Sinha A, Hartsilver E L
Department of Anaesthetics, Royal Devon and Exeter NHS Foundation Trust, Exeter, UK.
Int J Obstet Anesth. 2009 Jan;18(1):67-72. doi: 10.1016/j.ijoa.2008.07.003. Epub 2008 Nov 20.
Dopa-responsive dystonia, also known as hereditary progressive dystonia with diurnal fluctuation or Segawa's syndrome, is a rare hereditary progressive dystonia with two striking clinical features: a marked diurnal fluctuation of symptoms with symptoms worsening throughout the day and improving after sleep, and a dramatic response to levodopa therapy. Whilst rare, it is treatable, with function being normal or near normal after levodopa therapy. We present our experience of providing anaesthesia for caesarean section in a patient with dopa-responsive dystonia and discuss the safety of levodopa therapy during pregnancy and the anaesthetic management of these patients.
多巴反应性肌张力障碍，也称为伴有日间波动的遗传性进行性肌张力障碍或Segawa综合征，是一种罕见的遗传性进行性肌张力障碍，具有两个显著的临床特征：症状有明显的日间波动，症状在一天中逐渐加重，睡眠后改善；对左旋多巴治疗有显著反应。虽然罕见，但它是可治疗的，左旋多巴治疗后功能正常或接近正常。我们介绍了为一名多巴反应性肌张力障碍患者进行剖宫产麻醉的经验，并讨论了左旋多巴治疗在孕期的安全性以及这些患者的麻醉管理。
