Outcomes in Paediatric Anti-NMDA Receptor Encephalitis with Early Aggressive Immunotherapy Including Rituximab

Babiker, M.O.E., Addison, R., Thomson, A., MacLeod, S., O'Regan, M.E., Callaghan, M., Dorris, L. and Zuberi, S.M. (2013) Outcomes in Paediatric Anti-NMDA Receptor Encephalitis with Early Aggressive Immunotherapy Including Rituximab. Annual Meeting of the British Paediatric Neurology Association, Manchester, UK, 23-25 Jan 2013. pp. 17-33. (doi:10.1111/dmcn.12068)

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Abstract

Objective: To describe neurological outcomes with aggressive immune-modulatory therapy, including rituximab in two cases of anti–N-methyl-D-aspartate receptor (NMDAR- Ab) encephalitis. Background: Up to 40% of NMDAR-Ab encephalitis presents in children and adolescents. It can result in significant acute and chronic neurological morbidity with a risk of death. Aggressive immune therapy has been suggested but no consensus exists on treatment protocols. Results: Case 1: A 7-year-old girl, presented with a cluster of focal epileptic seizures evolving into bilateral convulsive movements. MRI was normal. EEG was consistent with a genetic generalised epilepsy. She represented 2 months later with seizures, orofacial dyskinesia, poor memory, severe ag itation and insomnia. Neu ro psychological findings showed severe receptive and expressive lan guage disorder, low Perceptual Reasoning IQ and slowed Processing Speed (PS). NMDAR-Ab were positive. Methylpred- nisolone and IVIG were given concomitantly. Within 3 weeks she received rituximab with subsequent marked improvement in behaviour, cognition and PS 4 weeks post treatment. At 1 year sheisseizure-freewithnorelapses. Case 2: Following 2 weeks of intermittent aggression and headaches, a 14 year old girl presented with aphasia and a cluster of focal epileptic seizures. Over 3–4 days she developed orofacial dyskinesia, intermittent agitation with a complex hyperkinetic movement disorder and autonomic instability. MRI was normal, EEG encephalopathic. NMDAR-Ab were positive. She was trea- ted concomitantly with methylprednisolone and IVIG. In week two she had plasmapheresis and in week three rituximab. Herneurology improved dramatically with normal speech and behav iour at 6 weeks. Initial neuropsychological results indicated severe receptive and expressive language disorder with low scores on the WISC-IV and the Children’s Memory Scale. Conclusion: In two patients with NMDAR-Ab encephalitis early aggressive immunotherapy including rituximab resulted in rapid resolution of neurological and behavioural signs. Neuropsycho logical deficits have improved but not resolved at short term fol low up.

Item Type:Conference or Workshop Item
Additional Information:Abstract published in Developmental Medicine and Child Neurology 55(S1):30.
Status:Published
Refereed:Yes
Glasgow Author(s) Enlighten ID:Zuberi, Dr Sameer and Dorris, Dr Liam
Authors: Babiker, M.O.E., Addison, R., Thomson, A., MacLeod, S., O'Regan, M.E., Callaghan, M., Dorris, L., and Zuberi, S.M.
College/School:College of Medical Veterinary and Life Sciences > Institute of Health and Wellbeing > Mental Health and Wellbeing
College of Medical Veterinary and Life Sciences > School of Medicine, Dentistry & Nursing
Journal Name:Developmental Medicine & Child Neurology
ISSN:0012-1622

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