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Autoimmune encephalitis in children

clinical phenomenology, therapeutics, and emerging challenges

Dale, Russell C.a; Gorman, Mark P.b,*; Lim, Mingc,*

Current Opinion in Neurology: June 2017 - Volume 30 - Issue 3 - p 334–344
doi: 10.1097/WCO.0000000000000443

Purpose of review Auto-antibodies that bind to conformational extracellular epitopes of neuronal receptors or synaptic proteins have provided clinicians with essential biomarkers in acute neurology. This review summarizes the current status and challenges in the field.

Recent findings In children, anti-N-methyl-D-aspartate receptor encephalitis remains the most identifiable autoimmune encephalitis, although many patients have a clinical syndrome of brain inflammation in which no antibodies are identified. Anti-myelin oligodendrocyte glycoprotein antibody associated demyelination is now recognized as a major cause of monophasic and relapsing demyelination, often presenting with encephalopathy. We discuss the importance of auto-antibody detection methodology and the possible influence of intrathecal antibody synthesis on the speed of recovery and response to immune therapy. The current, often pragmatic rather than evidence-based therapeutic pathway will be discussed, highlighting key challenges such as the timing of second-line therapy, monitoring of disease activity, and identifying the patient who is responding poorly to treatment.

Summary Although there have been significant developments, future priorities include the need for paediatric-specific consensus definitions for seronegative suspected autoimmune encephalitis, novel tools for monitoring patients with autoimmune encephalitis, consensus treatment recommendations, and neuroprotective strategies.

aInstitute for Neuroscience and Muscle Research, the Children's Hospital at Westmead, University of Sydney, Sydney, Australia

bBoston Children's Hospital and Harvard Medical School, Boston, Massachusetts, USA

cEvelina London Children's Hospital and Faculty of Life Sciences and Medicine, King's College London, London, UK

Correspondence to Professor Russell C. Dale, Clinical School, Locked Bag 4001, Children's Hospital at Westmead, NSW 2145, Australia. Tel: +61 298450000; e-mail:

Copyright © 2017 Wolters Kluwer Health, Inc. All rights resereved.