Abstract
Segawa syndrome (dopa-responsive dystonia [DRD]) is a rare neurometabolic disorder characterized by progressive dystonia, diurnal variation and tremors. It is caused by an enzymatic defect (a mutation of the GTPCH1 gene located on chromosome 14q) in the synthesis of tetrahydrobiopterin, an important substrate for dopamine synthesis. In the case of early correct diagnosis, clinical symptoms are well-controlled by levodopa therapy. The disease has several features which may lead to organ dysfunctions (e.g. torticollis, scoliosis, dysphagia and immobilization), which may be of concern for the anesthesiologist. Presenting two case reports of female patients undergoing elective cesarean section and breast cancer surgery, the main principles of perioperative management are discussed. Either techniques of regional or general anesthesia can be performed safely. Preoperative medication with levodopa should not be interrupted. Pharmacological agents with an antidopaminergic mode of action have to be avoided as well as significant pain and emotional stress situations in the perioperative period. Surgery in an ambulatory setting may not be recommended.