TY - JOUR A1 - Jarius, Sven A1 - Ruprecht, Klemens A1 - Wildemann, Brigitte A1 - Kuempfel, Tania A1 - Ringelstein, Marius A1 - Geis, Christian A1 - Kleiter, Ingo A1 - Kleinschnitz, Christoph A1 - Berthele, Achim A1 - Brettschneider, Johannes A1 - Hellwig, Kerstin A1 - Hemmer, Bernhard A1 - Linker, Ralf A. A1 - Lauda, Florian A1 - Hayrettin, Christoph A. A1 - Tumani, Hayrettin A1 - Melms, Arthur A1 - Trebst, Corinna A1 - Stangel, Martin A1 - Marziniak, Martin A1 - Hoffmann, Frank A1 - Schippling, Sven A1 - Faiss, Jürgen H. A1 - Neuhaus, Oliver A1 - Ettrich, Barbara A1 - Zentner, Christian A1 - Guthke, Kersten A1 - Hofstadt-van Oy, Ulrich A1 - Reuss, Reinhard A1 - Pellkofer, Hannah A1 - Ziemann, Ulf A1 - Kern, Peter A1 - Wandinger, Klaus P. A1 - Bergh, Florian Then A1 - Boettcher, Tobias A1 - Langel, Stefan A1 - Liebetrau, Martin A1 - Rommer, Paulus S. A1 - Niehaus, Sabine A1 - Münch, Christoph A1 - Winkelmann, Alexander A1 - Zettl, Uwe K A1 - Metz, Imke A1 - Veauthier, Christian A1 - Sieb, Jörn P. A1 - Wilke, Christian A1 - Hartung, Hans P. A1 - Aktas, Orhan A1 - Paul, Friedemann T1 - Contrasting disease patterns in seropositive and seronegative neuromyelitis optica: A multicentre study of 175 patients JF - Journal of Neuroinflammation N2 - Background: The diagnostic and pathophysiological relevance of antibodies to aquaporin-4 (AQP4-Ab) in patients with neuromyelitis optica spectrum disorders (NMOSD) has been intensively studied. However, little is known so far about the clinical impact of AQP4-Ab seropositivity. Objective: To analyse systematically the clinical and paraclinical features associated with NMO spectrum disorders in Caucasians in a stratified fashion according to the patients' AQP4-Ab serostatus. Methods: Retrospective study of 175 Caucasian patients (AQP4-Ab positive in 78.3%). Results: Seropositive patients were found to be predominantly female (p < 0.0003), to more often have signs of co-existing autoimmunity (p < 0.00001), and to experience more severe clinical attacks. A visual acuity of <= 0.1 during acute optic neuritis (ON) attacks was more frequent among seropositives (p < 0.002). Similarly, motor symptoms were more common in seropositive patients, the median Medical Research Council scale (MRC) grade worse, and MRC grades <= 2 more frequent, in particular if patients met the 2006 revised criteria (p < 0.005, p < 0.006 and p < 0.01, respectively), the total spinal cord lesion load was higher (p < 0.006), and lesions >= 6 vertebral segments as well as entire spinal cord involvement more frequent (p < 0.003 and p < 0.043). By contrast, bilateral ON at onset was more common in seronegatives (p < 0.007), as was simultaneous ON and myelitis (p < 0.001); accordingly, the time to diagnosis of NMO was shorter in the seronegative group (p < 0.029). The course of disease was more often monophasic in seronegatives (p < 0.008). Seropositives and seronegatives did not differ significantly with regard to age at onset, time to relapse, annualized relapse rates, outcome from relapse (complete, partial, no recovery), annualized EDSS increase, mortality rate, supratentorial brain lesions, brainstem lesions, history of carcinoma, frequency of preceding infections, oligoclonal bands, or CSF pleocytosis. Both the time to relapse and the time to diagnosis was longer if the disease started with ON (p < 0.002 and p < 0.013). Motor symptoms or tetraparesis at first myelitis and > 1 myelitis attacks in the first year were identified as possible predictors of a worse outcome. KW - cerebrospinal-fluid KW - intractable hiccup KW - extensiv transverse myelitis KW - multiple sclerosis KW - anti-aquaporin-4 antibody KW - NMO-IGG KW - aquaporin-4 autoantibodies KW - immune-response KW - myasthenia gravis KW - immunoglobulin-G Y1 - 2012 U6 - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-133636 VL - 9 IS - 14 ER -