From the Department of Radiology (J.W.B., T.v.d.B., L.J.G., A.M.d.H., T.W., J.J.M.Z., W.P.T.M.M., P.A.d.J., J.H.), University Medical Center Utrecht, Utrecht /University, the Netherlands
From the Department of Radiology (J.W.B., T.v.d.B., L.J.G., A.M.d.H., T.W., J.J.M.Z., W.P.T.M.M., P.A.d.J., J.H.), University Medical Center Utrecht, Utrecht /University, the Netherlands.
AJNR Am J Neuroradiol. 2024 Apr 8;45(4):386-392. doi: 10.3174/ajnr.A8212.
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.
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.
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; = .02), lower mean GM volumes (597 [SD, 53] mL versus 632 [SD, 53] mL; < .01), more white matter lesions (2.6; range, 0.5-7.5 versus 1.1; range, 0.5-2.4) mL; = .05), and more lacunar infarctions (64 versus 8, = .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).
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.
弹性假黄瘤患者颈动脉虹吸段钙化可能会增加动脉血流搏动,从而导致其脑血管疾病高发。本研究旨在比较弹性假黄瘤患者与对照组之间颅内动脉血流搏动、脑容量和小血管疾病标志物,并探讨弹性假黄瘤患者动脉钙化与搏动之间的关系。
50 例弹性假黄瘤患者和 40 名年龄和性别匹配的对照组接受了 3T MR 成像检查,包括二维相位对比采集评估颈内动脉和大脑中动脉的血流搏动,以及 FLAIR 采集评估脑容量、白质病变和梗死。所有弹性假黄瘤患者均行 CT 扫描以测量虹吸钙化。比较患者和对照组之间的血流搏动(二维相位对比)、脑容量、白质病变和梗死(三维 T1 和三维 T2 FLAIR)。采用线性回归模型检验弹性假黄瘤患者虹吸钙化与搏动之间的关系。
弹性假黄瘤患者(平均年龄 57[标准差 12]岁,24 名男性)搏动指数显著升高(1.05[范围 0.94-1.21]与 0.94[范围 0.82-1.04];<0.01),平均 GM 体积显著降低(597[标准差 53]mL 与 632[标准差 53]mL;<0.01),白质病变更多(2.6[范围 0.5-7.5]与 1.1[范围 0.5-2.4]mL;=0.05),腔隙性梗死更多(64 例与 8 例;=0.04)。颈动脉虹吸钙化与弹性假黄瘤患者的搏动指数升高相关(β=0.10[95%CI 0.01-0.18])。
弹性假黄瘤患者颅内动脉血流搏动和小血管疾病指标升高。颈动脉虹吸钙化可能是弹性假黄瘤患者高发脑血管疾病的原因。