Neurodevelopmental outcomes in paediatric immune-mediated and autoimmune epileptic encephalopathy

Abstract

Recognition of paediatric autoimmune/immune-mediated encephalitis and epileptic encephalopathy (e.g. NMDAR-Ab encephalitis) has rapidly increased over the last ten years. While we are succeeding in the diagnosis and identification and even early treatment of these encephalitidies, with studies describing >80% are making a "good" recovery, we are now recognising that a "good" medical outcome does not cover the cognitive, social and behavioural sequelae that can occur, particularly in paediatric patients. Basic measures of medical outcome, for example the modified Rankin Scale (MRS) or the Paediatric Cerebral Performance Category (PCPC), offer the advantage of being quick to use, but do not reveal the more complex difficulties that can impact the future of affected children. This article reviews the current literature on neurodevelopmental outcomes in children affected with autoimmune and immune-mediated encephalitis/epileptic encephalopathy and provides guidance on post-onset surveillance aimed at identifying those most likely to experience ongoing long-term difficulties.

Keywords: ADEM; Autoimmune encephalitis; Cognition/behaviour; MOG antibodies; Neurodevelopmental outcomes; Paediatric NMDAR-Ab encephalitis.