Faculty of Medicine and Health Sciences, McGill University, Montreal, QC, Canada.
Department of Anesthesia, McGill University, Montreal, QC, Canada.
Can J Anaesth. 2022 Nov;69(11):1419-1425. doi: 10.1007/s12630-022-02306-0. Epub 2022 Aug 19.
Stiff person syndrome (SPS), an autoimmune disease that manifests with episodic muscle rigidity and spasms, has anesthetic considerations because postoperative hypotonia may occur. This hypotonia has been linked to muscle relaxants and volatile anesthetics and may persist in spite of neostigmine administration and train-of-four (TOF) monitoring suggesting full reversal. We present a patient with SPS who experienced hypotonia following total intravenous anesthesia (TIVA), which was promptly reversed with sugammadex. These observations are considered in light of the relevant medical literature.
A 46-yr-old female patient with SPS underwent breast lumpectomy and sentinel node biopsy. Anesthesia consisted of TIVA (propofol/remifentanil) with adjunctive administration of rocuronium 20 mg to obtain adequate intubating conditions. Despite return of the TOF ratio to 100% within 30 min, hypotonia was clinically evident at conclusion of surgery two hours later. Sugammadex 250 mg reversed residual muscle relaxation permitting uneventful extubation. A literature review identified six instances of postoperative hypotonia (TIVA, n = 3; volatile anesthetics, n = 3) in spite of neostigmine administration (n = 2) and TOF monitoring suggesting full reversal (n = 4).
Patients with SPS may show hypotonia regardless of general anesthetic technique (TIVA vs inhalational anesthesia), which can persist despite recovery of the TOF ratio and may be more effectively reversed by a chelating agent than with an anticholinesterase. If general anesthesia is required, we suggest a cautious approach to administering muscle relaxants including using the smallest dose necessary, considering the importance of clinical assessment of muscle strength recovery in addition to TOF monitoring, and discussing postoperative ventilation risk with the patient prior to surgery.
僵人综合征（SPS）是一种自身免疫性疾病，表现为阵发性肌肉僵硬和痉挛，由于术后可能出现低张力，因此存在麻醉考虑因素。这种低张力与肌肉松弛剂和挥发性麻醉剂有关，尽管给予新斯的明和肌松监测（TOF）提示完全逆转，但可能持续存在。我们报告了一例 SPS 患者，在全凭静脉麻醉（TIVA）后出现低张力，并用琥珀酸司可林迅速逆转。这些观察结果结合相关文献进行了考虑。
一位 46 岁的女性 SPS 患者接受了乳房肿块切除术和前哨淋巴结活检。麻醉采用 TIVA（丙泊酚/瑞芬太尼），并辅助给予罗库溴铵 20mg 以获得适当的插管条件。尽管 TOF 比值在 30 分钟内恢复到 100%，但在两小时后手术结束时仍出现明显的低张力。给予 250mg 琥珀酸司可林逆转残余肌肉松弛，使拔管过程顺利。文献复习发现 6 例术后低张力（TIVA，n=3；挥发性麻醉剂，n=3），尽管给予新斯的明（n=2）和 TOF 监测提示完全逆转（n=4）。
SPS 患者可能表现出低张力，无论全身麻醉技术（TIVA 与吸入麻醉）如何，尽管 TOF 比值恢复，但可能持续存在，并且用螯合剂逆转可能比用抗胆碱酯酶更有效。如果需要全身麻醉，我们建议谨慎使用肌肉松弛剂，包括使用必要的最小剂量，除了 TOF 监测外，还要考虑临床评估肌肉力量恢复的重要性，并在手术前与患者讨论术后通气风险。
