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Neuromyelitis optica

Neuromyelitis optica

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Zusammenfassung

Die Entdeckung eines Serumautoantikörpers gegen das astrozytäre Wasserkanalprotein Aquaporin-4 (AQP4) als Biomarker der Neuromyelitis optica (NMO, Devic-Syndrom) hat das Verständnis über diese seltene immunvermittelte klinische Entität revolutioniert und ein rational begründetes therapeutisches Vorgehen ermöglicht. In dieser Übersicht stellen wir die klinischen Besonderheiten der NMO sowie neue Erkenntnisse zur pathophysiologischen Bedeutung der gegen Aquaporin-4 gerichteten humoralen und zellulären Immunantwort dar.

Summary

The discovery of serum autoantibodies against the astrocytic water channel aquaporin-4 (AQP4) as a biomarker of neuromyelitis optica (NMO, Devic syndrome) has revolutionized our understanding of this rare immune-mediated clinical entity and has provided a rationale for the use of therapeutic strategies targeting the humoral effector arm of autoimmune responses. This article reviews the clinical features of NMO and highlights recent findings that have elucidated how antibodies and T cells with specificity for AQP4 may be involved in the pathophysiology of the disorder.

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Abbreviations

ADH:

antidiuretisches Hormon

AQP4:

Aquaporin-4

AQP4-Ak:

Aquaporin-4-Antikörper

BHS:

Blut-Hirn-Schranke

LETM:

longitudinal extensive transverse Myelitis

MRT:

Magnetresonanztomographie

MRZ-Reaktion:

polyspezifische intrathekale Immunantwort gegen Masern-, Röteln-, Varizella-zoster-Virus

MS:

Multiple Sklerose

NMO:

Neuromyelitis optica

NK-Zellen:

natürliche Killerzellen

NMO-IgG:

Neuromyelitis-optica-Immunoglobulin G

OKB:

oligoklonale Banden

ON:

Optikusneuritis

ZNS:

zentrales Nervensystem

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Interessenkonflikt

Die korrespondierende Autorin weist für sich und ihre Koautoren auf folgende Beziehungen hin: B. Wildemann und F. Paul werden durch das Bundesministerium für Bildung und Forschung unterstützt (Kompetenznetz Multiple Sklerose). F. Paul wir von der DFG unterstützt (Exc 257). B. Wildemann erhielt Forschungsunterstützung von Merck Serono und Bayer Healthcare. F. Paul erhielt Reiseunterstützung von der Guthy-Jackson Charitable Foundation.

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Authors and Affiliations

  1. Sektion Molekulare Neuroimmunologie, Neurologische Klinik, Universitätsklinikum Heidelberg, Im Neuenheimer Feld 400, 69120, Heidelberg, Deutschland

    B. Wildemann & S. Jarius

  2. Neurocure Clinical Research Center and Experimental and Clinical Research Center, Charité-Universititätsmedizin, Campus Mitte, Berlin, Deutschland

    F. Paul

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  1. B. Wildemann
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  2. S. Jarius
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  3. F. Paul
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Correspondence to B. Wildemann.

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Wildemann, B., Jarius, S. & Paul, F. Neuromyelitis optica. Nervenarzt 84, 436–441 (2013). https://doi.org/10.1007/s00115-012-3602-x

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