Case descriptionWe describe the anesthetic management and postoperative care of a 36 years old pregnant woman with CADASIL disease who needed urgent cesarean section. According to her past medical history, she was already diagnosed as CADASIL syndrome 3 years ago. Her medical history included left hemi lateral paresis, accompanied by walking difficulties, urinary incontinence, psychiatric symptoms, apathy, diabetes mellitus, hypertension, and ovarian cyst. CADASIL diseases, was diagnosed after a hemi paretic attack when she was 32 years old. As a consequence of headache, was followed by neurologic symptoms. After admission to hospital, the clinical and neurological examination, MRI and CT-scan, and by genetic examinations, CADASIL disease was diagnosed. She got one dilatation and curettage due to a missed abortion pregnancy under sedation, 2 years ago at a primary care clinic. Now, her symptoms associated with CADASIL were intermittent headache and left hemi lateral paresis, and hypertension. She was taking methyldopa and aspirin (80 mg daily). Her diabetes was controlled by Insulin during pregnancy. She admitted to the Alzahra hospital due to hypertension one day ago. She received 5mg hydralazine and stopped aspirin. On preoperative evaluation, there were no abnormalities in laboratory tests and radiologic evaluations except slightly elevated U/A protein 1+. Her brain CT was checked two months ago revealed the confluent low densities at the subcortical and deep white matter of both cerebral hemispheres and several small old infarctions in both basal ganglia and thalami. But the neurologist who examined the patient after admission judged her still neurologically normal.Because of recent aspirin medication, we decided to conduct general anesthesia with informed consent. After premedication with ranitidine 50 mg and metoclopramide 10 mg IV, the patient was taken to the operating room. With standard monitoring (ECG, pulse oximetry, noninvasive blood pressure, and capnography) and preoxygenation, a rapid sequence induction with cricoid pressure using fentanyl, thiopental, and succinylcholine was carried out. For intubation used a cuffed 7.0 mm ETT. Neuromuscular block was achieved with atracurium, and anesthesia was maintained by controlled ventilation with an oxygen/N2O 50%/50% and isoflurane 1% mixture. With recommendations concerning the anaesthetic management of patients with CADASIL arteriopathy, we kept mean arterial blood pressure greater than 60 mm Hg and end-tidal carbon dioxide around 40 mm Hg so as to prevent any cerebral ischemic or vasospastic phenomenon. Patient's blood sugar within the perioperative was in the normal range. Moreover, the cerebral venous return was preserved by the patient received 8 ml/kg of ringer's solution in the left lateral position. The condition of neonate was assessed by apgar score at 1 st and 5 th after delivery who was 8 and 9 respectively. Mother received oxytocin 5 IU by continues infusion after delivery. Surgery and recovery were uneventful. The neurological examination ...