HEREDITARY CEREBRAL-HEMORRHAGE WITH AMYLOIDOSIS-DUTCH TYPE - BETTER CORRELATION OF COGNITIVE DETERIORATION WITH ADVANCING AGE THAN WITH NUMBER OF FOCAL LESIONS OR WHITE-MATTER HYPERINTENSITIES

The relationship between cognitive deterioration and abnormalities det ected by magnetic resonance imaging (MRI) was investigated to determin e the radiological correlates of cognitive deterioration in hereditary cerebral hemorrhage with amyloidosis-Dutch type (HCHWA-D). Twenty HCH WA-D subjects (12 patients who had suffered one or more strokes and ei ght who had not suffered a stroke) were studied with MRI and underwent extensive neuropsychological examination. On MRI the number of focal lesions was counted, and white matter hyperintensities (WMHs) were sco red semiquantitatively. A significant correlation between cognitive de terioration and WMH score and number of focal lesions was found. Howev er, cognitive deterioration, WMH score, and the number of focal lesion s all increase with age, and therefore their mutual correlation can be explained as an age effect. This study shows that cognitive deteriora tion in HCHWA-D is not correlated with abnormalities detected by MRI ( number of focal lesions and subcortical WMHs) independently of age. Al though a contribution of white matter changes and/or focal lesions, po ssibly in combination with age, to cognitive deterioration cannot be e xcluded. Cognitive deterioration in these HCHWA-D patients is probably primarily the result of chronic damage of amyloid angiopathy to the b rain, to which may be superimposed cognitive impairment from focal cer ebral hemorrhage or infarction.