ArticlePloS one2014
Increased arterial diameters in the posterior cerebral circulation in men with Fabry disease.
Article in PloS one, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 21 papers.
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Who cites it
21 citing papers in PubMed, 38 citations in OpenAlex.
- Automated quantification of skin Gb3 load and white matter lesion assessment in Fabry disease.Orphanet journal of rare diseases · 2026Article
- Stroke in Fabry Disease: Identification of Risk Factors for Stroke in a Large Single-Centre Cohort.European journal of neurology · 2025Article
- Prevalence and Clinical Correlates of Cerebrovascular Alterations in Fabry Disease: A Cross-Sectional Study.Brain sciences · 2025Article
- Perivascular spaces and basilar artery remodeling in Fabry disease-a dual vascular pathology.Frontiers in neurology · 2025Article
- Clinical and Pathophysiologic Correlates of Basilar Artery Measurements in Fabry Disease.AJNR. American journal of neuroradiology · 2024Article
- Variability in Intracranial Vessel Diameters and Considerations for Neurovascular Models: A Systematic Review and Meta-Analysis.Stroke (Hoboken, N.J.) · 2024Article
- Review
- Basilar artery diameter as neuroimaging biomarker in Chinese Fabry disease patients.Orphanet journal of rare diseases · 2023Article
- Pulvinar Sign, Stroke and Their Relationship with Fabry Disease: A Systematic Review and Metanalysis.Neurology international · 2022Review
- Characteristics of Neurological Symptoms in Adult Japanese Patients with Fabry Disease.Internal medicine (Tokyo, Japan) · 2021Article
- Factors Associated With the Dilation of Perivascular Space in Healthy Elderly Subjects.Frontiers in aging neuroscience · 2021Article
- Evaluation of Cerebral Microvascular Regulatory Mechanisms with Transcranial Doppler in Fabry Disease.Brain sciences · 2020Article
- Neuroimaging in Fabry disease: current knowledge and future directions.Insights into imaging · 2018Review
- Redefining the Pulvinar Sign in Fabry Disease.AJNR. American journal of neuroradiology · 2017Article
- Biomarkers and Imaging Findings of Anderson-Fabry Disease-What We Know Now.Diseases (Basel, Switzerland) · 2017Review
- Basilar Artery Changes in Fabry Disease.AJNR. American journal of neuroradiology · 2017Article
- Fabry Disease: Recognition, Diagnosis, and Treatment of Neurological Features.Current treatment options in neurology · 2016Review
- Brain MR Imaging Findings of Cardiac-Type Fabry Disease with an IVS4+919G>A Mutation.AJNR. American journal of neuroradiology · 2016Article
- Article
- Eight-Year Follow-Up of Neuropsychiatric Symptoms and Brain Structural Changes in Fabry Disease.PloS one · 2015Article
Corrections and comments
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Authors and funding
8 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
A high load of white matter lesions and enlarged basilar arteries have been shown in selected patients with Fabry disease, a disorder associated with an increased stroke risk. We studied a large cohort of patients with Fabry disease to differentially investigate white matter lesion load and cerebral artery diameters. We retrospectively analyzed cranial magnetic resonance imaging scans of 87 consecutive Fabry patients, 20 patients with ischemic stroke, and 36 controls. We determined the white matter lesion load applying the Fazekas score on fluid-attenuated inversion recovery sequences and measured the diameters of cerebral arteries on 3D-reconstructions of the time-of-flight-MR-angiography scans. Data of different Fabry patient subgroups (males-females; normal-impaired renal function) were compared with data of patients with stroke and controls. A history of stroke or transient ischemic attacks was present in 4/30 males (13%) and 5/57 (9%) females with Fabry disease, all in the anterior circulation. Only one man with Fabry disease showed confluent cerebral white matter lesions in the Fazekas score assessment (1%). Male Fabry patients had a larger basilar artery (p<0.01) and posterior cerebral artery diameter (p<0.05) compared to male controls. This was independent of disease severity as measured by renal function and did not lead to changes in arterial blood flow properties. A basilar artery diameter of >3.2 mm distinguished between men with Fabry disease and controls (sensitivity: 87%, specificity: 86%, p<0.001), but not from stroke patients. Enlarged arterial diameters of the posterior circulation are present only in men with Fabry disease independent of disease severity.
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