Vaskulitiden des zentralen Nervensystems

Zusammenfassung

Vaskulitiden des zentralen Nervensystems (ZNS) sind definiert durch entzündliche Veränderungen intrakranieller und gelegentlich spinaler Gefäße und kommen als zerebrale Manifestationen systemischer Vaskulitiden, als sekundäre Vaskulitis bei nicht vaskulitischer Grunderkrankung oder als isolierte ZNS-Angiitis (IAZNS) vor. Obwohl selten, müssen sie aufgrund der Vielfalt und fehlenden Spezifität klinischer und radiologischer Befunde sehr häufig differenzialdiagnostisch berücksichtigt werden. Bei Patienten mit subakuten Krankheitsbildern, multifokaler neurologischer Symptomatik, Kopfschmerzen, psychiatrischen Symptomen oder systemischer Entzündung sowie jungen Schlaganfallpatienten ohne typische Risikofaktoren bzw. mit rheumatologischen Begleiterkrankungen sollte an eine ZNS-Vaskulitis gedacht werden. Neben dem Nachweis der Vaskulitis selbst sind die Klärung der Ätiologie und insbesondere der Ausschluss von Infektionen besonders wichtig. Auch durch invasive diagnostische Maßnahmen wie die zerebrale Angiografie und die als diagnostischer Goldstandard geltende ZNS-Biopsie kann speziell die Diagnose einer IAZNS oft nur wahrscheinlich gemacht werden. Neben der Therapie etwaiger Grunderkrankungen werden zerebrale Vaskulitiden meist kombiniert mit Kortikosteroiden und Immunsuppressiva behandelt. Dabei sollten Schwere der Erkrankung und der Grad der diagnostischen Sicherheit berücksichtigt werden.

Abstract

Central nervous system (CNS) vasculitides are defined by inflammatory changes of intracranial and, occasionally, spinal vessel walls. They occur as cerebral manifestations of systemic vasculitides or can be a result of non-vasculitic diseases or present as isolated angiitis of the CNS. Although being rare diseases, they frequently have to be considered in the differential diagnosis due to the etiologically non-specific nature of clinical and radiological findings. A subacute presentation of multifocal neurological symptoms, headache, psychiatric symptoms or systemic inflammation, or the occurrence of stroke in patients of young age lacking a vascular risk profile or with known concomitant rheumatological diseases should raise the suspicion of CNS vasculitis. In addition to the confirmation of vasculitis, its etiology must be clarified, and – most importantly – infections have to be ruled out. In the case of isolated CNS angiitis, even invasive diagnostic measures such as cerebral angiography and CNS biopsy, which is the diagnostic gold standard, often do not lead to a definitive diagnosis. In addition to the treatment of any causative conditions, cerebral vasculitides are typically treated with a combination of corticosteroids and immunosuppressants. Instead of a uniform approach, disease severity and the degree of diagnostic certainty should be considered for the choice of treatment.

Schlüsselwörter

Vaskulitis - zentrales Nervensystem - Schlaganfall - rheumatische Erkrankungen - Kollagenosen

Key words

vasculitis - central nervous system - stroke - rheumatic diseases - connective tissue diseases

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