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近期诊断为僵人综合征患者的急诊麻醉管理

Emergent Anesthetic Management in a Patient Recently Diagnosed With Stiff Person Syndrome.

作者信息

Pinto Ana Sofia, Dinis Rita Lopes, Godinho Pedro, Carmona Cristina

机构信息

Department of Anesthesiology and Pain Therapy, Hospital Professor Doutor Fernando Fonseca, Amadora, PRT.

出版信息

Cureus. 2024 Oct 5;16(10):e70899. doi: 10.7759/cureus.70899. eCollection 2024 Oct.

Abstract

Stiff person syndrome (SPS) is a rare autoimmune neurological disorder characterized by muscle rigidity and episodic spasms that involve axial and limb musculature. It has important implications during anesthesia, as it leads to gamma-aminobutyric acid (GABA)-mediated inhibitory networks malfunction. This report describes the anesthetic management of a 56-year-old patient with SPS and hereditary spherocytosis undergoing emergent splenectomy due to splenic hematoma and hemoperitoneum after a fall. Total intravenous anesthesia (TIVA) was performed with the adjunctive administration of rocuronium in order to obtain adequate intubation and surgical conditions. Careful management of patients with SPS is strongly suggested given their sensitivity to inhalational anesthetics and neuromuscular blockers, which can lead to hypotonia and muscle weakness requiring maintenance of mechanical ventilation in the postoperative period.

摘要

僵人综合征(SPS)是一种罕见的自身免疫性神经疾病,其特征为肌肉僵硬和发作性痉挛,累及躯干和四肢肌肉组织。它在麻醉期间具有重要影响,因为它会导致γ-氨基丁酸(GABA)介导的抑制性神经网络功能失调。本报告描述了一名56岁患有SPS和遗传性球形红细胞增多症的患者,因跌倒后脾血肿和腹腔积血而接受急诊脾切除术的麻醉管理。采用全静脉麻醉(TIVA)并辅助使用罗库溴铵,以获得足够的插管条件和手术条件。鉴于SPS患者对吸入性麻醉剂和神经肌肉阻滞剂敏感,这可能导致肌张力减退和肌肉无力,术后需要维持机械通气,因此强烈建议对这类患者进行仔细管理。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/704c/11534327/2e6dc2b31980/cureus-0016-00000070899-i01.jpg

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