Spontane Dissektionen der extra- und intrakraniellen hirnversorgenden Arterien^[*]

Zusammenfassung

Hintergrund: Das Manuskript ist eine aktualisierte Version der 2012 entstandenen DGN-Leitlinie zu spontanen Dissektionen der extrakraniellen und intrakraniellen hirnversorgenden Arterien. Ziel der Leitlinie ist, den gegenwärtigen Stand des Wissens zur Diagnostik und Therapie anhand der zur Verfügung stehenden wissenschaftlichen Literatur zusammenzufassen und daraus wissenschaftlich begründete Empfehlungen abzuleiten. Die Leitlinie behandelt ausschließlich spontane Dissektionen, traumatische Dissektionen werden nicht berücksichtigt.

Methodik: Das Leitliniengremium wurde durch Experten der Neurologie gebildet, die eine besondere Expertise in der vaskulären Neurologie besitzen und insbesondere auf dem Gebiet der Dissektion der hirnversorgenden Arterien Forschungsarbeit geleistet haben. Sie wurden von der Deutschen Gesellschaft für Neurologie bestimmt. Die Vorstände der Deutschen Gesellschaften für Neuroradiologie, Neurochirurgie und Gefäßchirurgie waren an der Formulierung der Leitlinie ebenfalls beteiligt und stimmten dieser zu. Die seit 2012 neu erschienene Literatur wurde von den Autoren gesichtet, und, soweit innovativ und im Hinblick auf die Anzahl der Patienten bedeutsam, in die aktuelle Fassung der Leitlinie eingearbeitet. Die Leitlinie nimmt Stellung zu (1) neuen Erkenntnissen über die Pathophysiologie der Dissektion (weil diese für die Auswahl der geeigneten Diagnostik und die Wahl der optimalen Untersuchungszeitpunkte sowie für das Verständnis der gewählten Therapieansätze bedeutsam ist), (2) zur Wertigkeit der verschiedenen diagnostischen Verfahren (insbesondere der extrakraniellen und transkraniellen Farbduplexsonografie, der CT-Angiografie, der MR-Angiografie und spezieller MRT-Techniken zum Nachweis des intramuralen Hämatoms, einschließlich der Hirnparenchymdiagnostik) und (3) zu den verschiedenen therapeutischen Ansätzen in der Prävention von Insulten. Im therapeutischen Kapitel werden auch interventionelle Therapieansätze bewertet, die aber bei der Dissektion der Zervikalarterien nur eine sehr untergeordnete Rolle spielen. Aufgrund des Fehlens großer randomisierter kontrollierter Studien wurde als Leitlinienstufe der Typ S1 ausgewählt. Die Regeln der AWMF und der DGN zum Umgang mit potenziellen Interessenkonflikten wurden während des Konsensprozesses gewahrt.

Ergebnis: Unter Berücksichtigung der aktuellen wissenschaftlichen Literatur werden konsensusbasierte Empfehlungen mit Erläuterung der Pathophysiologie dieser wenig erforschten Gefäßkrankheit und mit Empfehlungen zur klinischen und gestuften bildgebenden Diagnostik, zur frühen Sekundärprävention mit Thrombozytenfunktionshemmern, Heparin und Antikoagulanzien und zu den Verlaufscharakteristika der Erkrankung sowie der erforderlichen Verlaufsdiagnostik erarbeitet. Die Indikation zu invasiven interventionellen oder zu intensivmedizinischen Maßnahmen in speziellen Einzelfällen wurde ebenfalls erörtert.

Schlussfolgerung: Der Pathophysiologie der Dissektion der hirnversorgenden Arterien liegt eine intramurale Blutung zugrunde. Die duale bildgebende Diagnostik mittels MRT und neurovaskulärem Ultraschall ist weiterhin die Diagnostik der ersten Wahl, der Stellenwert der CCT und CTA als reliable Alternative zum MRT hat zugenommen. Die Sekundärprophylaxe von Hirninfarkten oder flüchtigen Insulten sollte primär mit Thrombozytenfunktionshemmern erfolgen, da in den bislang vorliegenden Studien keine Überlegenheit der Antikoagulation nachgewiesen werden konnte. Als Verlaufsuntersuchung ist der neurovaskuläre Ultraschall am besten geeignet. Interventionelle, rekanalisierende Verfahren sind im Einzelfall gerechtfertigt, da positive Fallberichte vorliegen. In seltenen Fällen kann eine mehrtägige Intensivtherapie mit induzierter Hypertonie erforderlich werden.

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.

Schlüsselwörter

Leitlinie - Schlaganfall - Dissektion - hirnversorgende Arterien - zervikale Arterien

Keywords

guideline - stroke - Dissection - brainsupplying arteries - cervical arteries

^* Den Leitlinientext zusammen mit der Interessenkonflikterklärung finden Sie auch auf www.dgn.org/leitlinien sowie www.awmf.de.

^** Die Autoren teilen sich die Erstautorenschaft.

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