Clinical variability of children with anti-N-methyl-d-aspartate receptor encephalitis in southern Brazil: a cases series and review of the literature
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Anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis is an immune-mediated disease of the central nervous system (CNS). The aim of this study was to describe the variability of clinical presentation in anti-NMDAR encephalitis, treatment and outcomes in a case series of children and adolescents.
Retrospectively analyse patients diagnosed with anti-NMDAR encephalitis, from 2010 to 2018.
The study population consisted of nine children with anti-NMDAR encephalitis from southern Brazil, six females and three males, aged 5 months to 16 years (mean 5 years). The time of follow-up varied between 1 and 7 years, with a mean of 3 years. The most frequent first manifestation consisted of seizures. All patients described had psychiatric symptoms and a wide spectrum of neurologic findings. Five patients had unilateral symptoms. Magnetic resonance imaging and electroencephalogram were normal in most patients. Cerebrospinal fluid pleocytosis occurred in five patients. All patients were administered immunoglobulin and/or steroids. Seven patients (78%) required cyclophosphamide and/or rituximab. Almost half of the patients fully recovered from all symptoms.
A wide variety of symptoms were observed in this study and, although unilateral symptoms are rarely reported in the literature, a high frequency was observed among Brazilian children. Alternatives to first-line therapy should be considered in patients with clinical suspicion, even if they have not had a good response with first-line therapy.
KeywordsAnti-N-methyl-d-aspartate receptor Encephalitis Paediatrics Autoimmune
We would like to express our sincere gratitude to Prof. Josep Dalmau from the University of Barcelona and Prof. Lindsey McCracken from the University of Pennsylvania, for the antibody testing in our patients.
Daniel Almeida do Valle: acquisition of data, analysis and interpretation of data, revisions of the manuscript
Joselainy Stela Pires Galeazzi: acquisition of data, analysis and interpretation of data, revisions of the manuscript
Mayara de Rezende Machado: acquisition of data, analysis and interpretation of data
Vanessa Catarine Silva Abreu Ribeiro dos Santos: acquisition of data, analysis and interpretation of data
Alcir Francisco da Silva: analysis and interpretation of the data, critical revision of the manuscript
Alfredo Lohr Júnior: analysis and interpretation of the data, critical revision of the manuscript
Mara Lúcia Schmitz Ferreira Santos: study design, analysis and interpretation of the data, critical revision of the manuscript
Rosana Herminia Scola: study design, drafting and critical revision of the manuscript for important intellectual content
Compliance with ethical standards
Conflict of interest
The authors declare that they have no conflict of interest.
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