Influence of vascular risk factors and neuropsychological profile on functional performances in CADASIL: results from the MIcrovascular LEukoencephalopathy Study (MILES)
Article first published online: 19 JUL 2013
© 2013 The Author(s) European Journal of Neurology © 2013 EFNS
European Journal of Neurology
Volume 21, Issue 1, pages 65–71, January 2014
How to Cite
Ciolli, L., Pescini, F., Salvadori, E., Del Bene, A., Pracucci, G., Poggesi, A., Nannucci, S., Valenti, R., Basile, A. M., Squarzanti, F., Bianchi, S., Dotti, M. T., Adriano, E., Balestrino, M., Federico, A., Gandolfo, C., Inzitari, D. and Pantoni, L. (2014), Influence of vascular risk factors and neuropsychological profile on functional performances in CADASIL: results from the MIcrovascular LEukoencephalopathy Study (MILES). European Journal of Neurology, 21: 65–71. doi: 10.1111/ene.12241
- Issue published online: 10 DEC 2013
- Article first published online: 19 JUL 2013
- Manuscript Accepted: 17 JUN 2013
- Manuscript Received: 16 MAR 2013
- MIUR. Grant Numbers: 2006065719, 20095JPSNA
- Fondazione Cassa di Risparmio di Pistoia e Pescia. Grant Number: 2008.0182
- CADASIL ;
- functional impairment;
- neuropsychological evaluation;
- small vessel disease
Background and purpose
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an inherited cerebral small vessel disease that may lead to disability and whose phenotype modulators are still unknown.
In the MIcrovascular LEukoencephalopathy Study (MILES), we assessed the influence of vascular risk factors and the effect of different cognitive domains (memory, psychomotor speed and executive functions) performances on functional abilities in CADASIL in comparison with age-related leukoencephalopathy (ARL).
We evaluated 51 CADASIL patients (mean age 50.3 ± 13.8 years, 47.1% males) and 68 ARL patients (70.6 ± 7.4 years, 58.8% males). Considering vascular risk factors, after adjustment for age, CADASIL patients had higher mean BMI values than ARL patients. Stroke history frequency was similar in the two groups. After adjustment for age, more CADASIL patients were disabled (impaired on ≥2 items of the Instrumental Activities of Daily Living scale) in comparison with ARL patients, and CADASIL patients had worse functional performances evaluated with the Disability Assessment for Dementia (DAD) scale. In CADASIL patients, hypertension was related to both DAD score and disability. The cognitive profile of CADASIL and ARL patients was similar, but on a stepwise linear regression analysis functional performances were mainly associated with the memory index (β = −0.418, P < 0.003) in CADASIL patients and the executive function index (β = −0.321, P = 0.028) in ARL.
This study suggests that hypertension may contribute to functional impairment in CADASIL and that memory impairment has a large influence on functional decline in contrast with that observed in a sample of subjects with ARL.