Abstract
Here we describe clinical, neuropsychological and neuroradiological findings in 6 subjects belonging to two unrelated Italian cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) kindreds from the same geographic area who shared a common Arg1006Cys mutation. Subjects from Family A were virtually asymptomatic, and yet showed MRI pathological findings and a cluster of sub-clinical neuropsychological defects mainly centred on the visuospatial domain; patients from Family B had presented several clinically relevant episodes and showed a general cognitive impairment compatible with the clinical picture of vascular dementia. The present clinical observations are consistent with the hypothesis of a geographical clustering for CADASIL, and highlight that sub-clinical cognitive impairment may help to identify this syndrome in families presenting with only migraine.
Original language | English |
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Pages (from-to) | 252-256 |
Number of pages | 5 |
Journal | Neurological Sciences |
Volume | 27 |
Issue number | 4 |
DOIs | |
Publication status | Published - Sep 2006 |
Keywords
- CADASIL
- Cognitive impairment
- Genetics
- Neuropsychology
- Vascular dementia
ASJC Scopus subject areas
- Neuroscience(all)
- Clinical Neurology