Merta M, Rysavá R
I. interní klinika 1. lékarské fakulty Univerzity Karlovy, Praha, Czech Republic.
Sb Lek. 1996;97(4):479-85.
An overview of new insights in pathogenesis of intracranial manifestations-especially concerning intracranial aneurysms (ICA) and subarachnoid hemorrhage-in autosomal dominant polycystic kidney disease (ADPKD) is given. Results of CT examinations of 65 patients with ADPKD are presented: they disclosed findings suspects of intracranial aneurysms in 4 patients, other vessel abnormalities and malformations in 6 patients and cystic dilatations in cisterna magna-of arachnoid cyst type in 6 patients. A case story of a patient successfully treated for subarachnoid hemorrhage due to aneurysm rupture is presented. Indications and screening methods in ADPKD patients with ICA are discussed. Brain abnormalities in ADPKD-especially ICA-suggest the complex nature of this genetic disorder, and regarding to the possible clinical impact, should be adequately managed.
本文概述了常染色体显性多囊肾病（ADPKD）颅内表现——尤其是颅内动脉瘤（ICA）和蛛网膜下腔出血——发病机制的新见解。呈现了65例ADPKD患者的CT检查结果：其中4例发现疑似颅内动脉瘤，6例发现其他血管异常和畸形，6例发现蛛网膜囊肿型小脑延髓池囊性扩张。讲述了一名因动脉瘤破裂导致蛛网膜下腔出血而成功接受治疗的患者的病例。讨论了ADPKD合并ICA患者的指征和筛查方法。ADPKD中的脑部异常——尤其是ICA——表明了这种遗传疾病的复杂性，鉴于其可能的临床影响，应进行适当处理。
