Autoimmune encephalitis in children and adolescents

Encefalitis je upala moždanog parenhima. Uz virusnu infekciju kao glavni uzrok, unazad 15 godina opisani su autoimuni encefalitisi kao posebni entitet. Anti-N-Metil-D-Aspartat Receptor (NMDA-R) encefalitis je glavni i najčešći predstavnik dosad opisanih autoimunih encefalitisa. Nastaje kao posljedica autoimune reakcije na GluN1 podjedinicu NMDA-R. Bolesnici su većinom ženskog spola starosti 12-45 godina. Etiološki bolest je uzrokovana autoimunom reakcijom na tumor, većinom teratom jajnika, ili nastaje kao post-infektivna posljedica herpes simpleks encefalitisa (HSE). Bolest počinje prodromalnim stadijem u 70% pacijenata nakon koje nastupa prvi stadij bolesti karakteriziran akutnim i bizarnim simptomima iz spektra psihijatrijskih poremećaja. Ovaj stadij je diferencijalno dijagnostički bitan zbog akutno nastalih simptoma nalik psihijatrijskim bolestima ili poremećaju ovisnosti. Cilj ovog rada bio je prikazati sedam pacijenata hospitaliziranih u Klinici za infektivne bolesti „Dr. Fran Mihaljević“ u razdoblju od 2012. do 2018. godine. Medijan dobi pacijenata iznosio je 9 godina i 8 mjeseci (raspon 10 mjeseci – 17 godina i 4 mjeseca). Prosječno vrijeme od prvih simptoma do dijagnoze bolesti iznosilo je 3 tjedna. Niti jednom pacijentu nije dijagnosticiran tumor, a najčešći simptomi bili su poremećaji ponašanja, halucinacije, poremećaj pamćenja te smanjena razina svijesti i konvulzije. Svi bolesnici odgovorili su na primljenu imunoterapiju te nije zabilježen relaps bolesti niti u jednog bolesnika. Prepoznavanje ovog entiteta važno je u kliničkom svijetu kako bi se pravovremeno dala imunoterapija i spriječilo pogoršanje kliničke slike.Encephalitis is inflammation of brain parenchyma. Besides viral infection as a main causative agent, for the last 15 years, autoimmune encephalitides have been described as a specific entity. Anti-N-Methyl-D-Aspartate Receptor (anti-NMDA-R) encephalitis is the main and most commonly diagnosed representative of autoimmune encephalitides. Disease mechanism is autoimmune with the primary target of the GluN1 subunit of NMDA-R. Typically it occurs in women aged 12-45 years. This autoimmune encephalitis develops due to an autoimmune reaction triggered by a tumour, mainly ovarian teratoma, or it develops as a post-infectious consequence of herpes simplex encephalitis (HSE). Before the development of symptoms, up to 70% of patients may experience prodromal symptoms followed by the first stage of the disease characterized by acute and bizarre symptoms from the spectrum of the psychiatric diseases. This stage of the disease can be easily misdiagnosed as psychiatric disease or substance use disorder. The aim of this study conducted at the Hospital for Infectious Diseases „Dr. Fran Mihaljević“ was to analyze patients diagnosed with anti-NMDA-R encephalitis in the period 2012-2018. The median age of the patients was 9 years and 8 months. The average time which passed from disease onset to the diagnosis was 3 weeks. None of the patients were diagnosed with a tumour and the most common symptoms were behavioral symptoms, hallucinations, memory deficit, decreased level of consciousness and convulsions. All patients responded to immunotherapy and relapse was not observed among them. Fast recognition of this disease is important in the clinical world because timely given immunotherapy prevents worsening of clinical features