Spontaneous Dissections of Extracranial and Intracranial Brain Arteries

Ringelstein E., Dittrich R., Sitzer M., Arnold M., Haring H., Frese A.

Abstract

Background: The manuscript is an update of the 2012 published version of the guideline and recommendations of the German Neurological Society (DGN) for spontaneous extra- and intracranial dissections of the brain supplying arteries. Focused on the aspects, pathophysiology, diagnostics and therapy the aim was to summarize the actual scientific literature to provide up-to-date evidence-based recommendations. The guideline does not consider traumatic dissections. Methods: Experts in the field of Neurology with outstanding knowledge of vascular neurology especially with expertise in cervical artery dissections were nominated by the German Neurological Society. In cooperation with the German Societies of Neuroradiology, Neurosurgery and Vascular Surgery these guideline recommendations were prepared. The actual scientific literature was analyzed and weighted. The guideline focuses on (1) new insights concerning the pathophysiology of cervical artery dissections (this is important for the optimal diagnostic modalities and time course of investigations, as well as for the optimal therapy), (2) the diagnostic value of neurovascular ultrasound, CT-Angiography, MR-Angiography and special MRI techniques to detect the intramural hematoma and (3) different therapeutic strategies in the acute and chronic phase of the disease including interventional therapies and intensive care medicine in rare cases. The guideline was prepared at an S1-level according to the standards of the Association of the Scientific Medical Societies in Germany (Arbeitsgemeinschaft der Wissenschaftlichen Medizinischen Fachgesellschaften e.V., AWMF). Results: With respect to the actual scientific literature consensual recommendations concerning the pathophysiology, the stepwise diagnostics, secondary prevention by platelet inhibition and anticoagulation are provided. The guideline also covers systemic thrombolysis, interventional therapy, special pharmacologic therapy and follow-up investigations in cervical artery dissection. Conclusion: The pathophysiology of cervical artery dissection is now better understood, the disease is caused by an intramural hematoma of the brain arteries. In case of suspected cervical artery dissection, a dual diagnostic approach with MRI and Ultrasound remains the first-line standard although CTA has become a reliable alternative. The preferred secondary stroke prevention consists of antiplatelet agents because of absence of randomized studies demonstrating superiority of anticoagulation. Neurovascular ultrasound is the method of choice during follow-up investigations. In individual cases, various recanalizing interventional therapies or intensive care with induced hypertension can become necessary.