Alvin Chi-Chung Ho1, Sophelia Hoi-Shan Chan2, Eric Chan3, Sheila Suet-Na Wong4, Sharon Tsui-Hang Fung5, Sharon Wan-Wah Cherk5, Eva Lai-Wah Fung6, Kam-Hung Ma7, Kwing-Wan Tsui7, Eric Kin-Cheong Yau8, Virginia Chun-Nei Wong1. 1. Department of Paediatrics and Adolescent Medicine, Queen Mary Hospital, The University of Hong Kong, Hong Kong. 2. Department of Paediatrics and Adolescent Medicine, Queen Mary Hospital, The University of Hong Kong, Hong Kong. Electronic address: sophehs@hku.hk. 3. Division of Clinical Immunology, Department of Pathology and Clinical Biochemistry, Queen Mary Hospital, Hong Kong. 4. Department of Paediatrics and Adolescent Medicine, United Christian Hospital, Hong Kong. 5. Department of Paediatrics, Kwong Wah Hospital, Hong Kong. 6. Department of Paediatrics, Prince of Wales Hospital, Hong Kong. 7. Department of Paediatrics and Adolescent Medicine, Alice Ho Miu Ling Nethersole Hospital, Hong Kong. 8. Department of Paediatrics and Adolescent Medicine, Princess Margaret Hospital, Hong Kong.
Abstract
AIM: The study aims to analyze the incidence, clinical features, investigation findings and treatment outcomes of anti-N-methyl-d-aspartate receptor encephalitis in children from Hong Kong. METHOD: A retrospective study was carried out on paediatric patients diagnosed with anti-NMDAR encephalitis in Hong Kong from January 2009 to December 2015. RESULTS: Fifteen patients (67% female, 93% Chinese) were identified over seven years and the estimated incidence in Hong Kong was 2.2/million children per year (95% CI 1.2-3.6). The median age of presentation was 12 years (range 1-17 years). The most common symptom groups observed were abnormal psychiatric behavior or cognitive dysfunction (14/15, 93%) and seizures (14/15, 93%), followed by speech dysfunction (13/15, 87%), movement disorders (12/15, 80%), decreased level of consciousness (10/15, 67%) and autonomic dysfunction or central hypoventilation (5/15, 33%). The median number of symptom groups developed in each patient was 5 (range 3-6). All patients were treated with intravenous immunoglobulin and/or steroids. Three patients (20%) with more severe presentation required additional plasmapheresis and rituximab. Outcome was assessable in 14 patients. Among those eleven patients who had only received intravenous immunoglobulin and/or steroids, nine patients (82%) achieved full recovery. One patient (9%) had residual behavioral problem, while another one (9%) who developed anti-NMDAR encephalitis after herpes simplex virus encephalitis was complicated with dyskinetic cerebral palsy and epilepsy. Among those three patients who required plasmapheresis and rituximab, one (33%) had full recovery and two (66%) had substantial recovery. The median duration of follow up was 20.5 months (range 3-84 months). CONCLUSION: Anti-NMDAR encephalitis is an acquired, severe, but potentially treatable disorder. Ethnicity may play a role in the incidence of anti-NMDAR encephalitis and we have provided a local incidence with the majority of patients being Chinese. The diagnosis of anti-NMDAR encephalitis should be considered in children presenting with a constellation of symptoms including psychiatric and neurological manifestations. Patients may respond to first line immunotherapy. For those who do not, second line therapy is indicated in order to achieve a better outcome.
AIM: The study aims to analyze the incidence, clinical features, investigation findings and treatment outcomes of anti-N-methyl-d-aspartate receptor encephalitis in children from Hong Kong. METHOD: A retrospective study was carried out on paediatric patients diagnosed with anti-NMDAR encephalitis in Hong Kong from January 2009 to December 2015. RESULTS: Fifteen patients (67% female, 93% Chinese) were identified over seven years and the estimated incidence in Hong Kong was 2.2/million children per year (95% CI 1.2-3.6). The median age of presentation was 12 years (range 1-17 years). The most common symptom groups observed were abnormal psychiatric behavior or cognitive dysfunction (14/15, 93%) and seizures (14/15, 93%), followed by speech dysfunction (13/15, 87%), movement disorders (12/15, 80%), decreased level of consciousness (10/15, 67%) and autonomic dysfunction or central hypoventilation (5/15, 33%). The median number of symptom groups developed in each patient was 5 (range 3-6). All patients were treated with intravenous immunoglobulin and/or steroids. Three patients (20%) with more severe presentation required additional plasmapheresis and rituximab. Outcome was assessable in 14 patients. Among those eleven patients who had only received intravenous immunoglobulin and/or steroids, nine patients (82%) achieved full recovery. One patient (9%) had residual behavioral problem, while another one (9%) who developed anti-NMDAR encephalitis after herpes simplex virus encephalitis was complicated with dyskinetic cerebral palsy and epilepsy. Among those three patients who required plasmapheresis and rituximab, one (33%) had full recovery and two (66%) had substantial recovery. The median duration of follow up was 20.5 months (range 3-84 months). CONCLUSION: Anti-NMDAR encephalitis is an acquired, severe, but potentially treatable disorder. Ethnicity may play a role in the incidence of anti-NMDAR encephalitis and we have provided a local incidence with the majority of patients being Chinese. The diagnosis of anti-NMDAR encephalitis should be considered in children presenting with a constellation of symptoms including psychiatric and neurological manifestations. Patients may respond to first line immunotherapy. For those who do not, second line therapy is indicated in order to achieve a better outcome.
Authors: Thaís Armangue; Marianna Spatola; Alexandru Vlagea; Simone Mattozzi; Marc Cárceles-Cordon; Eloy Martinez-Heras; Sara Llufriu; Jordi Muchart; María Elena Erro; Laura Abraira; German Moris; Luis Monros-Giménez; Íñigo Corral-Corral; Carmen Montejo; Manuel Toledo; Luis Bataller; Gabriela Secondi; Helena Ariño; Eugenia Martínez-Hernández; Manel Juan; Maria Angeles Marcos; Laia Alsina; Albert Saiz; Myrna R Rosenfeld; Francesc Graus; Josep Dalmau Journal: Lancet Neurol Date: 2018-07-23 Impact factor: 44.182
Authors: Anna E M Bastiaansen; Marienke A A M de Bruijn; Sabine L Schuller; Eugenia Martinez-Hernandez; Juliëtte Brenner; Manuela Paunovic; Yvette S Crijnen; Maxim J H L Mulder; Marco W J Schreurs; Esther de Graaff; Peter A E Smitt; Rinze F Neuteboom; Juna M de Vries; Maarten J Titulaer Journal: Neurol Neuroimmunol Neuroinflamm Date: 2021-12-22