P-EP022. Clinical diagnosis, EEG evaluation and treatment for NMDAR autoimmune encephalitis

Abstract

Introduction. NMDAR autoimmune encephalitis is a very rare disease. Autoimmune encephalitis (AE) is an increasingly recognized etiology of acute neuropsychiatric decline in adults and children. The clinical manifestations of AE are broad and include seizures, movement disorders, behavior and mood changes, psychosis, memory/ cognitive impairment, autonomic dysfunction, and altered level of consciousness. We described one case of NMDAR autoimmune encephalitis. Diagnosis was made base on clinical sign, serial electroencephalogram (EEG) patterns and NMDAR CSF evaluation. The clinical course of the disease, specific treatment and outcome were follow up until 3 months. Results. An 11-year-old female presented with focal seizure, psychiatric features, behavioral changes followed by autonomic dysfunction, orofacialbrachial dyskinesias, speech reduction and gradual altered consciousness. Serial EEG examination between August until November 2020 showed progressive changes. Initial EEG was normal (August 2020). Second EEG showed continuous slow activity 5–6 Hz generally and excessive beta activity (September 21, 2020). Last EEG (September 26, 2020) before treatment showed excessive beta activity range 14–20 Hz, Extreme Delta Brush (EDB) and Generalized Rhythmic Delta Activity (GRDA) 2–3 Hz. Based on clinical manifestation and EEG examination we start immunotherapy treatment with IVIG and Methylprednisolone IV to avoid fatal complication of the disease. Evaluation of anti-NMDAR from cerebrospinal fluid tapping was done simultaneously. Patient showed significant clinical improvement and Modified Ranking Scale improved after immunotherapy. Conclusion. Early identification of NMDAR autoimmune encephalitis manifestation is very important. Combination of symptom consist of psychiatric manifestations (behavioral changes), clinical symptom (autonomic dysfunction, orofacialbrachial dyskinesias, speech reduction and altered consciousness), should be followed by serial EEG evaluation. Immediate immunotherapy treatment can be started based on clinical diagnosis to avoid fatal complication.