Matsubara Shunji, Satoh Koichi, Satomi Junichiro, Shigekiyo Toshio, Kinouchi Tomoya, Miyake Hajimu, Nagahiro Shinji
Department of Neurosurgery, Institute of Health Biosciences, The University of Tokushima Graduate School.
Neurol Med Chir (Tokyo). 2014;54(3):245-52. doi: 10.2176/nmc.cr2012-0311. Epub 2013 Oct 25.
Two patients with protein S deficiency with acquired multiple pial and dural arteriovenous fistulae (AVFs) following superior sagittal sinus (SSS) thrombosis are reported. Case 1 is a 38-year-old male with protein S deficiency who developed generalized seizure due to SSS thrombosis. Local fibrinolysis was achieved in the acute stage. His 10-month follow-up angiogram revealed an asymptomatic acquired dural AVF arising from the middle meningeal artery and the anterior cerebral artery with drainage to the thrombosed cortical vein in the right frontal lobe. Furthermore, his 2-year follow-up angiogram revealed a de novo pial AVF from the middle cerebral artery in the Sylvian fissure with drainage to the cortical vein initially thrombosed. However, this asymptomatic pial AVF caused bleeding in the ipsilateral cerebral hemisphere 12 years after onset, whereas the dural AVF spontaneously disappeared. Surgical disconnection was successfully performed to eliminate the source of hemorrhage. Case 2 is a 50-year-old male with a past history of SSS thrombosis with protein S deficiency who developed pulsatile tinnitus and generalized seizure. His angiogram showed a cortical dural AVF in the left parietal lobe and a sporadic dural AVF involving the right sigmoid sinus. The parietal lesion was eliminated by transarterial embolization followed by craniotomy. However, a de novo pial AVF emerged from the middle cerebral artery adjacent to the previously treated lesion. Of four cortical AVFs in two patients, thrombosis of cortical veins caused by protein S deficiency might play an important role in their formation. Long-term follow-up is required because this peculiar disorder has an unusual clinical course.
本文报告了2例蛋白S缺乏症患者，其在上矢状窦（SSS）血栓形成后出现了后天性多发性软脑膜和硬脑膜动静脉瘘（AVF）。病例1为一名38岁男性，患有蛋白S缺乏症，因SSS血栓形成而发生全身性癫痫发作。急性期实现了局部纤溶。他10个月的随访血管造影显示，一个无症状的后天性硬脑膜AVF，起源于脑膜中动脉和大脑前动脉，引流至右侧额叶血栓形成的皮质静脉。此外，他2年的随访血管造影显示，在外侧裂出现一个起源于大脑中动脉的新生软脑膜AVF，引流至最初血栓形成的皮质静脉。然而，这个无症状的软脑膜AVF在发病12年后导致同侧脑半球出血，而硬脑膜AVF自发消失。成功进行了手术切断以消除出血源。病例2为一名50岁男性，有蛋白S缺乏症伴SSS血栓形成病史，出现搏动性耳鸣和全身性癫痫发作。他的血管造影显示左顶叶有一个皮质硬脑膜AVF和一个累及右侧乙状窦的散发性硬脑膜AVF。通过动脉内栓塞继而行开颅手术消除了顶叶病变。然而，在先前治疗病变附近的大脑中动脉出现了一个新生的软脑膜AVF。在这2例患者的4个皮质AVF中，蛋白S缺乏引起的皮质静脉血栓形成可能在其形成中起重要作用。由于这种特殊疾病有不寻常的临床病程，需要长期随访。
