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

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Item Type:Article
Title:Contrasting disease patterns in seropositive and seronegative neuromyelitis optica: A multicentre study of 175 patients
Creators Name:Jarius, S. and Ruprecht, K. and Wildemann, B. and Kuempfel, T. and Ringelstein, M. and Geis, C. and Kleiter, I. and Kleinschnitz, C. and Berthele, A. and Brettschneider, J. and Hellwig, K. and Hemmer, B. and Linker, R.A. and Lauda, F. and Mayer, C.A. and Tumani, H. and Melms, A. and Trebst, C. and Stangel, M. and Marziniak, M. and Hoffmann, F. and Schippling, S. and Faiss, J.H. and Neuhaus, O. and Ettrich, B. and Zentner, C. and Guthke, K. and Hofstadt-van Oy, U. and Reuss, R. and Pellkofer, H. and Ziemann, U. and Kern, P. and Wandinger, K.P. and Bergh, F.T. and Boettcher, T. and Langel, S. and Liebetrau, M. and Rommer, P.S. and Niehaus, S. and Muench, C. and Winkelmann, A. and Zettl U, U.K. and Metz, I. and Veauthier, C. and Sieb, J.P. and Wilke, C. and Hartung, H.P. and Aktas, O. and Paul, F.
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.
Keywords:Neuromyelitis Ooptica, Devic Disease, Devic Syndrome, Longitudinally Extensive Transverse Myelitis, Recurrent Optic Neuritis, NMO-IgG, Aquaporin-4 (AQP4) Antibody , Epidemiology, Clinical Features, Magnetic Resonance Imaging, Cerebrospinal Fluid
Source:Journal of Neuroinflammation
ISSN:1742-2094
Publisher:BioMed Central (U.K.)
Volume:9
Page Range:14
Date:19 January 2012
Official Publication:https://doi.org/10.1186/1742-2094-9-14
PubMed:View item in PubMed

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