Autoimmun vermittelte Erkrankungen des ZNS jenseits der MS

Autoimmun vermittelte Erkrankungen des ZNS umfassen viele komplexe Krankheitsbilder, die über die MS hinausgehen. Der Artikel beleuchtet seltenere, aber bedeutende Entzündungen der weißen und grauen Hirn- und Rückenmarksubstanz. Dabei werden die Krankheitsmechanismen, spezifischen Autoantikörper und Bildgebungsmuster erläutert, die für eine präzise Diagnose entscheidend sind. Zudem werden Empfehlungen für die Bildgebung und Differenzialdiagnostik gegeben.

Abstract

Autoimmune-mediated diseases of the central nervous system encompass many complex conditions that extend beyond multiple sclerosis. The article highlights rarer but significant inflammations of the white and gray matter of the brain and spinal cord. It explains the disease mechanisms, specific autoantibodies, and imaging patterns that are crucial for an accurate diagnosis. Additionally, recommendations for imaging and differential diagnosis are provided.

Kernaussagen

• Die Differenzierung einer NMOSD oder einer MOGAD von einer MS ist wichtig, weil bei einer Fehldiagnose die für MS vorgesehenen Medikamente bei beiden Krankheitsentitäten den Krankheitsverlauf ungünstig beeinflussen können.

• Red Flags für NMOSD und MOGAD, die für andere Diagnosen (vor allem MS) sprechen, sind (nach Matthews-Jurynczyk und Wingerchuk):

  • MS-typische Herde im Gehirn, vor allem wenn (i) mindestens eine Läsion am Seitenventrikel und im unteren Temporallappen angrenzt, (ii) periventrikuläre Läsionen vom Typ Dawson-Finger und (iii) juxtakortikale U-Faser-Läsionen vorhanden sind

  • partielle transverse Myelitis, „short segment myelitis“ (SSM) mit longitudinaler Ausdehnung < 3 Wirbelkörperhöhen

  • meningeale Kontrastmittelaufnahme;

  • unveränderte Kontrastmittelaufnahme über einen Zeitraum von mehr als 3 Monaten

• Bei AE mit limbischem Syndrom sollten sich temporomesiale T2/FLAIR-hyperintense Veränderungen auf beiden Seiten zeigen; bei einseitiger Signalerhöhung ist bildgebend allenfalls eine limbische Enzephalitis differenzialdiagnostisch in Erwägung zu ziehen.

Schlüsselwörter

Autoimmunerkrankungen - Neuromyelitis-optica-Spektrum-Erkrankungen - Myelin-Oligodendrozyten-Glykoprotein-Immunoglobulin-G-Antikörper-assoziierte Erkrankungen - akute disseminierte Enzephalomyelitis - autoimmune Enzephalitis

Keywords

autoimmune mediated diseases - neuromyelitis optica spectrum disorder - myelin oligodendrocyte glycoprotein antibody diseases - acute disseminated encephalomyelitis - autoimmune encephalitis

Publication History

Article published online:
20 September 2024

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