Clinical and MRI Profiles Predicting Clinical Overt Stroke in Patients
with CADASIL

J Korean Neurol Assoc > Volume 30(2); 2012 > Article

Journal of the Korean Neurological Association 2012;30(2): 93-99.

카다실 환자에서 뇌졸중발생 유무에 따른 임상증상과 뇌자기공명영상소견의 차이

이정석, 최재철 강사윤 송숙근 강지훈 송정국 a 나해리 b

제주대학교 의과대학 신경과학교실, 예방의학교실 a , 보바스기념병원 신경과 b

Clinical and MRI Profiles Predicting Clinical Overt Stroke in Patients with CADASIL

Jung Seok Lee

Departments of Neurology and Preventive Medicine a , Jeju National University School of Medicine, Jeju, Korea Department of Neurology b , Bobath Memorial Hospital, Seongnam, Korea

Abstract

Background: Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an inherited arteriopathy caused by mutation in the Notch 3 gene. Cognitive impairment, which is the second most frequent clinical manifestation, worsens with recurrent stroke. Comparison studies between the pre- and poststroke phases in CADASIL have not yet been performed in Asia. Here we describe the clinical characteristics of both the pre- and poststroke phases and identified the risk factors for stroke in CADASIL.
Methods: Fifty-three consecutive patients were investigated. Patients were divided into two groups depending on the presence (poststroke group; n=31) or absence (prestroke group; n=22) of clinically overt stroke. All patients underwent an MRI scan with the same protocol. Cognition was assessed by applying detailed neuropsychological tests.
Results: The poststroke group exhibited an increase in lacunae and cerebral microbleeds. The memory scores on the Alzheimer’s Dementia Assessment Scale cognitive subscale and the number of correct Stroop color-naming scores were lower in the poststroke group than in the prestroke group. Hypertension was more prevalent in the poststroke group, while chronic headache was more prevalent in the prestroke group.
Conclusions: These findings suggest that cerebral microbleeds as well as lacunae predict the risk of clinically overt stroke, which leads to a worsening of frontal-lobe function in CADASIL. Key Words: Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), Cerebral microbleeds, Cognition, Lacunae, Stroke