Background: Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a rare but the most common genetic cause of ischemic strokes.(1)(2) It affects 500 families worldwide, mainly European. (2) Few cases of anesthetic management were published.(1) (2)
Case report: 48 year-old man with history of coronary angioplasty, hypertension and asymptomatic CADASIL syndrome was admitted for partial parotidectomy. Aspirin was stopped 5 days before and surgery took place under balanced general anesthesia, using ASA standard monitoring, capnography and BIS. Remifentanil, propofol and sevoflurane were used for induction. Intubation was performed without muscle relaxation. Anesthesia was maintained with remifentanil and desflurane. Through the procedure, the patient was hemodynamically stable, with MAP 60-70 mmHg and heart rate 50-60 bpm. The end-tidal carbon dioxide was kept between 27 to 34 mmHg. Surgeons repeatedly monitored facial nerve function and the procedure went successfully. Extubation went uneventfully. Paracetamol and an elastomeric pump perfusion with tramadol and droperidol were administered. Anti-platelet therapy was reintroduced after 24h of surgery and the patient was discharged at 3rd postoperative day. At 1 month reevaluation there weren’t any complications. 
Discussion: CADASIL syndrome is a progressive non-arteriosclerotic arteriopathy with variable presentation: migraine, stroke, dementia, cognitive impairment and neuropsychiatric symptoms. (1) (2) Cerebral hypoperfusion appears early and cerebral autoregulation and reactivity to carbon dioxide may be impaired. (1) (2) Most patients take anti-platelet medication. (1)(2) Cardiovascular risk factors hasten the disease’s progression.(2) The main goal is the prevention of cerebral ischemia or vasospastic events by maintaining normal levels of blood pressure, normocapnia and normovolemia.(1) (2) Whenever locorregional anesthesia is a possibility, it can be safely performed.(1) (2)
Learning points: Anesthetic management of CADASIL syndrome should focus on maintenance of adequate cerebral perfusion while preventing ischemic and vasospastic events. Be aware of common use of anti-platelet medication. 
References: 
1 – Chon, Lee. Anesthesic management of a patient with CADASIL syndrome. Anesthesiology and Pain Medicine. 2012; 7. 
2- Errando et al. Anesthesic management of a patient diagnosed with CADASIL. Anestesiología, Reanimación y Terapéutica del dolor. 2012; 59.

Anesthetic management of a patient with CADASIL syndrome