Stroke:血管性埃勒斯-当洛斯综合征患者自发性颈动脉夹层

2021-03-04 MedSci原创 MedSci原创

颈动脉病变在血管性埃勒斯-当洛斯综合征患者中很常见,且大多无临床症状。局部夹层和动脉瘤是最常见的病变类型,但短暂性脑缺血发作或卒中较少见。

血管性埃勒斯-当洛斯综合征是一种罕见的遗传性结缔组织疾病,其致病原因是COL3A1基因变异。动脉并发症可影响所有解剖部位,约25%累及主动脉以上主干(SATs),但尚未有研究对颈动脉病变进行系统评估。

近日,心血管疾病领域权威杂志Stroke上发表了一篇研究文章,该研究旨在明确在血管性埃勒斯-当洛斯综合征诊断时和随访期间自发性SAT病变的准确患病率。该研究的次要目的是探究他们的神经结局(短暂性脑缺血发作或卒中),以及与性别、基因型、确定状态之间的可能关系。

该研究是2000年至2017年期间在三级转诊中心经分子检测证实为血管性埃勒斯-当洛斯综合征患者的单中心队列进行的一项回顾性研究。

144例患者被纳入该研究进行分析,56.9% (n=82)患者有SAT病变:64.6%为女性。大多数病变是在早期动脉评估中发现的(48%是在第一次检查中发现,平均年龄为35.7±13.0岁)。在40岁时首次发现SAT病变的累积发生率为41.7%。在整个研究期间,研究人员共发现了183个SAT病变(132个夹层和33个动脉瘤),且主要发生于颈内动脉(56.3%)和椎动脉(28.9%),在COL3A1未突变患者中更为罕见(P=0.008)。82例SAT病变患者中有16例(19.5%)出现短暂性脑缺血发作或卒中,且与年龄、性别、治疗或高血压无关。

由此可见,颈动脉病变在血管性埃勒斯-当洛斯综合征患者中很常见,且大多无临床症状。局部夹层和动脉瘤是最常见的病变类型,但短暂性脑缺血发作或卒中较少见

原始出处:

Salma Adham.et al.Spontaneous Cervical Artery Dissection in Vascular Ehlers-Danlos Syndrome A Cohort Study.stroke.2021.https://www.ahajournals.org/doi/10.1161/STROKEAHA.120.032106

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    2021-03-06 sodoo