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Basilar Artery Changes in Fabry Disease.
R Manara1, R Y Carlier2, S Righetto3
1From the Sezione di Neuroscienze (R.M.), University of Salerno, Salerno, Italy.
AJNR. American Journal of Neuroradiology
|January 28, 2017
Summary
Fabry disease causes significant basilar artery remodeling, including increased diameter and length, particularly in males. This remodeling varies with age and sex, offering insights into Fabry disease
Area of Science:
- Neurology
- Vascular Biology
- Genetics
Background:
- Fabry disease is associated with basilar artery dolichoectasia.
- Previous studies have limited data on the prevalence, severity, and progression of basilar artery changes in Fabry disease.
Purpose of the Study:
- To quantitatively assess basilar artery parameters in a large cohort of Fabry disease patients using MRA.
- To compare these parameters between Fabry disease patients and controls.
Main Methods:
- Retrospective analysis of 1.5T MRA scans from 110 Fabry disease patients and 108 controls.
- Measurement of basilar artery mean diameter, curved length, linear length, and tortuosity index.
Main Results:
- Fabry disease patients exhibited increased basilar artery mean diameter and linear length compared to controls.
- Basilar artery dimensions showed age-dependent changes, with significant differences observed in male Fabry disease patients.
- Male Fabry disease patients had more pronounced increases in diameter, curved length, and tortuosity compared to females and male controls.
Conclusions:
- The basilar artery undergoes significant remodeling in Fabry disease, influenced by age and sex.
- These findings provide valuable insights into the pathophysiology of cerebral vasculature in Fabry disease.
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