Abstract

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a newly described subtype of limbic encephalitis. This disease is probably the commonest autoimmune encephalitis predominantly affecting children and young adults. It has a higher incidence among young females. Patients manifest with a constellation of symptoms including psychosis, dyskinesia, and seizures. Antibodies against NR1/NR2 heteromers of the NMDAR glutamate receptor mediate this type of encephalitis. Patients with anti-NMDAR encephalitis might have underlying tumors, which are typically teratomas. Exploring for occult tumor and resection is crucial in management. First-tier immunotherapy include steroids, intravenous immunoglobulin and plasma exchange, while rituximab and cyclophosphamide are frequently used as second-tier immunotherapy. Around 80% of patients achieve almost complete recovery, however some patients suffer from cognitive deficits or death. Early diagnosis and prompt treatment can lead to better recovery and outcome. The unique feature of this disease reflects the intimate relationship between psychiatry and neurology. In this article, we describe the clinical phenotype and outcome of four affected children from Bahrain.

Keywords: Anti-NMDAR encephalitis, Limbic encephalitis, Epilepsy, Psychosis, Dyskinesia, Immunotherapy