Anaesthesia for caesarean section in a patient with dopa-responsive dystonia or Segawa’s syndrome

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.