Abstract TP202: Risk of Incident Stroke and Dementia in Cadasil : A 6-Year Follow-Up of 85 Korean Patients

Abstract

Background: CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy) is the most common hereditary cerebrovascular disease. The most frequent clinical manifestation is an ischemic stroke, and the risk of incident stroke was very high in Caucasian CADASIL patients. Also, two-thirds of patients with CADASIL developed dementia by age 65 in Caucasian. Although clinical manifestations of the Asian CADASIL patients have been reported to be different from those of Caucasian, little is known for clinical course and long-term prognosis. This study aimed to investigate the risk of incident stroke and dementia in Korean CADASIL patients. Methods: We retrospectively reviewed the medical records of patients who were diagnosed with CADASIL at our institution from March 2012 to February 2015. The patients were selected by the following criteria; (1) aged ≥18 years, (2) diagnosis of CADASIL by a NOTCH3 gene mutation, (3) followed for at least 36 months. Primary outcomes were (1) any stroke (ischemic or hemorrhagic), (2) dementia, and (3) death of any cause. Results: Of 102 CADASIL patients who were evaluated at baseline, 85 patients met the inclusion criteria (mean age 68.9±12.1 years). The median follow-up was 57 months (interquartile range: 50-68 months). Of the 85 patients, 46 patients (54.1%) had a history of ischemic or hemorrhagic stroke, and 17 patients (20.0%) had dementia at baseline examination. Of the 85 patients, 13 patients (15.3%) experienced a new ischemic stroke. The risk of incident ischemic strokes was significantly greater in patients with a history of stroke than in without (23.9% vs. 5.1%, P=0.016). However, vascular risk factors such as hypertension, diabetes, hyperlipidemia, and smoking were not associated with increased risk of incident stroke. Of 68 patients who had no dementia at baseline, incident dementia developed in 11 patients (16.2%). During follow-up, two patients (2.3%) died due to lung cancer and pneumonia respectively. Conclusions: The risk of incident ischemic stroke or dementia seems to be lower than expected in Korean CADASIL patients. These findings suggest that clinical course and prognosis could be rather benign in Korean patients compared with the Caucasian patients with CADASIL.