eprintid: 21792 rev_number: 13 eprint_status: archive userid: 1589 dir: disk0/00/02/17/92 datestamp: 2016-08-22 11:32:13 lastmod: 2024-06-30 22:06:58 status_changed: 2016-08-22 11:32:13 type: article metadata_visibility: show creators_name: Jarius, Sven creators_name: Ruprecht, Klemens creators_name: Wildemann, Brigitte creators_name: Kümpfel, Tania creators_name: Ringelstein, Marius creators_name: Geis, Christian creators_name: Kleiter, Ingo creators_name: Kleinschnitz, Christoph creators_name: Berthele, Achim creators_name: Brettschneider, Johannes creators_name: Hellwig, Kerstin creators_name: Hemmer, Bernhard creators_name: Linker, Ralf A. creators_name: Lauda, Florian creators_name: Mayer, Christoph A. creators_name: Tumani, Hayrettin creators_name: Melms, Arthur creators_name: Trebst, Corinna creators_name: Stangel, Martin creators_name: Marziniak, Martin creators_name: Hoffmann, Frank creators_name: Schippling, Sven creators_name: Faiss, Jürgen H. creators_name: Neuhaus, Oliver creators_name: Ettrich, Barbara creators_name: Zentner, Christian creators_name: Guthke, Kersten creators_name: Hofstadt-van Oy, Ulrich creators_name: Reuss, Reinhard creators_name: Pellkofer, Hannah creators_name: Ziemann, Ulf creators_name: Kern, Peter creators_name: Wandinger, Klaus P. creators_name: Bergh, Florian creators_name: Boettcher, Tobias creators_name: Langel, Stefan creators_name: Liebetrau, Martin creators_name: Rommer, Paulus S. creators_name: Niehaus, Sabine creators_name: Münch, Christoph creators_name: Winkelmann, Alexander creators_name: Zettl, Uwe K. creators_name: Metz, Imke creators_name: Veauthier, Christian creators_name: Sieb, Jörn P. creators_name: Wilke, Christian creators_name: Hartung, Hans P. creators_name: Aktas, Orhan creators_name: Paul, Friedemann title: Contrasting disease patterns in seropositive and seronegative neuromyelitis optica: A multicentre study of 175 patients subjects: ddc-610 divisions: i-712000 divisions: i-911100 abstract: 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. Conclusion: This study provides an overview of the clinical and paraclinical features of NMOSD in Caucasians and demonstrates a number of distinct disease characteristics in seropositive and seronegative patients. date: 2012 publisher: BioMed Central id_scheme: DOI ppn_swb: 1659106311 own_urn: urn:nbn:de:bsz:16-heidok-217928 language: eng bibsort: JARIUSSVENCONTRASTIN2012 full_text_status: public publication: Journal of Neuroinflammation volume: 9 number: 14 place_of_pub: London pagerange: 1-17 issn: 1742-2094 citation: Jarius, Sven ; Ruprecht, Klemens ; Wildemann, Brigitte ; Kümpfel, Tania ; Ringelstein, Marius ; Geis, Christian ; Kleiter, Ingo ; Kleinschnitz, Christoph ; Berthele, Achim ; Brettschneider, Johannes ; Hellwig, Kerstin ; Hemmer, Bernhard ; Linker, Ralf A. ; Lauda, Florian ; Mayer, Christoph A. ; Tumani, Hayrettin ; Melms, Arthur ; Trebst, Corinna ; Stangel, Martin ; Marziniak, Martin ; Hoffmann, Frank ; Schippling, Sven ; Faiss, Jürgen H. ; Neuhaus, Oliver ; Ettrich, Barbara ; Zentner, Christian ; Guthke, Kersten ; Hofstadt-van Oy, Ulrich ; Reuss, Reinhard ; Pellkofer, Hannah ; Ziemann, Ulf ; Kern, Peter ; Wandinger, Klaus P. ; Bergh, Florian ; Boettcher, Tobias ; Langel, Stefan ; Liebetrau, Martin ; Rommer, Paulus S. ; Niehaus, Sabine ; Münch, Christoph ; Winkelmann, Alexander ; Zettl, Uwe K. ; Metz, Imke ; Veauthier, Christian ; Sieb, Jörn P. ; Wilke, Christian ; Hartung, Hans P. ; Aktas, Orhan ; Paul, Friedemann (2012) Contrasting disease patterns in seropositive and seronegative neuromyelitis optica: A multicentre study of 175 patients. Journal of Neuroinflammation, 9 (14). pp. 1-17. ISSN 1742-2094 document_url: https://archiv.ub.uni-heidelberg.de/volltextserver/21792/1/12974_2011_Article_503.pdf