CADASIL - A NEW MODEL OF SUBCORTICAL DEME NTIA

We report 3 cases of Cadasil with dementia. In the 3 cases, the dement ial had a subcortical and frontal presentation. It associated behaviou ral symptoms, amnesia, executive functions disturbances, bradyphrenia, slowing of information processing and frontal symptoms, without aphas ia, apraxia or agnosia. One patient showed overt demential before any focal neurological sign. Imaging of the brain was consistent with subc ortical infarcts and leukoencephalopathy, without involvement of the c erebral cortex. We suggest that demential and psycohaffective disturba nces are major diagnostic criteria for Cadosil. This pathology is prob ably a good model for the neuropsychological study and the physiopatho logical analysis of the concept of subcortical dementia.