Characteristics and Long-Term Outcome of Acute Ischemic Stroke in Patients With Cerebral Autosomal Dominant Arteriopathy With Subcortical Infarcts and Leukoencephalopathy Compared With Sporadic Small Vessel Occlusion

Abstract

Background: Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) often presents as acute ischemic stroke involving cerebral perforators. This study compared the clinical and imaging characteristics and stroke recurrence between patients with CADASIL and those with sporadic small vessel occlusion.

Methods: We retrospectively analyzed 70 patients with CADASIL and 70 age- and sex-matched patients with sporadic small vessel occlusion, all of whom experienced acute ischemic stroke. Clinical characteristics and imaging findings were compared, and factors associated with stroke recurrence over a 10-year follow-up period were assessed using Cox proportional hazards models.

Results: Patients with sporadic small vessel occlusion had more vascular risk factors, whereas patients with CADASIL had more extensive small vessel disease. Acute ischemic stroke lesions in CADASIL were more commonly located in the anterior circulation (78.6% versus 59.4%) and frequently involved the subcortex (28.6% versus 4.3%), centrum semiovale (25.7% versus 0%), and splenium (15.7% versus 0%). In contrast, lesions were less common in the basal ganglia (11.4% versus 32.9%) and brainstem (8.6% versus 24.3%). Multiple simultaneous lesions were more frequent in CADASIL (35.7% versus 7.1%). The 10-year stroke recurrence rate in CADASIL was 50%, with a hazard ratio (HR) of 7.12 (95% CI, 2.99-16.94; P<0.001). In multivariable Cox regression, CADASIL was the strongest independent predictor of recurrence (adjusted HR, 15.692 [95% CI, 4.452-49.166]; P<0.001), along with older age, hypertension, elevated initial diastolic blood pressure, periventricular white matter hyperintensities (grade 2), and multiple lesion involvement.

Conclusions: Patients with CADASIL patients with acute ischemic stroke have distinct imaging patterns and a significantly higher recurrence risk compared with those with sporadic small vessel occlusion.

Keywords: CADASIL; acute ischemic stroke; small vessel occlusion.