Clinical Case Reports

Biography

Biography: Pratibha Singhi

Abstract

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is an important and potentially treatable cause of autoimmune encephalitis in the pediatric age group. It is characterized by neuropsychiatric symptoms, seizures, prominent movement disorders, fluctuating level of consciousness, dysautonomia and central hypoventilation following a minor viral prodrome. In young children, identification of initial behavioral symptoms can be challenging as there may be only irritability, temper tantrums and hyperactivity initially. Diagnosis is entirely clinical and confirmation requires presence of anti-NMDA receptor (NR-1 subunit) antibody in serum and cerebrospinal fluid. Screening of occult malignancy is warranted. We retrospectively reviewed 6 children diagnosed with anti-NMDAR encephalitis from our centre. All children presented with behavioral changes, psychosis, seizures, oro-lingual-facial dyskinesia, extreme irritability, insomnia and mutism. The symptoms were persistent and course was progressive over 4-8 weeks. Neuroimaging and electroencephalography were non-specific. Intravenous pulse methylprednisolone and immunoglobulins were used as first line therapeutic agents. Only one patient responded to first line immunotherapy; five out of six children required second line immunotherapy with rituximab or cyclophosphamide. One patient recovered following rituximab and two patients showed a good response to cyclophosphamide pulse therapy. In all children the screening for tumor was negative. Anti NMDA receptor encephalitis is severe yet potentially treatable autoimmune encephalitis. “Eyes see what the mind knows” hence pediatricians need to be aware of this rapidly upcoming entity not so rare in children. Early diagnosis by high clinical suspicion and confirmation by an easily available diagnostic test makes it potentially treatable. Aggressive immunotherapy is the key to favorable outcome.