Abstract | OBJECTIVE: 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.
|
Authors | Sukhvir Wright, Yael Hacohen, Leslie Jacobson, Shakti Agrawal, Rajat Gupta, Sunny Philip, Martin Smith, Ming Lim, Evangeline Wassmer, Angela Vincent |
Journal | Archives of disease in childhood
(Arch Dis Child)
Vol. 100
Issue 6
Pg. 521-6
(Jun 2015)
ISSN: 1468-2044 [Electronic] England |
PMID | 25637141
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
|
Copyright | Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions. |
Chemical References |
- Autoantibodies
- Receptors, N-Methyl-D-Aspartate
|
Topics |
- Adolescent
- Autoantibodies
(blood)
- Autoimmune Diseases of the Nervous System
(diagnosis, epidemiology, therapy)
- Child
- Child, Preschool
- Encephalitis
(diagnosis, epidemiology, therapy)
- Female
- Humans
- Immunotherapy
- Infant
- Male
- Prospective Studies
- Receptors, N-Methyl-D-Aspartate
(immunology)
- Surveys and Questionnaires
- United Kingdom
(epidemiology)
|