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Anti-N-methyl-d-aspartate receptor encephalitis in children: Incidence and experience in Hong Kong.
Brain Dev. 2018 Jun; 40(6):473-479.BD

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.

Authors+Show Affiliations

Department of Paediatrics and Adolescent Medicine, Queen Mary Hospital, The University of Hong Kong, Hong Kong.Department of Paediatrics and Adolescent Medicine, Queen Mary Hospital, The University of Hong Kong, Hong Kong. Electronic address: sophehs@hku.hk.Division of Clinical Immunology, Department of Pathology and Clinical Biochemistry, Queen Mary Hospital, Hong Kong.Department of Paediatrics and Adolescent Medicine, United Christian Hospital, Hong Kong.Department of Paediatrics, Kwong Wah Hospital, Hong Kong.Department of Paediatrics, Kwong Wah Hospital, Hong Kong.Department of Paediatrics, Prince of Wales Hospital, Hong Kong.Department of Paediatrics and Adolescent Medicine, Alice Ho Miu Ling Nethersole Hospital, Hong Kong.Department of Paediatrics and Adolescent Medicine, Alice Ho Miu Ling Nethersole Hospital, Hong Kong.Department of Paediatrics and Adolescent Medicine, Princess Margaret Hospital, Hong Kong.Department of Paediatrics and Adolescent Medicine, Queen Mary Hospital, The University of Hong Kong, Hong Kong.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

29599011

Citation

Ho, Alvin Chi-Chung, et al. "Anti-N-methyl-d-aspartate Receptor Encephalitis in Children: Incidence and Experience in Hong Kong." Brain & Development, vol. 40, no. 6, 2018, pp. 473-479.
Ho AC, Chan SH, Chan E, et al. Anti-N-methyl-d-aspartate receptor encephalitis in children: Incidence and experience in Hong Kong. Brain Dev. 2018;40(6):473-479.
Ho, A. C., Chan, S. H., Chan, E., Wong, S. S., Fung, S. T., Cherk, S. W., Fung, E. L., Ma, K. H., Tsui, K. W., Yau, E. K., & Wong, V. C. (2018). Anti-N-methyl-d-aspartate receptor encephalitis in children: Incidence and experience in Hong Kong. Brain & Development, 40(6), 473-479. https://doi.org/10.1016/j.braindev.2018.02.005
Ho AC, et al. Anti-N-methyl-d-aspartate Receptor Encephalitis in Children: Incidence and Experience in Hong Kong. Brain Dev. 2018;40(6):473-479. PubMed PMID: 29599011.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Anti-N-methyl-d-aspartate receptor encephalitis in children: Incidence and experience in Hong Kong. AU - Ho,Alvin Chi-Chung, AU - Chan,Sophelia Hoi-Shan, AU - Chan,Eric, AU - Wong,Sheila Suet-Na, AU - Fung,Sharon Tsui-Hang, AU - Cherk,Sharon Wan-Wah, AU - Fung,Eva Lai-Wah, AU - Ma,Kam-Hung, AU - Tsui,Kwing-Wan, AU - Yau,Eric Kin-Cheong, AU - Wong,Virginia Chun-Nei, Y1 - 2018/03/26/ PY - 2017/09/11/received PY - 2018/01/07/revised PY - 2018/02/07/accepted PY - 2018/3/31/pubmed PY - 2018/9/14/medline PY - 2018/3/31/entrez KW - Anti-NMDAR encephalitis KW - Autoimmune encephalitis KW - Encephalitis KW - NMDAR antibody KW - Neuroimmunology SP - 473 EP - 479 JF - Brain & development JO - Brain Dev VL - 40 IS - 6 N2 - 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. SN - 1872-7131 UR - https://www.unboundmedicine.com/medline/citation/29599011/Anti_N_methyl_d_aspartate_receptor_encephalitis_in_children:_Incidence_and_experience_in_Hong_Kong_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0387-7604(18)30037-8 DB - PRIME DP - Unbound Medicine ER -