Brazilian Journal of Anesthesiology
https://bjan-sba.org/article/doi/10.1016/j.bjane.2013.02.004
Brazilian Journal of Anesthesiology
Clinical Informations

Anesthesia in a patient with Stiff Person Syndrome

Anestesia em paciente com síndrome da pessoa rígida

Ozgur Yagan; Kadir Özyilmaz; Ahmet Özmaden; Özgür Sayin; Volkan Hanci

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Abstract

Abstract Stiff Person Syndrome (SPS), typified by rigidity in muscles of the torso and extremities and painful episodic spasms, is a rare autoimmune-based neurological disease. Here we present the successful endotracheal intubation and application of TIVA without muscle relaxants on an SPS patient. A 46 years old male patient was operated with ASA-II physical status because of lumber vertebral compression fracture. After induction of anesthesia using lidocaine, propofol and remifentanil tracheal intubation was completed easily without neuromuscular blockage. Anesthesia was maintained with propofol, remifentanil and O2/air mixture. After a problem-free intraoperative period the patient was extubated and seven days later was discharged walking with aid. Though the mechanism is not clear neuromuscular blockers and volatile anesthetics may cause prolonged hypotonia in patients with SPS. We think the TIVA technique, a general anesthetic practice which does not require neuromuscular blockage, is suitable for these patients.

Keywords

Anesthesia, Total intravenous anesthesia, Stiff Person Syndrome

Resumo

Resumo A síndrome da pessoa rígida (SPR), caracterizada pela rigidez dos músculos do tronco e das extremidades e por episódios de espasmos dolorosos, é uma doença neurológica autoimune rara. Apresentamos o ocaso de intubação endotraqueal bem-sucedida e aplicação de AVT sem relaxantes musculares em um paciente com SPR. Paciente do sexo masculino, 46 anos, estado físico ASA-II, submetido à cirurgia devido à fratura por compressão da coluna lombar. Após a indução da anestesia com lidocaína, propofol e remifentanil, a intubação traqueal foi concluída com facilidade, sem bloqueio neuromuscular. A anestesia foi mantida com propofol, remifentanil e mistura de ar/O2. Após o período intraoperatório, que transcorreu sem intercorrências, o paciente foi extubado e, sete dias depois, recebeu alta, deambulando com ajuda. Embora o mecanismo não esteja claro, bloqueadores neuromusculares e anestésicos voláteis podem causar hipotonia prolongada em pacientes com SPR. Acreditamos que a técnica de AVT, uma prática de anestesia geral que não requer bloqueio neuromuscular, é adequada para esses pacientes.

Palavras-chave

Anestesia, Anestesia venosa total, Síndrome da pessoa rígida

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