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Notch3 mutations in CADASIL, a hereditary adult-onset condition causing stroke and dementia
STROKE is the third leading cause of death, and vascular dementia the second cause of dementia after Alzheimer's disease. CADASIL (for cerebral autosomal dominant arteriopathy with subcorticalExpand
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Early decompressive surgery in malignant infarction of the middle cerebral artery: a pooled analysis of three randomised controlled trials
BACKGROUND Malignant infarction of the middle cerebral artery (MCA) is associated with an 80% mortality rate. Non-randomised studies have suggested that decompressive surgery reduces this mortalityExpand
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The clinical spectrum of familial hemiplegic migraine associated with mutations in a neuronal calcium channel.
BACKGROUND Familial hemiplegic migraine, an autosomal dominant disorder characterized by attacks of transient hemiparesis followed by a migraine headache, is classically divided into pure familialExpand
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Sequential-Design, Multicenter, Randomized, Controlled Trial of Early Decompressive Craniectomy in Malignant Middle Cerebral Artery Infarction (DECIMAL Trial)
Background and Purpose— There is no effective medical treatment of malignant middle cerebral artery (MCA) infarction. The purpose of this clinical trial was to assess the efficacy of earlyExpand
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Strong clustering and stereotyped nature of Notch3 mutations in CADASIL patients
BACKGROUND CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy) is commonly overlooked or misdiagnosed owing to its recent identification and itsExpand
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Clinical spectrum of CADASIL: a study of 7 families
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an inherited arterial disease of the brain recently mapped to chromosome 19. We studied 148Expand
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Mapping of a second locus for familial hemiplegic migraine to 1q21–q23 and evidence of further heterogeneity
Familial hemiplegic migraine (FHM) is an autosomal dominant variety of migraine with aura. We previously mapped an FHM gene on the short arm of chromosome 19. Mutations in this gene, recently shownExpand
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Role of COL4A1 in small-vessel disease and hemorrhagic stroke.
Small-vessel diseases of the brain underlie 20 to 30 percent of ischemic strokes and a larger proportion of intracerebral hemorrhages. In this report, we show that a mutation in the mouse Col4a1Expand
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Patterns of MRI lesions in CADASIL
Objective: To investigate the location and severity of MRI signal abnormalities in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). Background:Expand
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Cerebral hemodynamics in CADASIL before and after acetazolamide challenge assessed with MRI bolus tracking.
BACKGROUND White matter lesions in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) are underlaid by severe ultrastructural changes of theExpand
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