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The Challenging Clinical Management of Patients with Cranial Dural Arteriovenous Fistula and Secondary Parkinson’s Syndrome: Pathophysiology and Treatment Options
Cerebrovascular Diseases Extra Pub Date : 2020-10-22 , DOI: 10.1159/000510597
Julia Velz 1, 2 , Zsolt Kulcsar 2, 3 , Fabian Büchele 2, 4 , Heiko Richter 5 , Luca Regli 6, 7
Affiliation  

Cranial dural arteriovenous fistula (cDAVF) may rarely lead to parkinsonism and rapid cognitive decline. Dysfunction of the extrapyramidal system and the thalamus, due to venous congestion of the Galenic system with subsequent parenchymal edema, is likely to represent an important pathophysiological mechanism. Here, we report a case of a 57-year-old man with a cDAVF of the straight sinus (Borden type III; DES-Zurich bridging vein shunt [BVS] type with direct, exclusive, and strained leptomeningeal venous drainage [LVD]) and subsequent edema of both thalami, the internal capsule, the hippocampi, the pallidum, and the mesencephalon. Several attempts at venous embolization were unsuccessful, and the neurological condition of the patient further deteriorated with progressive parkinsonism and intermittent episodes of loss of consciousness (KPS 30). A suboccipital mini-craniotomy was performed and the culminal vein was disconnected from the medial tentorial sinus, achieving an immediate fistula occlusion. Three-month follow-up MRI revealed complete regression of the edema. Clinically, parkinsonism remitted completely, allowing for tapering of dopaminergic medication. His cognition markedly improved in further course. The purpose of this report is to highlight the importance of rapid and complete cDAVF occlusion to reverse venous hypertension and prevent progressive clinical impairment. The review of the literature underlines the high morbidity and mortality of these patients. Microsurgical disconnection of the fistula plays an important role in the management of these patients and, surprisingly, has not been reported so far.
Cerebrovasc Dis Extra 2020;10:124–138


中文翻译:

颅硬脑膜动静脉瘘和继发性帕金森综合症患者的具有挑战性的临床治疗:病理生理学和治疗选择

颅硬脑膜动静脉瘘(cDAVF)可能很少导致帕金森氏症和快速的认知功能下降。锥体外系和丘脑的功能障碍,由于盖伦系统的静脉充血并随后发生实质性水肿,很可能代表了重要的病理生理机制。在这里,我们报道一例57岁的男子,其cDAVF为直窦(Borden III型; DES-Zurich桥接静脉分流[BVS]型,具有直接,排他性和应变性软脑膜静脉引流[LVD])以及随后的丘脑,内囊,海马,苍白球和中脑水肿。进行静脉栓塞的几次尝试均未成功,并且患者的神经系统疾病因进行性帕金森病和意识丧失的间歇发作而进一步恶化(KPS 30)。进行枕下颅骨小颅骨切开术,并将高位静脉与腱中膜窦分开,立即将瘘管闭塞。三个月的随访MRI显示水肿完全消退。临床上,帕金森综合症完全缓解,可以逐渐减少多巴胺能药物。他的认知在以后的过程中明显提高。本报告的目的是强调快速和完全cDAVF闭塞对逆转静脉高血压和预防进行性临床损害的重要性。文献综述强调了这些患者的高发病率和死亡率。瘘管的显微外科手术断开在这些患者的治疗中起着重要作用,令人惊讶的是,迄今为止尚未见报道。立即瘘管闭塞。三个月的随访MRI显示水肿完全消退。临床上,帕金森综合症完全缓解,可以逐渐减少多巴胺能药物。他的认知在以后的过程中明显提高。本报告的目的是强调快速和完全cDAVF闭塞对逆转静脉高血压和预防进行性临床损害的重要性。文献综述强调了这些患者的高发病率和死亡率。瘘管的显微外科手术断开在这些患者的治疗中起着重要作用,令人惊讶的是,迄今为止尚未见报道。立即瘘管闭塞。三个月的随访MRI显示水肿完全消退。临床上,帕金森综合症完全缓解,可以逐渐减少多巴胺能药物。他的认知在以后的过程中明显提高。本报告的目的是强调快速和完全cDAVF闭塞对逆转静脉高血压和预防进行性临床损害的重要性。文献综述强调了这些患者的高发病率和死亡率。瘘管的显微外科手术断开在这些患者的治疗中起着重要作用,令人惊讶的是,迄今为止尚未见报道。他的认知在以后的过程中明显提高。本报告的目的是强调快速和完全cDAVF闭塞对逆转静脉高血压和预防进行性临床损害的重要性。文献综述强调了这些患者的高发病率和死亡率。瘘管的显微外科手术断开在这些患者的治疗中起着重要作用,令人惊讶的是,迄今为止尚未见报道。他的认知在以后的过程中明显提高。本报告的目的是强调快速和完全cDAVF闭塞对逆转静脉高血压和预防进行性临床损害的重要性。文献综述强调了这些患者的高发病率和死亡率。瘘管的显微外科手术断开在这些患者的治疗中起着重要作用,令人惊讶的是,迄今为止尚未见报道。
Cerebrovasc Dis Extra 2020; 10:124–138
更新日期:2020-10-28
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