The Challenging Clinical Management of Patients with Cranial Dural Arteriovenous Fistula and Secondary Parkinson’s Syndrome: Pathophysiology and Treatment Options

• Main Authors: Julia Velz, Zsolt Kulcsar, Fabian Büchele, Heiko Richter, Luca Regli
• Format: Article
• Language: English
• Published: Karger Publishers 2020-10-01
• Series: Cerebrovascular Diseases Extra
• Subjects: intracranial dural arteriovenous fistula; venous sinus thrombosis; shunt; venous hypertension; parkinsonism
• Online Access: https://www.karger.com/Article/FullText/510597

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.