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Contrasting disease patterns in seropositive and seronegative neuromyelitis optica: A multicentre study of 175 patients

Please always quote using this URN: urn:nbn:de:bvb:20-opus-133636
  • 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-AbBackground: 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.show moreshow less

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Author: Sven Jarius, Klemens Ruprecht, Brigitte Wildemann, Tania Kuempfel, Marius Ringelstein, Christian Geis, Ingo Kleiter, Christoph Kleinschnitz, Achim Berthele, Johannes Brettschneider, Kerstin Hellwig, Bernhard Hemmer, Ralf A. Linker, Florian Lauda, Christoph A. Hayrettin, Hayrettin Tumani, Arthur Melms, Corinna Trebst, Martin Stangel, Martin Marziniak, Frank Hoffmann, Sven Schippling, Jürgen H. Faiss, Oliver Neuhaus, Barbara Ettrich, Christian Zentner, Kersten Guthke, Ulrich Hofstadt-van Oy, Reinhard Reuss, Hannah Pellkofer, Ulf Ziemann, Peter Kern, Klaus P. Wandinger, Florian Then Bergh, Tobias Boettcher, Stefan Langel, Martin Liebetrau, Paulus S. Rommer, Sabine Niehaus, Christoph Münch, Alexander Winkelmann, Uwe K Zettl, Imke Metz, Christian Veauthier, Jörn P. Sieb, Christian Wilke, Hans P. Hartung, Orhan Aktas, Friedemann Paul
URN:urn:nbn:de:bvb:20-opus-133636
Document Type:Journal article
Faculties:Medizinische Fakultät / Neurologische Klinik und Poliklinik
Language:English
Parent Title (English):Journal of Neuroinflammation
Year of Completion:2012
Volume:9
Issue:14
Source:Journal of Neuroinflammation 2012, 9:14. doi:10.1186/1742-2094-9-14
DOI:https://doi.org/10.1186/1742-2094-9-14
Dewey Decimal Classification:6 Technik, Medizin, angewandte Wissenschaften / 61 Medizin und Gesundheit / 616 Krankheiten
Tag:NMO-IGG; anti-aquaporin-4 antibody; aquaporin-4 autoantibodies; cerebrospinal-fluid; extensiv transverse myelitis; immune-response; immunoglobulin-G; intractable hiccup; multiple sclerosis; myasthenia gravis
Release Date:2017/01/13
Licence (German):License LogoCC BY: Creative-Commons-Lizenz: Namensnennung