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Article

Long-term prognosis and causes of death in CADASIL: a retrospective study in 411 patients.

Brain : a journal of neurology - 1 Nov 2004

Opherk Christian, Peters Nils, Herzog Jürgen, Luedtke Rainer, Dichgans Martin

Abstract excerpt

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a hereditary angiopathy caused by mutations in the NOTCH3 gene. The clinical course is highly variable. Little is known about the long-term prognosis and the causes of death in CADASIL patients. Likewise, the impact of gender and NOTCH3 genotype on disease progression remains largely unexplored. We identified...

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Identifiers

PMID: 15364702
DOI: 10.1093/brain/awh282

Genes

NOTCH3

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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Long-term prognosis and causes of death in CADASIL: a retrospective study in 411 patients.PMID 15364702Read ↗

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Short-term frequently relapsing ischemic strokes followed by rapidly progressive dementia in CADASIL: a case report and literature reviewSemantic proximity Read ↗

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Changing clinical patterns and increasing prevalence in CADASIL.Semantic proximity Read ↗

The phenotypic spectrum of CADASIL: clinical findings in 102 cases.Semantic proximity Read ↗

Genetic and nongenetic factors associated with CADASIL: A retrospective cohort study.Semantic proximity Read ↗

Acute simultaneous multiple lacunar infarcts as the initial presentation of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy.Semantic proximity Read ↗

Effect of NOTCH3 EGFr Group, Sex, and Cardiovascular Risk Factors on CADASIL Clinical and Neuroimaging Outcomes.Semantic proximity Read ↗

Diversity of stroke presentation in CADASIL: study from patients harboring the predominant NOTCH3 mutation R544C.Semantic proximity Read ↗

Comparison of models for stroke-free survival prediction in patients with CADASIL.Semantic proximity Read ↗

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