Stiff-Person Syndrome: A Review
DOI:
https://doi.org/10.46531/sinapse/AR/220033/2022Palavras-chave:
Síndrome da Pessoa Rígida/ diagnóstico, Síndrome da Pessoa Rígida/ etiologia, Síndrome da Pessoa Rígida/ tratamentoResumo
A síndrome de stiff-person é uma doença autoimune neurológica rara, que se manifesta por rigidez muscular progressiva e espasmos dolorosos, com envolvimento predominante dos músculos axiais e proximais dos membros. A maioria dos doentes apresenta anticorpos dirigidos à descarboxilase do ácido glutâmico, o passo limitante na produção do neurotransmissor inibitório ácido gama-aminobutírico. A síndrome de stiff-person paraneoplásica associa-se frequentemente a anticorpos anti-anfifisina e a carcinoma da mama. O tratamento com fármacos que aumentam o tónus GABA-érgico, incluindo a classe das benzodiazepinas, combinado com imunoterapia pode melhorar os sintomas neurológicos, no entanto, o prognóstico é imprevisível e a recuperação espontânea é rara. A literatura relativa a este assunto ainda é escassa e, por se tratar de uma entidade rara, é pouco reconhecida e provavelmente subdiagnosticada. Este artigo de revisão descreve as principais atualizações e sistematiza a informação existente acerca deste tema.Downloads
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Direitos de Autor (c) 2024 Ana Azevedo, Rita Gomes, Joana Damásio, Ernestina Santos
Este trabalho encontra-se publicado com a Creative Commons Atribuição-NãoComercial 4.0.
