Early diagnosis of autoimmune encephalitis in children

Abstract

Autoimmune neurological syndromes have become increasingly recognised over the last decade. Early immune suppressive treatment of autoimmune encephalitis can improve outcomes1. However, symptomatic management of autoimmune encephalitis, particularly anti-NMDAR encephalitis is not well described in the literature. In children presenting with an acute encephalopathy the differentials for management are based on early clinical features and investigation modalities such as electroencephalography (EEG) and magnetic resonance imaging (MRI). Autoimmune encephalitis often presents with movement disorders accompanying other clinical features2. Some of these syndromes such as anti-NMDAR encephalitis and autoimmune basal ganglia encephalitis were recognised entities at the start of my PhD tenure, while others like autoimmune relapse after Herpes encephalitis with movement disorders were not well understood, although thought to be immune mediated. At the start of my PhD, certain EEG and MRI features were proposed to be indicative of particular syndromes such as extreme delta brush in anti-NMDAR encephalitis, periodic discharges in Herpes simplex encephalitis (HSE) and bilateral basal ganglia changes in autoimmune basal ganglia encephalitis3. Hypotheses • The description of discriminatory movement disorder phenomenology, EEG features on early EEG and MRI brain patterns can help early recognition and treatment of autoimmune encephalitis in children. • Symptomatic management of anti-NMDAR encephalitis utilises polypharmacy and pleiotropic effects of various medications. • Herpes encephalitis relapse with movement disorder is autoimmune.