Department of Neurology, Hainan General Hospital, Hainan Affiliated Hospital of Hainan Medical University, Supported by Hainan Province Clinical Medical Center, Haikou, China.
Department of Urology, Hainan General Hospital, Hainan Affiliated Hospital of Hainan Medical University, Haikou, China.
BMC Neurol. 2022 May 2;22(1):164. doi: 10.1186/s12883-022-02693-3.
To describe the characteristics of patients with cerebral venous sinus thrombosis (CVST) and dural arteriovenous fistula (AVF) associated with protein S (PS) deficiency.
We conducted a search of medical records in Hainan General Hospital from January 2000 to December 2020 for coexistence of CVST and dural AVF associated with PS deficiency and searched PubMed、Embase and Chinese biomedical databases (CBM) for all literature describing CVST and dural AVF with PS. We analyzed clinical characteristics, location, sequence of CVST and dural AVF, level of PS, therapeutic methods and prognosis.
We presented 1 patient in our hospital's database combined CVST and dural AVF associated with PS, plus 5 cases reported in literature. The most common symptoms were headache, generalized seizure, disturbance of consciousness. The most frequent location of CVST was at internal cerebral vein, while transverse sinus, sigmoid sinus, parietal region in dural AVF. Two patients developed dural AVF several months or years after CVST. Clinical characteristics and level of PS were summarized.
These findings alert physicians to consider PS deficiency in patients who suffer from CVST, especially those combined with dural AVF.
描述蛋白 S（PS）缺乏症与脑静脉窦血栓形成（CVST）和硬脑膜动静脉瘘（AVF）相关患者的特征。
我们检索了海南总医院 2000 年 1 月至 2020 年 12 月期间与 PS 缺乏相关的 CVST 和硬脑膜 AVF 并存的病历，并在 PubMed、Embase 和中国生物医学数据库（CBM）中检索了所有描述 PS 相关 CVST 和硬脑膜 AVF 的文献。我们分析了临床特征、CVST 和硬脑膜 AVF 的位置、序列、PS 水平、治疗方法和预后。
我们在医院的数据库中发现了 1 例合并 PS 缺乏的 CVST 和硬脑膜 AVF 的患者，并结合文献中的 5 例报告。最常见的症状是头痛、全身性癫痫、意识障碍。CVST 最常见的部位是大脑内静脉，而硬脑膜 AVF 最常见的部位是横窦、乙状窦、顶区。2 例患者在 CVST 发生数月或数年后出现硬脑膜 AVF。总结了临床特征和 PS 水平。
这些发现提醒医生在 CVST 患者中，尤其是合并硬脑膜 AVF 的患者中，要考虑 PS 缺乏症。
