P14. Anti-NMDA receptor encephalitis: A paediatric case report

Abstract

Aim Anti-NMDA-receptor encephalitis is a rare autoimmune disease first described in 2007. One of the main challenges in daily clinical practise is its recognition and over the past years milder cases with primarily psychiatric symptoms have been recorded. So far clear treatment guidelines are missing and beside the paraneoplastic association with ovarial teratoma little is known on the origin of the underlying autoimmune process. Methods Case report. Results We present the case of a 12 years old girl who after a prodromal phase of 2–3 weeks with a strange gaze, anxiety and discrete articulation problems developed an episode with agitation and confusion lasting for a few hours; within the following days epileptic seizures and severe speech problems emerged. Movement disorders, autonomic instability, decreased consciousness or memory disturbances were missing. NMDAR antibodies in serum were negative. Lumbar puncture showed mild mononuclear pleocytosis, normal protein and an elevated NMDAR antibody titer. Cerebral MRI was normal, EEG showed slowing in the temporal regions. Treatment with high dose steroids and IVIG led to marked and rapid improvement within 1 week. IVIG therapy will be repeated after 1 month and steroids will be continued up to 3 months. Conclusions As described previously lack of NMDAR antibodies in serum does not exclude anti-NMDAR encephalitis, and necessitates testing of CSF. Rising awareness for the disease is important as recognition of milder variants and early diagnosis will result in earlier treatment and most probably better outcome.