ArticleAJNR. American journal of neuroradiology2017
Basal Ganglia T1 Hyperintensity in Hereditary Hemorrhagic Telangiectasia.
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 5 papers.
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Who cites it
5 citing papers in PubMed, 9 citations in OpenAlex.
- Minimal encephalopathy in hereditary hemorrhagic telangiectasia patients with portosystemic vascular malformations.Orphanet journal of rare diseases · 2024Observational
- Hereditary haemorrhagic telangiectasias with recurrent ischemic stroke hinted by manganese deposition in the basal ganglia: a case report and literature review.BMC neurology · 2024Review
- T1 relaxation: Chemo-physical fundamentals of magnetic resonance imaging and clinical applications.Insights into imaging · 2024Review
- Neuroimaging Pearls from the MDS Congress Video Challenge. Part 1: Genetic Disorders.Movement disorders clinical practice · 2022Review
- Brain Magnetic Resonance Imaging Abnormalities in Patients with Hereditary Hemorrhagic Telangiectasia.Internal medicine (Tokyo, Japan) · 2021Article
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Authors and funding
6 authors at 3 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
background and purposeThe implications of basal ganglia T1 hyperintensity remain unclear in patients with hereditary hemorrhagic telangiectasia. This study was performed to assess the prevalence of this imaging finding in a large cohort of patients with hereditary hemorrhagic telangiectasia and to identify any association between this phenomenon and other disease manifestations. MATERIALS AND
methodsIn this retrospective study, we identified all patients at our institution diagnosed with definite hereditary hemorrhagic telangiectasia from 2001 to 2017. Patients who did not undergo brain MR imaging were excluded. Patient demographics, laboratory results, and hereditary hemorrhagic telangiectasia disease characteristics were noted. Basal ganglia hyperintensity was evaluated both qualitatively and quantitatively relative to the signal intensity in the ipsilateral thalami. Statistical analysis was performed with commercially available software.
resultsA total of 312 patients (41% men, 59% women; mean age, 51 ± 18 years) with definite hereditary hemorrhagic telangiectasia were identified. Basal ganglia T1 hyperintensity was present in 23.4% of patients and demonstrated a statistically significant association with older age (
conclusionsBasal ganglia T1 hyperintensity occurs in >23% of patients with hereditary hemorrhagic telangiectasia and is associated with hepatic vascular malformations, hepatic dysfunction, and elevated cardiac output. The presence of this finding on screening MR imaging in patients with hereditary hemorrhagic telangiectasia should prompt further evaluation for visceral lesions causing arteriovenous shunting.
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