Dados do Trabalho

Título

AUTOIMMUNE ENCEPHALITIS IN THE PEDIATRIC PATIENT: A RETROSPECTIVE STUDY AT A TERTIARY CARE CENTER

Introdução

Autoimmune Encephalitis (AE) is a group of treatable inflammatory diseases of the central nervous system that affect children and adolescents, most of whom are previously healthy. They are a significant cause of morbidity and mortality worldwide. They can be triggered by tumors, viral infections or have a cryptogenic etiology. Anti-NMDA receptor encephalitis is the prototype of AE in Pediatrics. The speed of recovery, the degree of residual deficit and the rate of relapses naturally depend on the type of AE.

Objetivo

To describe the clinical profile of children and adolescents with diagnosis of autoimmune encephalities followed at a single tertiary care center.

Método

We conducted retrospective evaluation of children and adolescents (age range: 0-16 years) diagnosed with autoimmune encephalitis, treated at a single tertiary care center, between July 2011 and June 2020. Demographic, clinical, laboratory, therapeutic and of clinical evolution were reviewed.

Resultados e Conclusões

Ten patients were diagnosed with autoimmune encephalitis: 70% with confirmed anti-NMDA receptor encephalities, 10% with anti-GAD encephalities and 20% seronegative. 50% were female and 50% male. 40% were preschoolers, with a mean age of 2.5 years; 50% schoolchildren, with a mean age of 7.4 years and 10% adolescents, with a mean age of 13 years. During clinical evolution, 70% required intensive care unit care. 90% had viral prodromes preceding the onset of neurological symptoms. All patients presented with seizures, cognitive and behavioral changes, sleep disorders, and movement disorders. 60% had CSF pleocytosis and/or hyperproteinorachia. 67% of patients showed abnormal MRI findings. No neoplasia was detected in the screening. 100% received first-line therapy, and only 20% needed second-line therapy. During clinical follow-up, one child had disease recurrence and one evolved with epilepsy and learning disability.
The results obtained are in line with literature data, and provide guidance on initial laboratory investigation, the importance of early and accurate diagnosis, therapy and expected results with clinical follow-up.

Palavras Chave

encephalitis; autoimmunity; pediatrics

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Fonte de Fomento (se houver)

Referências (se houver)

1. DALE, Russell C.; GORMAN, Mark P.; LIM, Ming. Autoimmune encephalitis in children. CurrentOpinion In Neurology, [s.l.], v. 30, n. 3, p.334-344, jun. 2017. Ovid Technologies (Wolters Kluwer Health).
http://dx.doi.org/10.1097/wco.0000000000000443.

2. WELLS, Elizabeth et al. Neuroimmune Disorders Of The central nervous system in children in the molecular era. NatureReviewsNeurology, [s.l.], v. 14, n. 7, p.433-445, 20 jun. 2018. Springer Science and
Business Media LLC. http://dx.doi.org/10.1038/s41582-018-0024-9.

3. DALMAU, Josep; GRAUS, Francesc. Antibody-MediatedEncephalitis. New EnglandJournalOf Medicine, [s.l.], v. 378, n. 9, p.840-851, mar. 2018. Massachusetts Medical Society.
http://dx.doi.org/10.1056/nejmra1708712.

Área

Neuroimunologia, esclerose múltipla e outras doenças desmielinizantes