Korean J Pathol.  2003 Apr;37(2):129-133.

Cerebral Amyloid Angiopathy: A Case Report

• Kim NR
• Chung JG
• Lee SK

Abstract

Symptomatic cerebral amyloid angiopathy (CAA) is characterized by lobar cerebral hemorrhage, transient neurological symptoms, and dementia with leukoencephalopathy in the absence of hypertension or coagulopathy. Contrary to its common appearance in English literature, pathologically confirmed CAA has rarely been reported in Korea. A 77-year-old normotensive, nondemented woman was admitted for a case of incidentally found intracerebral lobar hemorrhage at the left occipito-parietal area. On neurological examination, she displayed symptoms of a drowsy mentality and Gerstmann syndrome. Light-microscopically, medium to large cortical and meningeal vessels appeared to be thickened with amorphous glassy materials showing apple-green birefringence under polarized light on Congo red stain, which is consistent with CAA. The amyloid deposits were detected in the medium-sized vessels of the leptomeninges and cerebral cortex, but not in the capillaries and small arterioles in the white matter. Electron microscopy revealed amyloid fibrils and basal lamina duplication, indicating damaged vessels. Careful cortical biopsy during evacuating hematoma for histopathologic and ultrastructural examination is essential for diagnosis of CAA in case of clinical probability because the ignored sampling procedure and sampling error of patchy, segmental distribution of affected vessels might be ascribed to rare incidence of CAA in Korea.

MeSH Terms

Aged
Amyloid
Arterioles
Basement Membrane
Biopsy
Birefringence
Capillaries
Cerebral Amyloid Angiopathy*
Cerebral Cortex
Cerebral Hemorrhage
Congo Red
Dementia
Diagnosis
Female
Gerstmann Syndrome
Hematoma
Hemorrhage
Humans
Hypertension
Incidence
Korea
Leukoencephalopathies
Microscopy, Electron
Neurologic Examination
Plaque, Amyloid
Selection Bias
Amyloid
Congo Red