Publications by authors named "H Chabriat"

Background And Objectives: Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is the most frequent small artery brain disease caused by pathogenic variants of the NOTCH3 gene. During the disease, we still do not know how the various deficits progress and develop with each other at different stages of the disease. We aim to model disease progression and identify possible progressive subgroups and the effects of different covariates on clinical worsening.

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Background: The presence of antiphospholipid antibodies (aPL) has been suggested as a potential cause of moyamoya angiopathy (MMA), but this remains uncertain. In this case-control study, we aimed to compare the prevalence of circulating aPL in patients with MMA and in non-MMA cerebrovascular controls.

Methods: For comparison, we included 95 patients with MMA from the French National Referral Centre for this condition and 182 age- and sex-matched non-MMA controls with a different cerebrovascular disease, all younger than 55 years.

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Article Synopsis
  • NOTCH3cys variants are common and linked to various small vessel diseases, including early-onset stroke and dementia, but there is no comprehensive staging system to assess their severity.
  • A cohort study created and validated a simple staging system for NOTCH3-SVD by analyzing data from several international cohorts and the UK Biobank, focusing on the impact of these variants on CVD outcomes and cognition.
  • The new system includes 9 disease stages, aiding in understanding the relationship between stages and clinical outcomes like ischemic strokes, cognitive function, and brain damage.
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Article Synopsis
  • Intravascular lymphoma is a rare B-cell lymphoma that primarily affects small blood vessels and often involves the central nervous system (CNS), making early diagnosis challenging due to non-specific symptoms.
  • A study analyzed data from 17 patients, finding that most experienced sudden neurological episodes, rapid cognitive decline, and unique MRI characteristics, alongside other clinical signs such as elevated LDH levels.
  • The research identified key features, or "red flags," that could aid in quicker diagnosis of CNS-involving intravascular lymphoma, emphasizing the importance of recognizing these indicators.
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Background: In ischemic cerebral small vessel diseases (cSVD), recurrent ischemic stroke is rare (2%-3% per year). Because acute ischemia may not always lead to stroke in cSVD due to the small size of lesions, acute stroke may not reliably reflect ischemic activity or the risk of further clinical worsening, as both incident lacunes and incidental diffusion-weighted imaging-positive lesions can occur without stroke symptoms. We aimed to evaluate the total ischemic activity by measuring the incidence of magnetic resonance imaging (MRI)-proven incident ischemia, independent of the presence of stroke symptoms in a large cohort of cSVD.

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