*Correspondence: acoan@unicamp.br.
Abstract
Background: 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.
Objective: To describe the clinical profile of children and adolescents with diagnosis of autoimmune
encephalities followed at a single tertiary care center.
Methods: 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
clinical evolution were reviewed.
Results: 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.
Conclusion: 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.
