannina Gaslini Institute, Genoa, Italy;6. Child and Adolescent Neuropsychiatry Unit, Department of Paediatrics, ARNAS Civico – Di Cristina, Palermo, Italy;7. Paediatric Neurophysiology Unit, Department of Paediatrics, University of Padua, Italy;8. Child Neuropsychiatry, Department of Life and Reproduction Sciences, University of Verona, Italy;9. Unit of Clinical Neurophysiology, Department of Neurological Sciences, Foundation IRCCS Ca'' Granda Ospedale Maggiore Policlinico, Milan, Italy;10. Institute for Maternal and Child Health – IRCCS “Burlo Garofolo”, Trieste, Italy;11. Department of Neurology, S. Raffaele Hospital, Milan, Italy;12. Child Neurology and Psychiatry Dept, Pediatric Hospital, Cagliari, Italy;13. Neurology Unit, Bambino Gesù Children''s Hospital, Rome, Italy;1. Faculty of Medicine, The University of Jordan, Amman, Jordan;2. Department of Pediatrics, Division of Child Neurology, Faculty of Medicine –The University of Jordan, Amman, Jordan;3. Distinguished Professor of Pediatrics and Infectious Disease, Department of Pediatrics, Faculty of Medicine, The University of Jordan, Amman, Jordan;1. Department of Pediatric Neuroscience, Foundation IRCCS Neurological Institute “C. Besta”, Milan, Italy;2. Neuromuscular Diseases and Neuroimmunology Unit, Foundation IRCCS Neurological Institute “C. Besta”, Milan, Italy;3. Clinical Neurophysiology and Epilepsy Center, Foundation IRCCS Neurological Institute C. Besta, Italy;1. Department of Neurology and Neurotherapeutics, University of Texas Southwestern Medical Center, Dallas, Texas;2. Department of Neurology and Neurotherapeutics, Children''s Medical Center Dallas, Dallas, Texas
Abstract:ObjectiveAnti-NMDAR encephalitis is an acute autoimmune neurological disorder that is increasingly recognized in pediatric populations. Several studies of the disorder have been conducted worldwide but there are few publications in Thailand. Here, we describe the clinical manifestations, treatment outcomes, and prognostic factors in children with anti-NMDAR encephalitis.MethodsBetween January 2007 and September 2017, we conducted a retrospective/prospective cohort study of children diagnosed with anti-NMDAR encephalitis from three tertiary care hospitals in Thailand: King Chulalongkorn Memorial Hospital, Chonburi Hospital, and Prapokklao Hospital. We assessed the Modified Rankin Score (mRS) score for each participant to measure severity of disease and treatment outcome at baseline, 12, and 24 months.ResultsWe recruited 14 participants (1–13 years with median age 8.4 years). Participants were followed up for a median of 20.5 months. Clinical manifestations included behavioral dysfunction (100%), movement disorder (93%), speech disorder (79%), sleep disorder (79%), and seizures (79%). All patients received first-line immunotherapy (corticosteroids: 100%, intravenous immunoglobulin: 79%, plasma exchange: 21%). Second-line immunotherapy (cyclophosphamide) was administered to 57% of patients. During the first 12 months, 8 patients (62%) achieved a good outcome (mRS ≤ 2). At 24 months, 9 patients (81%) had achieved a good outcome. Altered consciousness and central hypoventilation were predictors of poor outcome. (p < 0.05).ConclusionsWe observed similar clinical manifestation of anti-NMDAR encephalitis in Thai children to those reported in other countries. Furthermore, the percentage of patients with good outcomes in our study was comparable with previous studies. Further studies are required to investigate other populations in other regions of Thailand.
Keywords:Pediatric encephalitis  NMDAR  Autoimmune encephalitis
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