钠通道性肌强直患者的麻醉管理：一例报告
Anesthetic management of a patient with sodium-channel myotonia: a case report.
作者信息
Matsumoto Naohisa, Nishimoto Rei, Matsuoka Yoshikazu, Takeda Yoshimasa, Morimatsu Hiroshi
机构信息
Department of Anesthesiology and Resuscitology, Okayama University Hospital, 2-5-1 Shikata-cho, kita-ku, Okayama City, Okayama, 700-8558, Japan.
出版信息
JA Clin Rep. 2019 Nov 25;5(1):77. doi: 10.1186/s40981-019-0300-8.
BACKGROUND
Sodium-channel myotonia (SCM) is a nondystrophic myotonia, characterized by pure myotonia without muscle weakness or paramyotonia. The prevalence of skeletal muscle channelopathies is approximately 1 in 100,000, and the prevalence of SCM is much lower. To our knowledge, this is the first report on anesthetic management of a patient with SCM.
CASE PRESENTATION
A 23-year-old woman with congenital nasal dysplasia and SCM was scheduled to undergo rhinoplasty with autologous costal cartilage. Total intravenous anesthesia without muscle relaxants was administered followed by continuous intercostal nerve block. Although transient elevation of potassium level in the blood was observed during surgery, the patient did not show exacerbation of myotonic or paralytic symptoms in the postoperative period.
CONCLUSION
Total intravenous anesthesia and peripheral nerve block can be administered safely to a patient with SCM. However, careful monitoring of the symptoms and electrolytes is recommended.
背景
钠通道性肌强直（SCM）是一种非营养不良性肌强直，其特征为单纯性肌强直，无肌肉无力或副肌强直。骨骼肌通道病的患病率约为十万分之一，而SCM的患病率则低得多。据我们所知，这是首例关于SCM患者麻醉管理的报告。
病例介绍
一名23岁患有先天性鼻发育异常和SCM的女性计划接受自体肋软骨隆鼻手术。采用无肌松剂的全静脉麻醉，随后进行连续肋间神经阻滞。尽管手术期间观察到血液中钾水平短暂升高，但患者在术后并未出现肌强直或麻痹症状加重的情况。
结论
全静脉麻醉和外周神经阻滞可安全用于SCM患者。然而，建议仔细监测症状和电解质。
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