title: Small artery dementia in Japan: radiological differences between CADASIL, leukoaraiosis and Binswanger's disease.
abstract: CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy) is a hereditary small artery disease which is phenotypically similar to Binswanger's disease (BD), a nonhereditary form of small artery disease. Recent studies have indicated that lesions in the temporopolar, medial frontopolar areas and external capsule are frequently seen in Caucasian patients with CADASIL. However, it remains unclear whether magnetic resonance (MR) imaging findings are helpful in diagnosing small artery disease outside countries with Caucasian populations, since CADASIL is rare despite the high prevalence of small artery disease in Japan. We examined 58 patients with small artery disease, all of whom were devoid of major vessel occlusion or severe stenosis. These patients included 7 patients from 3 families with CADASIL, 27 nondemented patients with extensive leukoaraiosis (LA) and 24 patients with BD. On T(2)-weighted MR images, hyperintensities in the temporopolar areas were observed in all 7 patients with CADASIL, whereas these lesions were observed in only 1 subject from each of the nondemented LA and BD groups. Hyperintensities in the medial frontopolar areas were seen in 4 of the 7 patients with CADASIL (57%) and in 14 of the 24 patients with BD (58%), and were more frequent than in the nondemented LA group (4 of the 27 patients; 15%). In contrast, hyperintensities in the external capsule were frequently observed in all groups. Therefore, temporopolar lesions can also serve as diagnostic markers for CADASIL in non-Caucasian patients.
authors.affiliation: Department of Neurology, Graduate School of Medicine, Kyoto University, Sakyo, Japan. tomimoto@kuhp.kyoto-u.ac.jp
authors.forename: Hidekazu
authors.initials: H
authors.lastname: Tomimoto
authors.forename: Ryo
authors.initials: R
authors.lastname: Ohtani
authors.forename: Hideaki
authors.initials: H
authors.lastname: Wakita
authors.forename: Jin-Xi
authors.initials: JX
authors.lastname: Lin
authors.forename: Masafumi
authors.initials: M
authors.lastname: Ihara
authors.forename: Yukio
authors.initials: Y
authors.lastname: Miki
authors.forename: Fumiko
authors.initials: F
authors.lastname: Oshima
authors.forename: Takaho
authors.initials: T
authors.lastname: Murata
authors.forename: Kenichi
authors.initials: K
authors.lastname: Ishibashi
authors.forename: Toshihiko
authors.initials: T
authors.lastname: Suenaga
authors.forename: Toshiki
authors.initials: T
authors.lastname: Mizuno
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D044466:Asian People / Q000235:genetics*; D001921:Brain / Q000473:pathology*; D001933:Brain Stem / Q000473:pathology; D046589:CADASIL / Q000000981:diagnostic imaging* / Q000235:genetics; D003431:Cross-Cultural Comparison; D015140:Dementia, Vascular / Q000175:diagnosis* / Q000235:genetics; D003937:Diagnosis, Differential; D005260:Female; D005625:Frontal Lobe / Q000473:pathology; D006801:Humans; D007091:Image Processing, Computer-Assisted; D049292:Leukoaraiosis / Q000175:diagnosis* / Q000235:genetics; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009434:Neural Pathways / Q000473:pathology; D010375:Pedigree; D011859:Radiography; D012680:Sensitivity and Specificity; D013702:Temporal Lobe / Q000473:pathology; D044465:White People / Q000235:genetics*