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Review
. 2022 Jun;79(5):247-253.
doi: 10.1024/0040-5930/a001358.

[Primary CNS Vasculitis - An Overview]

[Article in German]
Helly Hammer  1 , Nicole Kamber  1 , Christoph Friedli  1 , Robert Hoepner  1 , Lara Diem  1 , Andrew Chan  1 , Anke Salmen  1
Affiliations
Review

[Primary CNS Vasculitis - An Overview]

[Article in German]
Helly Hammer et al. Ther Umsch. 2022 Jun.

Abstract
in English, German

Primary CNS Vasculitis - An Overview Abstract. Cerebral vasculitis, especially the primary vasculitis of the central nervous systems (primary CNS vasculitis), are rare inflammatory diseases of the small- and medium-sized vessels of the CNS. The pathogenesis of primary CNS vasculitis is unclear. Infectious triggers are hypothesized to induce an activation of the immune system with resulting inflammation of the blood vessels within the CNS. Clinically, primary CNS vasculitis presents heterogeneously with leading symptoms such as headache, memory impairment and other neurological deficits. A broad diagnostic work-up prior to treatment initiation is crucial. Treatment consists of immunotherapy (pulse and maintenance therapy) and requires long-term neurological treatment and follow-up due to the increased risk of recurrence of the disease.

Zusammenfassung. Die zerebralen Vaskulitiden, insbesondere die primäre Vaskulitis des Zentralnervensystems (primäre ZNS-Vaskulitis), sind seltene entzündliche Erkrankungen der kleinen und mittelgrossen Gefässe des ZNS. Klinisch präsentiert sich die primäre ZNS-Vaskulitis heterogen mit Leitsymptomen wie Kopfschmerzen, Gedächtnisstörungen und anderen neurologischen Defiziten. Die Pathogenese der primären ZNS-Vaskulitis ist unklar. Vermutet wird eine infektgetriggerte Aktivierung des Immunsystems mit daraus resultierender Inflammation der Blutgefässe des ZNS. Die breite differentialdiagnostische Abklärung vor Therapieeinleitung spielt eine entscheidende Rolle. Die Behandlung besteht aus einer Immuntherapie (Puls- und Erhaltungstherapie) und bedarf aufgrund des erhöhten Rezidivrisikos der Erkrankung einer langfristigen neurologischen Anbindung und Therapie.

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