Clinical variability of children with anti-N-methyl-d-aspartate receptor encephalitis in southern Brazil: a cases series and review of the literature

• Published in: Neurological sciences Vol. 40; no. 2; pp. 351 - 356
• Main Authors: do Valle, Daniel Almeida, Galeazzi, Joselainy Stela Pires, Machado, Mayara de Rezende, dos Santos, Vanessa Catarine Silva Abreu Ribeiro, da Silva, Alcir Francisco, Lohr Júnior, Alfredo, Santos, Mara Lúcia Schmitz Ferreira, Scola, Rosana Herminia
• Format: Journal Article
• Language: English
• Published: Cham Springer International Publishing 01.02.2019; Springer Nature B.V
• Subjects: Adolescents; Central nervous system; Cerebrospinal fluid; Children; Cyclophosphamide; EEG; Encephalitis; Glutamic acid receptors; Literature reviews; Magnetic resonance imaging; Medicine; Medicine & Public Health; Monoclonal antibodies; N-Methyl-D-aspartic acid receptors; Neurology; Neuroradiology; Neurosciences; Neurosurgery; Original Article; Patients; Pleocytosis; Population studies; Psychiatry; Rituximab; Seizures; Steroid hormones; Brazil; Encephalitis; Anti-N-methyl; Autoimmune; aspartate receptor; Paediatrics; Anti-N-methyl-D-aspartate receptor
• ISSN: 1590-1874; 1590-3478
• DOI: 10.1007/s10072-018-3648-z

Purpose 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. Methods Retrospectively analyse patients diagnosed with anti-NMDAR encephalitis, from 2010 to 2018. Results 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. Conclusions 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.