Cerebral Amyloid Angiopathy Presenting with Progressive Dementia

J Korean Neurol Assoc > Volume 16(5); 1998 > Article

Journal of the Korean Neurological Association 1998;16(5): 718-723.

치매로 발병한 뇌 아밀로이드 혈관병증

김용범, 나덕렬·서연림*·이수주·이광호·김종수**

성균관대학교 의과대학 삼성서울병원 신경과,신경외과* 진단병리과**

Cerebral Amyloid Angiopathy Presenting with Progressive Dementia

Yong Bum Kim, M.D., Duk L. Na, M.D., Yeon Lim Seo, M.D.*, Soo Joo Lee, M.D., Kwang Ho Lee, M.D., Jong Soo Kim, M.D.**

Department of Neurology, Neurosurgery** and Diagnostic Pathology* Samsung Medical Center, College of Medicine, Sung Kyun Kwan University

Abstract

Cerebral amyloid angiopathy (CAA) can present with lobar hemorrhage, progressive dementia, or transient neurologic symptoms. To date, Pathology-confirmed CAA which presented with progressive dementia has rarely been repored in Korea. A 79-year-old normotensive man presented with progressive dementia for one year. Neurologic examination was remarkable for hyperreflexia and bilateral grasp response. Mild parkinsonian feature was also present. Cognitive domains impaired on neuropsychological test included verbal and nonverbal memory, language, visuospatial function and frontal/executive function. Brain MRI, especially gradiant-echo imaging, showed multiple microhemorrhages in the cortical areas along with leukoaraosis. Six months after initial evaluation, he developed massive ICH involving right frontal lobe with midline shift. Hematoma removal as well as cortical biopsy was performed. Cortical and meningeal vessels were thickened with amorphous materials showing apple-green birefringence, a feature consistent with CAA. CAA should be suspected in patient with subacute dementia when their MRI shows cortical microhemorrhage and leukoaraiosis. Key words : dementia, amyloid angiopathy, hemorrhage