Arterial internal elastic lamina calcifications in pseudoxanthoma elasticum (PXE) may reduce the normal attenuation by the carotid siphon. This could contribute to increased intracranial pulsatility seen in PXE patients. We compared velocity pulsatility and distensibility along the internal carotid artery and in the middle cerebral artery between 50 PXE patients and 40 age and sex-matched controls, using 2D phase-contrast velocity mapping on 3T MRI. Distensibility was lower and pulsatility higher in patients. However, pulsatility attenuation over the siphon was similar between patients and controls. Siphon dysfunction does, therefore, not explain the increased intracranial arterial pulsatility in PXE.
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