ArticleAJNR. American journal of neuroradiology2017
Redefining the Pulvinar Sign in Fabry Disease.
Article in AJNR. American journal of neuroradiology, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 22 papers, 1 of them a synthesis that pooled it.
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Who cites it
22 citing papers in PubMed, 1 synthesis or guideline pooled it, 39 citations in OpenAlex.
- Neuropsychiatric Symptoms and Their Association With Sex, Age, and Enzyme Replacement Therapy in Fabry Disease: A Systematic Review.Frontiers in psychiatry · 2022Pooled it
- Review
- Expanded screening for Fabry disease in patients with chronic kidney disease not on dialysis: a multicenter Italian experience.Renal failure · 2025Article
- Cryptogenic strokes and neurological symptoms of Fabry disease.Frontiers in neurology · 2025Article
- Clinical and Pathophysiologic Correlates of Basilar Artery Measurements in Fabry Disease.AJNR. American journal of neuroradiology · 2024Article
- In vivo demonstration of globotriaosylceramide brain accumulation in Fabry Disease using MR Relaxometry.Neuroradiology · 2024Article
- Review
- Clinical and neuroimaging review of monogenic cerebral small vessel disease from the prenatal to adolescent developmental stage.Japanese journal of radiology · 2024Review
- Dapaglifozin on Albuminuria in Chronic Kidney Disease Patients with FabrY Disease: The DEFY Study Design and Protocol.Journal of clinical medicine · 2023Article
- T1-hyperintensity in the pulvinar ("Pulvinar sign") in Klinefelter (47, XXY) syndrome: a case report.Acta neurologica Belgica · 2023Article
- Fabry Disease and Central Nervous System Involvement: From Big to Small, from Brain to Synapse.International journal of molecular sciences · 2023Review
- Pulvinar Sign, Stroke and Their Relationship with Fabry Disease: A Systematic Review and Metanalysis.Neurology international · 2022Review
- Neuroradiological differentiation of white matter lesions in patients with multiple sclerosis and Fabry disease.Orphanet journal of rare diseases · 2022Article
- Heritable and non-heritable uncommon causes of stroke.Journal of neurology · 2021Review
- Characteristics of Neurological Symptoms in Adult Japanese Patients with Fabry Disease.Internal medicine (Tokyo, Japan) · 2021Article
- Screening for Fabry Disease in Kidney Transplant Recipients: Experience of a Multidisciplinary Team.Biomedicines · 2020Article
- Evaluation of Cerebral Microvascular Regulatory Mechanisms with Transcranial Doppler in Fabry Disease.Brain sciences · 2020Article
- Differential Diagnosis of Pediatric Multiple Sclerosis.Children (Basel, Switzerland) · 2019Review
- Optical Coherence Tomography Angiography Findings in Fabry Disease.Journal of clinical medicine · 2019Article
- Neuroimaging in Fabry disease: current knowledge and future directions.Insights into imaging · 2018Review
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Authors and funding
18 authors at 10 institutions in 4 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
background and purposeThe pulvinar sign refers to exclusive T1WI hyperintensity of the lateral pulvinar. Long considered a common sign of Fabry disease, the pulvinar sign has been reported in many pathologic conditions. The exact incidence of the pulvinar sign has never been tested in representative cohorts of patients with Fabry disease. The aim of this study was to assess the prevalence of the pulvinar sign in Fabry disease by analyzing T1WI in a large Fabry disease cohort, determining whether relaxometry changes could be detected in this region independent of the pulvinar sign positivity. MATERIALS AND
methodsWe retrospectively analyzed brain MR imaging of 133 patients with Fabry disease recruited through specialized care clinics. A subgroup of 26 patients underwent a scan including 2 FLASH sequences for relaxometry that were compared with MRI scans of 34 healthy controls.
resultsThe pulvinar sign was detected in 4 of 133 patients with Fabry disease (3.0%). These 4 subjects were all adult men (4 of 53, 7.5% of the entire male population) with renal failure and under enzyme replacement therapy. When we tested for discrepancies between Fabry disease and healthy controls in quantitative susceptibility mapping and relaxometry maps, no significant difference emerged for any of the tested variables.
conclusionsThe pulvinar sign has a significantly lower incidence in Fabry disease than previously described. This finding, coupled with a lack of significant differences in quantitative MR imaging, allows hypothesizing that selective involvement of the pulvinar is a rare neuroradiologic sign of Fabry disease.
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