Given that psychiatric symptoms are common, not only during the course of the illness but also on presentation, in children and adolescents with anti-N-methyl-d-aspartate receptor encephalitis, it is important that practitioners possess an adequate understanding of the clinical features and potential treatment of this disease. We describe the clinical characteristics of 24 patients who presented to the California Encephalitis Project.

Patients were referred by physicians, and standardized forms were used to gather demographic, clinical, and laboratory data.

Twenty-four patients between ages two and 18 years were identified. Psychosis was a primary presenting symptom in two thirds of patients, and just over 20% of these were admitted to an inpatient psychiatric facility. Ultimately, all patients developed psychiatric symptoms during the course of the illness. There was a characteristic progression of symptoms that was consistent with that noted in other studies, with prominent psychiatric symptoms initially, which then progressed to seizures and autonomic instability in most patients. All of those examined developed movement abnormalities, which included primarily orofacial dyskinesias and choreiform movements.

Anti-N-methyl-d-aspartate receptor encephalitis, unlike other paraneoplastic or autoimmune encephalitides, affects those 18 years and under an estimated 40% of the time based on prior studies. Recognizing the signs and symptoms of this condition is essential to making a diagnosis and initiating timely treatment. Child psychiatrists have an important role in this disorder.