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Antikörperassoziierte Erkrankungen der grauen Substanz des ZNS

Diagnostik und Therapie

Antibody-associated diseases of the gray matter of the CNS

Diagnosis and treatment

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Zusammenfassung

In den vergangenen 10 Jahren wurden mit der Entdeckung neuer Autoantikörper die Diagnostik und das Verständnis immunvermittelter Erkrankungen der grauen Substanz des Zentralnervensystems (ZNS) vorangetrieben. Die neu beschriebenen Antikörper gegen neurale Oberflächenantigene, also Rezeptoren und Kanäle sowie assoziierte Proteine, scheinen einen direkten pathogenen Effekt auf die Neurone des ZNS auszuüben; erfreulicherweise lässt sich ein günstiger Effekt von Immuntherapien belegen. Die Diagnose einer autoantikörperassoziierten Erkrankung der grauen ZNS-Substanz setzt die Kombination eines typischen klinischen Syndroms oder eines charakteristischen paraklinischen Befunds (Magnetresonanztomographie, Liquor, Histopathologie) mit dem Nachweis eines spezifischen Antikörpers voraus. Der Beitrag gibt zunächst einen Überblick über die mit diesen Autoantikörpern assoziierten Syndrome, sodann wird die Diagnostik dieser Syndrome besprochen, und schließlich werden vorläufige Empfehlungen zur Therapie unterbreitet.

Summary

In the past 10 years, the diagnosis and understanding of immune-mediated diseases of the gray matter of the CNS have greatly advanced with the discovery of autoantibodies in serum and CSF of affected patients. The newly described antibodies against neuronal surface antigens, i.e., receptors, channels, and associated proteins, seem to have a direct pathogenic effect on CNS neurons. Fortunately, there is a beneficial effect of immunotherapy in many patients. The diagnosis of autoantibody-associated disease of the CNS gray matter requires the combination of a typical clinical syndrome or a characteristic paraclinical finding (MRI, CSF, histopathology) with the detection of a specific antibody. In the following, an overview will first be given of the syndromes typically associated with autoantibodies. Thereafter, the diagnosis of these syndromes is discussed, and finally, preliminary treatment recommendations are given.

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Interessenkonflikt

Der korrespondierende Autor weist auf folgende Beziehungen hin: C.G. Bien führt im Labor seiner Klinik Autoantikörperdiagnostik durch, u. a. mit Assays der Firma Euroimmun, Lübeck. Für diese Diagnostik rechnet seine Klinik mit auswärtigen Einsendern Gebühren ab.

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  1. Epilepsie-Zentrum Bielefeld-Bethel, Krankenhaus Mara, Maraweg 21, 33617, Bielefeld, Deutschland

    C.G. Bien

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Correspondence to C.G. Bien.

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Bien, C. Antikörperassoziierte Erkrankungen der grauen Substanz des ZNS. Nervenarzt 82, 986–993 (2011). https://doi.org/10.1007/s00115-011-3257-z

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