Fortschr Neurol Psychiatr 2008; 76(1): 21-26
DOI: 10.1055/s-2007-993032
Übersicht
© Georg Thieme Verlag Stuttgart · New York

Neuromyelitis optica: eine mit Aquaporin-4-Antikörpern assoziierte nosologische Entität

Neuromyelitis Optica: A Separate Disease Entity Associated with Aquaporin-4-AntibodiesB.  Jordan1 , K.  Eger1 , M.  E.  Kornhuber1 , S.  Zierz1
  • 1Klinik und Poliklinik für Neurologie (Direktor: Prof. Dr. S. Zierz), Martin-Luther-Universität Halle-Wittenberg, Halle/Saale
Further Information

Publication History

Publication Date:
14 January 2008 (online)

Zusammenfassung

Die Neuromyelitis optica (NMO; Devic-Syndrom) bezeichnet eine oft schwerwiegend verlaufende, idiopathische, entzündliche schubhaft-remittierende Erkrankung der Sehnerven sowie des Myelons. Die Abgrenzung der NMO von der Multiplen Sklerose (MS) als separate Entität wurde lange kontrovers diskutiert und ist klinisch insbesondere im Initialstadium der Erkrankung schwierig. Obwohl beide Erkrankungen mit Demyelinisierung einhergehen, unterscheiden sie sich in typischen klinischen, bildgebenden und immunpathologischen Befunden. Durch die Identifikation von Antikörpern gegen Aquaporin (AQP) im Serum bei Neuromyelitis optica ist es kürzlich gelungen, diese Erkrankung klar von der Multiplen Sklerose zu trennen. Darüber hinaus wird durch den Nachweis der Aquaporin-Antikörper die hämatogen-humorale Autoimmunpathogenese der NMO belegt und eine frühzeitige effektive Therapieeinleitung zur Verhinderung schubassoziierter Behinderung ermöglicht.

Abstract

Neuromyelitis optica (NMO; Devic's Syndrome) is an idiopathic, often relapsing, severe inflammatory disorder preferentially affecting optic nerves and spinal cord. The distinction of NMO from multiple sclerosis (MS) as a separate disease entity has been controversally discussed for a long time. Though both diseases show demyelinisation, they differ in typical clinical, imaging and immunopathological findings. The recent identification of serum aquaporin (AQP)-4 antibody clearly separates NMO from MS. In addition, the identification of AQP-4 antibodies supports peripheral humoral autoimmune pathogenesis in NMO and permits early initiation of effective therapy for prevention of attack-related disability.

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Dr. Berit Jordan

Klinik und Poliklinik für Neurologie, Martin-Luther-Universität Halle-Wittenberg

Ernst-Grube-Str. 40

06097 Halle/Saale

Email: Berit.Jordan@medizin.uni-halle.de