Predictors of Clinical Worsening in Cerebral Autosomal Dominant Arteriopathy With Subcortical Infarcts and Leukoencephalopathy

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  • Hugues Chabriat
    From the Department of Neurology, GH Saint-Louis-Lariboisière, Assistance Publique des Hôpitaux de Paris (APHP), Université Paris 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...
  • Dominique Hervé
    From the Department of Neurology, GH Saint-Louis-Lariboisière, Assistance Publique des Hôpitaux de Paris (APHP), Université Paris 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...
  • Marco Duering
    From the Department of Neurology, GH Saint-Louis-Lariboisière, Assistance Publique des Hôpitaux de Paris (APHP), Université Paris 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...
  • Ophelia Godin
    From the Department of Neurology, GH Saint-Louis-Lariboisière, Assistance Publique des Hôpitaux de Paris (APHP), Université Paris 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...
  • Eric Jouvent
    From the Department of Neurology, GH Saint-Louis-Lariboisière, Assistance Publique des Hôpitaux de Paris (APHP), Université Paris 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...
  • Christian Opherk
    From the Department of Neurology, GH Saint-Louis-Lariboisière, Assistance Publique des Hôpitaux de Paris (APHP), Université Paris 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...
  • Nassira Alili
    From the Department of Neurology, GH Saint-Louis-Lariboisière, Assistance Publique des Hôpitaux de Paris (APHP), Université Paris 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...
  • Sonia Reyes
    From the Department of Neurology, GH Saint-Louis-Lariboisière, Assistance Publique des Hôpitaux de Paris (APHP), Université Paris 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...
  • Aude Jabouley
    From the Department of Neurology, GH Saint-Louis-Lariboisière, Assistance Publique des Hôpitaux de Paris (APHP), Université Paris 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...
  • Nikola Zieren
    From the Department of Neurology, GH Saint-Louis-Lariboisière, Assistance Publique des Hôpitaux de Paris (APHP), Université Paris 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...
  • Jean-Pierre Guichard
    From the Department of Neurology, GH Saint-Louis-Lariboisière, Assistance Publique des Hôpitaux de Paris (APHP), Université Paris 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...
  • Chahin Pachai
    From the Department of Neurology, GH Saint-Louis-Lariboisière, Assistance Publique des Hôpitaux de Paris (APHP), Université Paris 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...
  • Eric Vicaut
    From the Department of Neurology, GH Saint-Louis-Lariboisière, Assistance Publique des Hôpitaux de Paris (APHP), Université Paris 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...
  • Martin Dichgans
    From the Department of Neurology, GH Saint-Louis-Lariboisière, Assistance Publique des Hôpitaux de Paris (APHP), Université Paris 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...

Bibliographic Information

Other Title
  • Prospective Cohort Study

Description

<jats:sec> <jats:title>Background and Purpose—</jats:title> <jats:p>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.</jats:p> </jats:sec> <jats:sec> <jats:title>Methods—</jats:title> <jats:p>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.</jats:p> </jats:sec> <jats:sec> <jats:title>Results—</jats:title> <jats:p>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.</jats:p> </jats:sec> <jats:sec> <jats:title>Conclusions—</jats:title> <jats:p>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.</jats:p> </jats:sec>

Journal

  • Stroke

    Stroke 47 (1), 4-11, 2016-01

    Ovid Technologies (Wolters Kluwer Health)

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