Affiliations: [a] Department of Geriatrics, Lariboisière-Fernand Widal Hospital, AP-HP, Paris Denis Diderot University, Paris, France | [b] Department of Neurology, Lariboisière-Fernand Widal Hospital, AP-HP, Paris Denis Diderot University, Paris, France | [c] INSERM 708, Pierre et Marie Curie University, Paris, France | [d] Institute for Stroke and Dementia Research, Klinikum der Universität München, Campus Grosshadern, Ludwig-Maximilians-University, Munich, Germany | [e] Peking Union Medical College Hospital, Peking, China
Correspondence:
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Correspondence to: Pr Hugues Chabriat, Neurology Department, Lariboisière Hospital, 2 rue Ambroise Paré, 75010 Paris, France. Tel.: +33149952596; Fax: +33149952595; E-mail: hugues.chabriat@lrb.aphp.fr.
Abstract: To better characterize the clinical spectrum related to white-matter hyperintensities (WMH) in small vessel disease, 66 patients with WMH but without any lacunar infarct were selected out of a cohort of 248 CADASIL individuals. Characteristics of these patients were compared to those of patients with lacunar infarcts. Relationships between the normalized volume of WMH (nWMH), presence of microhemorrhages, brain parenchymal fraction (BPF). and cognitive performances were assessed. The Trail Making Test (TMT) A and B times, Mattis Dementia Rating Scale (MDRS) total score, attention subscore, verbal fluency score and delayed memory recall were significantly correlated with nWMH but not with BPF. Presence of microhemorrhages was associated with worse TMT B time and attention MDRS subscore after adjustment for WMH. All subjects had Mini-Mental Status Examination scores ≥24 and presented with no or only mild disability. These results suggest that CADASIL patients with isolated WMH can present with executive and attention deficit but not with severe disability and that additional lesions are needed to cause significant disability and/or dementia.
Keywords: CADASIL, cerebral small vessel disease, cognitive impairment, dementia, MRI, white matter hyperintensities
