A kindred affected by cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) |
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| Authors: | L. Trojano Michele Ragno Antonio Manca Giuseppe Caruso |
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| Affiliation: | (1) Fondazione Maugeri, Centro Medico di Telese, Loc. S. Stefano in Lanterria, I-82037 Telese Terme (BN), Italy Tel.: +39–824–909111 Fax: +39–824–909614, IT;(2) Division of Neurology, “C. & G. Mazzoni” Hospital, Ascoli Piceno, Italy, IT;(3) Department of Radiology, National Institute for Research and Treatment of Aging, I.N.R.C.A., Ancona, Italy, IT;(4) Department of Neurological Sciences, “Federico II” University, Naples, Italy, IT |
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| Abstract: | We report a 2-year prospective neuropsychological study of five asymptomatic subjects with magnetic resonance imaging (MRI) abnormalities from an Italian kindred affected by cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). These subjects completed tests for attention capacities, processing speed, abstract thinking, short-term memory, learning and constructional praxis. Seven normal subjects matched for age and education, belonging to the same pedigree and not having MRI hyperintensities were examined as controls. The results did not show significant differences between asymptomatic subjects and normal controls. Cognitive performance of asymptomatic subjects did not deteriorate during a 2-year follow-up. Our findings suggest that, at this stage of the disease process, the presence of diffuse leukoencephalopathy does not imply subtle cognitive defects. Received: 1 April 1997 Received in revised form: 10 November 1997 Accepted: 18 November 1997 |
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| Keywords: | Vascular dementia Dominant inheritance Cognition Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) Magnetic resonance imaging |
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