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

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 s
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
show moreshow less

Download full text files

Export metadata

  • Export Bibtex
  • Export RIS

Additional Services

    Share in Twitter Search Google Scholar
Author:Sven Jarius, Klemens Ruprecht, Brigitte Wildemann, Tania Kümpfel, Marius Ringelstein, Christian Geis, Ingo Kleiter, Christoph Kleinschnitz, Achim Berthele, Johannes Brettschneider, Kerstin Hellwig, Bernhard Hemmer, Ralf A. Linker, Florian Lauda, Christoph Mayer, 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 T. Bergh, Tobias Böttcher, Stefan Langel, Martin Liebetrau, Paulus S. Rommer, Sabine Niehaus, Christoph Münch, Alexander Winkelmann, Uwe Klaus Zettl, Imke Metz, Christian Veauthier, Jörn P. Sieb, Christian Wilke, Hans P. Hartung, Orhan Aktas, Friedemann Paul
Parent Title (English):Journal of neuroinflammation
Publisher:BioMed Central
Place of publication:London
Document Type:Article
Date of Publication (online):2012/01/19
Date of first Publication:2012/01/19
Publishing Institution:Universitätsbibliothek Johann Christian Senckenberg
Release Date:2012/10/12
Tag:Devic disease; Devic syndrome; NMO-IgG; Neuromyelitis optica; aquaporin-4 (AQP4) antibody; cerebrospinal fluid; clinical features; epidemiology; longitudinally extensive transverse myelitis; magnetic resonance imaging; recurrent optic neuritis
© 2012 Jarius et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. 
HeBIS PPN:358310466
Dewey Decimal Classification:610 Medizin und Gesundheit
Licence (German):License LogoCreative Commons - Namensnennung 2.0

$Rev: 11761 $