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    ABSTRACT: 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.
    European Journal of Neurology 07/2013; · 3.85 Impact Factor
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    ABSTRACT: Multiparametric quantitative blood oxygenation level dependent (mqBOLD) magnetic resonance Imaging (MRI) approach allows mapping tissular oxygen saturation (StO2) and cerebral metabolic rate of oxygen (CMRO2). To identify hemodynamic alteration related to severe intracranial arterial stenosis (SIAS), functional MRI of cerebrovascular reserve (CVR BOLD fMRI) to hypercapnia has been proposed. Diffusion imaging suggests chronic low grade ischemia in patients with impaired CVR. The aim of the present study was to evaluate how oxygen parameters (StO2 and CMRO2), assessed with mqBOLD approach, correlate with CVR in patients (n = 12) with SIAS and without arterial occlusion. The perfusion (dynamic susceptibility contrast), oxygenation, and CVR were compared. The MRI protocol conducted at 3T lasted approximately 1 h. Regions of interest measures on maps were delineated on segmented gray matter (GM) of middle cerebral artery territories. We have shown that decreased CVR is spatially associated with decreased CMRO2 in GM of patients with SIAS. Further, the degree of ipsilateral CVR reduction was well-correlated with the amplitude of the CMRO2 deficit. The altered CMRO2 suggests the presence of a moderate ischemia explained by both a decrease in perfusion and in CVR. CVR and mqBOLD method may be helpful in the selection of patients with SIAS to advocate for medical therapy or percutaneous transluminal angioplasty-stenting. Hum Brain Mapp, 2014. © 2014 Wiley Periodicals, Inc.
    Human Brain Mapping 10/2014; · 6.92 Impact Factor
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    ABSTRACT: Vascular dementia (VaD) is the most prevalent form of secondary dementia and the second most common of all dementias. The present paper aims to define guidelines on the basic principles for treating patients with suspected VaD (and vascular cognitive impairment – no dementia) using an evidence-based, systematized approach. The knowledge used to define these guidelines was retrieved from searches of several databases (Medline, Scielo, Lilacs) containing scientific articles, systematic reviews, meta-analyses, largely published within the last 15 years or earlier when pertinent. Information retrieved and selected for relevance was used to analyze diagnostic criteria and to propose a diagnostic system encompassing diagnostic criteria, anamnesis, as well as supplementary and clinical exams (neuroimaging and laboratory). Wherever possible, instruments were selected that had versions previously adapted and validated for use in Brazil that take into account both schooling and age. This task led to proposed protocols for supplementary exams based on degree of priority, for application in clinical practice and research settings. Demência vascular: avaliação cognitiva, funcional e comportamental. Recomendações do Departamento Científico de Neurologia Cognitiva e do Envelhecimento da Academia Brasileira de Neurologia. Parte I. Resumo – A demência vascular (DV) é a forma mais prevalente de demência secundária e asegunda forma mais comum. O presente artigo visa definir recomendações dos princípios básicos para tratamento dos pacientes com suspeita de DV (e comprometimento cognitivo leve sem demência) usando uma abordagem sistematizada baseada em evidências. O conhecimento usado para definir estas recomendações foi recuperado de pesquisa de várias bases de dados (Medline, Scielo, Lilacs) contendo artigos científicos, revisões sistemáticas, meta-análises, publicados nos últimos 15 anos, ou antes, se pertinente. As informações recuperadas e selecionadas pela relevância foram usadas para analisar os critérios diagnósticos e propor um sistema diagnóstico incluindo critérios diagnósticos, anamnese, bem como exames complementares (neuroimagem e laboratório). Sempre que possível, os instrumentos foram selecionados com versões previamente adaptadas e validadas para uso no Brasil, segundo escolaridade e idade. Os protocolos propostos para exames complementares basearam-se no grau de prioridade, para aplicação na prática clínica ou em pesquisa. Palavras-chave: recomendações, demência vascular, critérios, neuroimagem, exames laboratoriais.
    Dementia e Neuropsychologia 01/2011; 5:251-263.