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Título

Anti-NMDA autoimmune encephalitis: descriptive analysis of 75 patients from BrAIN database

Resumo

Introduction: Anti-NMDAR encephalitis is the most common type of autoimmune encephalitis (AIE), and clinical picture involves prodromic symptoms followed by a neuropsychiatric syndrome. Patients present acute behavioral changes, epilepsy, memory impairment, reduced level of consciousness movement disorders, language disturbances and autonomic instability. Pediatric population (<13 years old) usually present seizures and movement disorders, while adult patients tend to have behavioral changes. Data from the Brazilian population are lacking. Objective: To describe clinical and epidemiological profile from 75 anti-NMDAR encephalitis patients and to compare with prior reports. Method: Chart of patients with anti-NMDAR antibodies from BrAIN (Brazilian autoimmune encephalitis network) database from 2017 to 2022 were reviewed. Information on clinical, epidemiological and treatment were compiled. Patients were divided in two age groups: pediatric (younger than 13) and adult (13 or older). Results Of the 130 patients with AIE, we found 75 (58%) patients with anti-NMDAR antibodies. Approximately 69% were female, mean age was 13,8 ±10,1 years. There were 28 (37%) pediatric patients. Thirty-five (47%) patients presented prodromes, most commonly headache (n=17, 23%;), fever (n=17,23%;) and nausea/vomiting (n=10, 13%;). The most reported symptoms were behavioral, psychiatric, and cognitive changes (99%; n=74), epilepsy (85%; n=64) and movement disorders (81%; n=61). Most common symptoms in adult and pediatric population were movement disorders. Pleocytosis was reported in 56%. Abnormal EEG was present in 87% (n=65) and 18% (n=12) had extreme delta brush. Thirty-two patients (43%) had abnormal brain MRI, ten of them were children. Two patients reported neoplasia, one ovarian teratoma and the other a pulmonary mass under investigation. Methylprednisolone was the most common first line treatment (n=54, 52%) followed by intravenous immunoglobulin (n=35, 47%). Conclusion: In Brazil, anti-NMDAR encephalitis follows the typical clinical picture, being more frequent among young females. We found a lower association with neoplasm in our population when compared to other series.

Palavras Chave

NMDAR, autoimmune encephalitis