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N-methyl-D-aspartate receptor antibody-mediated neurological disease: results of a UK-based surveillance study in children
  1. Sukhvir Wright1,2,
  2. Yael Hacohen1,
  3. Leslie Jacobson1,
  4. Shakti Agrawal2,
  5. Rajat Gupta2,
  6. Sunny Philip2,
  7. Martin Smith2,
  8. Ming Lim1,3,
  9. Evangeline Wassmer1,
  10. Angela Vincent1
  1. 1Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, University of Oxford, Oxford, UK
  2. 2Department of Paediatric Neurology, Birmingham Children's Hospital, Birmingham, UK
  3. 3Children's Neurosciences, Evelina Children's Hospital @ Guy's and St Thomas’ NHS Foundation Trust, King's Health Partners Academic Health Science Centre, London, UK
  1. Correspondence to Professor Angela Vincent, Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, Level 6, West Wing, Oxford OX3 9DU, UK; angela.vincent{at}ndcn.ox.ac.uk

Abstract

Objective N-methyl-D-aspartate receptor antibody (NMDAR-Ab) encephalitis is a well-recognised clinico-immunological syndrome that presents with neuropsychiatric symptoms cognitive decline, movement disorder and seizures. This study reports the clinical features, management and neurological outcomes of paediatric NMDAR-Ab-mediated neurological disease in the UK.

Design A prospective surveillance study. Children with NMDAR-Ab-mediated neurological diseases were voluntarily reported to the British Neurological Surveillance Unit (BPNSU) from November 2010 to December 2011. Initial and follow-up questionnaires were sent out to physicians.

Results Thirty-one children fulfilled the criteria for the study. Eight presented during the study period giving an incidence of 0.85 per million children per year (95% CI 0.64 to 1.06); 23 cases were historical. Behavioural change and neuropsychiatric features were present in 90% of patients, and seizures and movement disorders both in 67%. Typical NMDAR-Ab encephalitis was reported in 24 children and partial phenotype without encephalopathy in seven, including predominantly psychiatric (four) and movement disorder (three). All patients received steroids, 22 (71%) received intravenous immunoglobulin, 9 (29%) received plasma exchange,and 10 (32%) received second-line immunotherapy. Of the 23 patients who were diagnosed early, 18 (78%) made a full recovery compared with only 1 of 8 (13%) of the late diagnosed patients (p=0.002, Fisher's exact test). Seven patients relapsed, with four needing additional second-line immunotherapy.

Conclusions Paediatric NMDAR-Ab-mediated neurological disease appears to be similar to adult NMDAR-Ab encephalitis, but some presented with a partial phenotype. Early treatment was associated with a quick and often full recovery.

  • Encephalitis
  • Autoantibody
  • NMDA receptors
  • immunotherapy
  • Neurology

This is an Open Access article distributed in accordance with the terms of the Creative Commons Attribution (CC BY 4.0) license, which permits others to distribute, remix, adapt and build upon this work, for commercial use, provided the original work is properly cited. See: http://creativecommons.org/licenses/by/4.0/

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