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Wright et al1 report the first prospective surveillance study of anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis in children. Patients with anti-NMDAR encephalitis usually have memory deficits, behavioural change and psychosis early in the disease course.2 Later they may become catatonic or comatose with central respiratory failure, bizarre writhing movements of the face or limbs, and/or profound autonomic instability. Seizures may occur at any point in the disease course. Diagnosis is confirmed by detection of antibodies to the NR1 subunit of the NMDAR. With appropriate immunotherapy many patients have a good recovery, even though this may take months. A minority of patients may relapse, but these relapses also tend to respond to treatment. Immunotherapy may include intravenous immunoglobulin, plasma exchange (PLEX), rituximab, cyclophosphamide or other treatments. In this disease, antibodies to the NMDAR receptor cause cross-linking and internalisation of the receptors and decreased NMDAR-mediated synaptic currents. Intrathecal production of antibodies may be important to pathogenesis.
Anti-NMDAR encephalitis was first described in women with ovarian teratoma but men are less frequently affected. Children of either sex have increasingly been diagnosed; one large series reported that 37% of patients were under 18 years of age.3 Prior series of paediatric patients have reported more atypical presentations (speech difficulties, developmental regression, movement problems) in children compared with the typical adult presentation with psychosis and behavioural symptoms.3 ,4
The incidence of anti-NMDAR …
Funding Dana Foundation (Neuromimmunology Award), National Institute of Neurological Disorders and Stroke (K08-NS-075142-01A1).
Competing interests EL received payment from Grifols for teaching a course and provided paid expert opinions for the US Division of Vaccine Injury Compensation.
Provenance and peer review Commissioned; internally peer reviewed.