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Encefalite anti-receptor NMDA : um caso clínico
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Introdução: A encefalite anti-receptor NMDA é uma patologia autoimune recentemente descrita e cada vez mais identificada, tanto em crianças como em adultos. Caso clínico: É descrito o caso clínico de uma criança de quatro anos que iniciou um quadro de vómitos e perda de conhecimento seguido de febre, crises epilépticas e alterações do comportamento. Apresentava pleocitose no LCR; múltiplas lesões hiperintensas córtico-subcorticais em FLAIR/T2 na RMN CE; lentificação da electrogénese de base e actividade paroxística multifocal no EEG. Verificou-se a presença de anticorpos anti-receptor NMDA no soro e LCR e iniciou imunoterapia com imunoglobulina intravenosa e metilprednisolona. Por persistência dos sintomas, realizou cinco ciclos de plasmaferese associados a imunoglobulina intravenosa, com remissão clínica. Um ano após a apresentação inicial da doença, mantém-se assintomático. Conclusão: A encefalite anti-receptor NMDA pode ter uma forma de apresentação particular na faixa etária apresentada, como crises epilépticas/”encefalite”, e deve ser incluída no diagnóstico diferencial de encefalite sem agente etiológico identificado em crianças. Discutem-se os aspectos mais relevantes relacionados com o diagnóstico e tratamento desta entidade na idade pediátrica.
Background: Anti-NMDA receptor encephalitis is an increasingly well recognized autoimmune disorder in children and adults. Clinical case: Four year old boy presenting with vomiting and loss of consciousness, progressing with fever, crisis and behavioral change. The CSF revealed pleocytosis; brain MRI showed cortical/subcortical areas of increased signal on FLAIR/T2 sequences; EEG revealed generalized slowing and multifocal paroxysmal discharges. Serum and cerebrospinal fluid was positive for autoantibodies directed against the NMDA receptor, and he was treated with a regimen of immunotherapy that included intravenous immunoglobulin and methylprednisolone. Due to first line therapy failure, he underwent five cycles of plasmapheresis associated with intravenous immunoglobulin. One year after initial presentation, the patient remains symptom free. Conclusion: Anti-NMDA receptor often presents with a pattern of defining characteristics among infants and toddlers, and should be included in the differential diagnosis of encephalitis of unknown etiology in children. Here, we discuss the main challenges concerning the diagnosis and treatment of this disease in pediatric patients.
Background: Anti-NMDA receptor encephalitis is an increasingly well recognized autoimmune disorder in children and adults. Clinical case: Four year old boy presenting with vomiting and loss of consciousness, progressing with fever, crisis and behavioral change. The CSF revealed pleocytosis; brain MRI showed cortical/subcortical areas of increased signal on FLAIR/T2 sequences; EEG revealed generalized slowing and multifocal paroxysmal discharges. Serum and cerebrospinal fluid was positive for autoantibodies directed against the NMDA receptor, and he was treated with a regimen of immunotherapy that included intravenous immunoglobulin and methylprednisolone. Due to first line therapy failure, he underwent five cycles of plasmapheresis associated with intravenous immunoglobulin. One year after initial presentation, the patient remains symptom free. Conclusion: Anti-NMDA receptor often presents with a pattern of defining characteristics among infants and toddlers, and should be included in the differential diagnosis of encephalitis of unknown etiology in children. Here, we discuss the main challenges concerning the diagnosis and treatment of this disease in pediatric patients.
Descrição
Trabalho Final do Curso de Mestrado Integrado em Medicina, Faculdade de Medicina, Universidade de Lisboa, 2015
Palavras-chave
Encefalite Antirreceptor de N-Metil-D-Aspartato Pediatria