CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy) is a rare inherited disease which begins with migraine and later develops repeated cerebral subcortical infarction and dementia. We present an anesthetic experience of an undiagnosed CADASIL woman complicated with preeclampsia. She developed headache, slurred speech, cognitive dysfunction and restlessness at 35 weeks' gestation and was diagnosed as hypertensive encepha- lopathy. Urgent cesarean section was decided. After ruling out meningitis by physical examination, and intracranial hemorrhage, cerebral swelling and hydro- cephalus by brain CT, spinal anesthesia was chosen. Mild sedation was necessary because the patient became restless and uncooperative during surgery. The anesthetic course was uneventful otherwise. She was either restless or lethargy and had hallucinatory episodes on 1st post-operative day. The neurologist suspected CADASIL because of multiple lacunar infarct lesions on MRI and her family history. The diagnosis was confirmed by skin biopsy and a genetic test.

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