Neuropediatrics 2012; 43 - VS12_06
DOI: 10.1055/s-0032-1307166

Anti-NMDA Receptor Encephalitis in children

K von Engelhardt 1, J Pietz 1, S Schubert-Bast 1
  • 1Zentrum für Kinder- und Jugendmedizin Heidelberg, Heidelberg, Germany

Aims: Anti-NMDA receptor encephalitis is increasingly frequent diagnosed also in children. The disease is caused by antibodies against the NR1 subunit of the NMDA receptor which plays an important role for synaptic plasticity und learning processes. The typical course of the disease is multistage starting with prodromes, followed by psychosis, movement disorders, seizures, vegetative dysregulation, and occasionally coma. Fatal casualities are described. The diagnosis is often delayed due to the variety of clinical symptoms especially in childhood.

Methods: We present three casuistics to exemplify the variability of the disease. We report on three girls aged 2 ½, 6 and 16 years with different disease courses. Different symptoms and stages of anti-NMDA receptor encephalitis are illustrated with videos.

Results: The three patients were treated with methylprednisolone and immunglobulines. Two of them received rituximab in addition. Plasmapharesis was performed in the 16 year old girl because of severity of symptoms. All patients recovered completely within a period of 2 1/2 to 4 months after start of treatment.

Conclusion: Anti-NMDA receptor encephalitis has to be kept in mind as an important differential diagnosis in children with encephalitis of unknown origin, movement disorders, seizures and in comatose patients.