Veltkamp R, Veltkamp C, Hartmann M, Schönffeldt-Varas Ph, Schwaninger M
Neurologische Klinik, Ruprecht-Karls-Universität Heidelberg.
Nervenarzt. 2004 Feb;75(2):149-52. doi: 10.1007/s00115-003-1644-9.
Autosomal dominant polycystic kidney disease (ADPKD) is a frequent, genetically heterogenous disease with renal and extrarenal manifestations. Intracranial aneurysms are found in about 10% of cases. Other vascular manifestations of ADPKD have been described only in small case series. We report a 44-year-old ADPKD patient who developed acute large middle cerebral artery infarction secondary to subpetrous dissection of the internal carotid artery. Six months after the stroke, pseudoaneurysm was demonstrated on magnetic resonance angiography at the site of a previous dissection. Based on this case report, we review the spectrum of neurovascular manifestations and stroke associated with ADPKD and summarize current concepts of the pathogenesis of this disease. Finally, special aspects of the diagnostic evaluation and therapeutic management in patients with ADPKD and cervicocephalic dissection are discussed.
常染色体显性多囊肾病(ADPKD)是一种常见的、具有遗传异质性的疾病,有肾脏和肾外表现。约10%的病例中可发现颅内动脉瘤。ADPKD的其他血管表现仅在小病例系列中有所描述。我们报告一名44岁的ADPKD患者,其因颈内动脉岩骨段下夹层分离继发急性大脑中动脉大面积梗死。卒中后6个月,磁共振血管造影显示先前夹层分离部位存在假性动脉瘤。基于本病例报告,我们回顾了与ADPKD相关的神经血管表现和卒中谱,并总结了该疾病发病机制的当前概念。最后,讨论了ADPKD合并颈脑夹层分离患者诊断评估和治疗管理的特殊方面。