Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2024 Apr 8;45(4):386-392.
doi: 10.3174/ajnr.A8212.

Increased Intracranial Arterial Pulsatility and Microvascular Brain Damage in Pseudoxanthoma Elasticum

J W Bartstra  1 , T van den Beukel  2 , G Kranenburg  3 , L J Geurts  2 , A M den Harder  2 , T Witkamp  2 , J M Wolterink  4 , J J M Zwanenburg  2 , E van Valen  4   5 , H L Koek  4   5 , W P T M Mali  2 , P A de Jong  2 , J Hendrikse  2 , W Spiering  3
Affiliations

Increased Intracranial Arterial Pulsatility and Microvascular Brain Damage in Pseudoxanthoma Elasticum

J W Bartstra et al. AJNR Am J Neuroradiol. .

Abstract

Background and purpose: Carotid siphon calcification might contribute to the high prevalence of cerebrovascular disease in pseudoxanthoma elasticum through increased arterial flow pulsatility. This study aimed to compare intracranial artery flow pulsatility, brain volumes, and small-vessel disease markers between patients with pseudoxanthoma elasticum and controls and the association between arterial calcification and pulsatility in pseudoxanthoma elasticum.

Materials and methods: Fifty patients with pseudoxanthoma elasticum and 40 age- and sex-matched controls underwent 3T MR imaging, including 2D phase-contrast acquisitions for flow pulsatility in the assessment of ICA and MCA and FLAIR acquisitions for brain volumes, white matter lesions, and infarctions. All patients with pseudoxanthoma elasticum underwent CT scanning to measure siphon calcification. Flow pulsatility (2D phase-contrast), brain volumes, white matter lesions, and infarctions (3D T1 and 3D T2 FLAIR) were compared between patients and controls. The association between siphon calcification and pulsatility in pseudoxanthoma elasticum was tested with linear regression models.

Results: Patients with pseudoxanthoma elasticum (mean age, 57 [SD, 12] years; 24 men) had significantly higher pulsatility indexes (1.05; range, 0.94-1.21 versus 0.94; range, 0.82-1.04; P = .02), lower mean GM volumes (597 [SD, 53] mL versus 632 [SD, 53] mL; P < .01), more white matter lesions (2.6; range, 0.5-7.5 versus 1.1; range, 0.5-2.4) mL; P = .05), and more lacunar infarctions (64 versus 8, P = .04) than controls (mean age, 58 [SD, 11] years; 20 men). Carotid siphon calcification was associated with higher pulsatility indexes in patients with pseudoxanthoma elasticum (β = 0.10; 95% CI, 0.01-0.18).

Conclusions: Patients with pseudoxanthoma elasticum have increased intracranial artery flow pulsatility and measures of small-vessel disease. Carotid siphon calcification might underlie the high prevalence of cerebrovascular disease in pseudoxanthoma elasticum.

PubMed Disclaimer

Figures

FIG 1.
FIG 1.
Clinical example of vascular and brain disease in PXE. Carotid siphon calcification on CT (A, red circles) and small-vessel disease on 3T MR imaging (B). The blue arrow shows a lacunar infarction; the orange arrows show WML.
FIG 2.
FIG 2.
Correlation between carotid siphon calcification and the PI in patients with PXE. Carotid siphon calcification was correlated with the intracranial PI (R = 0.51, P < .01, regression line; 95% CI, gray band). This association remained significant after multivariate adjustment for age and sex (β = 0.10; 95% CI, 0.02–0.18*) and age, sex, and hypertension (β = 0.10; 95% CI, 0.01–0.18*). Carotid siphon calcification was analyzed as 10log (1+carotid siphon calcification). P < .05 was statistically significant. A single asterisk indicates P < .05.
FIG 3.
FIG 3.
Arterial calcification, pulsatility, and microvascular brain damage in PXE. Carotid siphon calcification as a measure of intracranial arterial calcification is associated with increased flow pulsatility in PXE. The subsequent pulse pressure–induced microvascular damage, including lacunar infarctions and WML, might contribute to cognitive decline.
See this image and copyright information in PMC

Similar articles

See all similar articles

Cited by

References

    1. Plomp AS, Toonstra J, Bergen AA, et al. . Proposal for updating the pseudoxanthoma elasticum classification system and a review of the clinical findings. Am J Med Genet A 2010;152A:1049–58 10.1002/ajmg.a.33329 - DOI - PubMed
    1. Kranenburg G, de Jong PA, Mali WP, et al. . Prevalence and severity of arterial calcifications in pseudoxanthoma elasticum (PXE) compared to hospital controls: novel insights into the vascular phenotype of PXE. Atherosclerosis 2017;256:7–14 10.1016/j.atherosclerosis.2016.11.012 - DOI - PubMed
    1. Risseeuw S, Ossewaarde-van Norel J, van Buchem C, et al. . The extent of angioid streaks correlates with macular degeneration in pseudoxanthoma elasticum. Am J Ophthalmol 2020;220:82–90 10.1016/j.ajo.2020.07.022 - DOI - PubMed
    1. Leftheriotis G, Kauffenstein G, Hamel JF, et al. . The contribution of arterial calcification to peripheral arterial disease in pseudoxanthoma elasticum. PLoS One 2014;9:e96003 10.1371/journal.pone.0096003 - DOI - PMC - PubMed
    1. Kauw F, Kranenburg G, Kappelle LJ, et al. . Cerebral disease in a nationwide Dutch pseudoxanthoma elasticum cohort with a systematic review of the literature. J Neurol Sci 2017;373:167–72 10.1016/j.jns.2016.12.053 - DOI - PubMed

LinkOut - more resources