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Aktuelle Neurologie 2012; 39(01): 33-41
DOI: 10.1055/s-0031-1297261
Übersicht
© Georg Thieme Verlag KG Stuttgart · New York

Neuromyelitis optica

Neuromyelitis optica
B. Wildemann
1   Sektion Molekulare Neuroimmunologie, Neurologische Klinik, Universitätsklinikum Heidelberg
,
S. Jarius
1   Sektion Molekulare Neuroimmunologie, Neurologische Klinik, Universitätsklinikum Heidelberg
,
F. Paul
2   Neurocure Clinical Research Center and Experimental and Clinical Research Center, Charité – Universititätsmedizin, Berlin
› Author Affiliations
Further Information

Publication History

Publication Date:
20 January 2012 (online)

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Zusammenfassung

Die Neuromyelitis optica (NMO, Devic-Syndrom) ist eine entzündliche Erkrankung des Zentralnervensystems mit den Indexereignissen Optikusneuritis und Querschnittsmyelitis. In der Mehrzahl der Fälle ist die NMO autoimmun bedingt und mit dem Vorliegen von Autoantikörpern gegen den Wasserkanal Aquaporin-4 (AQP4) assoziiert. Mit der Entdeckung dieses hochspezifischen Serummarkers gelang erstmalig die Identifizierung eines definierten Zielautoantigens bei einer Erkrankung aus dem Formenkreis der Multiplen Sklerose. Neuere Beobachtungen weisen übereinstimmend darauf hin, dass AQP4-Antikörper auch pathophysiologisch bedeutsam sind und bieten eine Rationale für den Einsatz von Plasmapherese und B-Zell-Depletion in der Therapie der AQP4-Ak-positiven NMO.

Abstract

Neuromyelitis optica (NMO, Devic syndrome) is an autoimmune-mediated disorder of the central nervous system (CNS) with optic neuritis and longitudinally extensive transverse myelitis as key clinical features. The presence of highly specific serum autoantibodies, which target the water channel aquaporin-4 (AQP4), classifies NMO as a distinct entity and as the first inflammatory multiple sclerosis-like disorder of the CNS with a defined autoantigen. Recent observations strongly suggest that the AQP4 antibodies are pathophysiologically relevant and thus provide a rationale for the use of therapeutic strategies directed predominantly against the humoral effector arm of autoimmune responses such as plasma exchange or pharmacological B-cell depletion.

Schlüsselwörter

Neuromyelitis optica - Devic-Syndrom - Antikörper gegen Aquaporin-4 (NMO-IgG) - Longitudinal extensive transverse Myelitis (LETM) - Optikusneuritis

Keywords

neuromyelitis optica - Devic syndrome - antibodies to aquaporin-4 (NMO-IgG) - longitudinally extensive transverse myelitis - optic neuritis
 

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