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Medline ® Abstract for Reference 96

of 'Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL)'

Predictors of Clinical Worsening in Cerebral Autosomal Dominant Arteriopathy With Subcortical Infarcts and Leukoencephalopathy: Prospective Cohort Study.
Chabriat H, HervéD, Duering M, Godin O, Jouvent E, Opherk C, Alili N, Reyes S, Jabouley A, Zieren N, Guichard JP, Pachai C, Vicaut E, Dichgans M
Stroke. 2016 Jan;47(1):4-11. Epub 2015 Nov 17.
BACKGROUND AND PURPOSE: Predictors of clinical worsening in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy remain unknown. This study aims to identify demographic, clinical, and magnetic resonance imaging predictors of incident strokes, incident dementia, clinical deterioration, and death in patients with this genetically proven disease.
METHODS: Two hundred ninety subjects (mean age, 50.6±11.4 years) were assessed at baseline and followed up for 36 months. Incident clinical events were recorded, and clinical scores included the Mini Mental State Examination, Mattis Dementia Rating Scale, modified Rankin Scale, and Barthel index. The number of lacunes and microbleeds, the volume of white-matter hyperintensities, and brain parenchymal fraction were assessed on baseline magnetic resonance imaging. Data were analyzed by ANCOVA, multivariable logistic regression, and Cox proportional hazard models.
RESULTS: Incident stroke occurred in 55 of 278 patients (19.8%). Moderate or severe disability developed in 19 of 210 (9%) nondisabled individuals, incident dementia in 49 of 231 (20%) nondemented subjects, and 4.8% of patients died. Active smoking, the number of lacunes, and brain parenchymal fraction independently predicted incident stroke during follow-up. Gait disturbance, dementia, and brain parenchymal fraction predicted progression toward moderate or severe disability. Active smoking, disability, and brain parenchymal fraction predicted incident dementia. Age was the only significant predictor of death.
CONCLUSIONS: Clinical assessment and brain magnetic resonance imaging aid in predicting incident clinical events and clinical deterioration in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy. There is a bidirectional relationship between dementia and moderate or severe disability in predicting each other's onset. Active smoking is a modifiable risk factor associated with clinical progression in Notch3 mutation carriers.
From the Department of Neurology, GH Saint-Louis-Lariboisière, Assistance Publique des Hôpitaux de Paris (APHP), UniversitéParis Denis Diderot and DHU NeuroVasc Sorbonne Paris-Cité, Paris, France (H.C., D.H, O.G., E.J., N.A., S.R., A.J.); INSERM UMR 1161, Paris, France (H.C., D.H., E.J.); Institute for Stroke and Dementia Research, Klinikum der Universität München, Ludwig-Maximilians-University, Munich, Germany (M.D., C.O., N.Z., M.D.); Department of Neuroradiology, CHU Lariboisière, Assistance Publique des Hôpitaux de Paris, Paris, France (J.-P.G.); Bioclinica Inc, Lyon, France (C.P.); Unitéde Recherche Clinique, GH Saint Louis-Lariboisière, Assistance Publique des Hôpitaux de Paris, UniversitéParis Denis Diderot, Paris, France (E.V.); and Munich Cluster for Systems Neurology (SyNergy), Munich, Germany (M.D.). hugues.chabriat@lrb.aphp.fr.