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

被引：584

作者：

Jarius, Sven ^{[2
]}

Ruprecht, Klemens ^{[1
]}

Wildemann, Brigitte ^{[2
]}

Kuempfel, Tania ^{[3
]}

Ringelstein, Marius ^{[4
]}

Geis, Christian ^{[5
]}

Kleiter, Ingo ^{[6
,7
]}

Kleinschnitz, Christoph ^{[5
]}

Berthele, Achim ^{[8
]}

Brettschneider, Johannes ^{[9
]}

Hellwig, Kerstin ^{[7
]}

Hemmer, Bernhard ^{[8
]}

Linker, Ralf A. ^{[7
,10
]}

Lauda, Florian ^{[9
]}

Mayer, Christoph A. ^{[11
]}

Tumani, Hayrettin ^{[9
]}

Melms, Arthur ^{[12
]}

Trebst, Corinna ^{[13
]}

Stangel, Martin ^{[13
]}

Marziniak, Martin ^{[14
]}

Hoffmann, Frank ^{[15
]}

Schippling, Sven ^{[16
,17
]}

Faiss, Juergen H. ^{[18
]}

Neuhaus, Oliver ^{[19
]}

Ettrich, Barbara ^{[20
]}

Zentner, Christian ^{[15
]}

Guthke, Kersten ^{[21
]}

Hofstadt-van Oy, Ulrich ^{[22
]}

Reuss, Reinhard ^{[22
]}

Pellkofer, Hannah ^{[3
]}

Ziemann, Ulf ^{[11
]}

Kern, Peter ^{[18
]}

Wandinger, Klaus P. ^{[16
,17
,23
]}

Bergh, Florian Then ^{[20
]}

Boettcher, Tobias ^{[24
]}

Langel, Stefan ^{[25
]}

Liebetrau, Martin ^{[26
]}

Rommer, Paulus S. ^{[27
]}

Niehaus, Sabine ^{[28
]}

Muench, Christoph ^{[1
]}

Winkelmann, Alexander ^{[27
]}

Zettl U, Uwe K. ^{[27
]}

Metz, Imke ^{[29
]}

Veauthier, Christian ^{[30
]}

Sieb, Joern P. ^{[30
]}

Wilke, Christian ^{[31
]}

Hartung, Hans P. ^{[4
]}

Aktas, Orhan ^{[4
]}

Paul, Friedemann ^{[1
]}

机构：

[1] Charite, Dept Neurol, D-13353 Berlin, Germany

[2] Heidelberg Univ, Dept Neurol, Div Mol Neuroimmunol, Heidelberg, Germany

[3] Univ Munich, Inst Clin Neuroimmunol, Munich, Germany

[4] Univ Dusseldorf, Dept Neurol, Dusseldorf, Germany

[5] Univ Wurzburg, Dept Neurol, D-8700 Wurzburg, Germany

[6] Univ Regensburg, Dept Neurol, Regensburg, Germany

[7] Ruhr Univ Bochum, St Josef Hosp, Dept Neurol, Bochum, Germany

[8] Tech Univ Munich, Klinikum Rechts Isar, Dept Neurol, D-8000 Munich, Germany

[9] Univ Ulm, Dept Neurol, D-7900 Ulm, Germany

[10] Univ Erlangen Nurnberg, Dept Neurol, Erlangen, Germany

[11] Goethe Univ Frankfurt, Dept Neurol, Frankfurt, Germany

[12] Univ Tubingen, Dept Neurol, D-7400 Tubingen, Germany

[13] Hannover Med Sch, Dept Neurol, D-3000 Hannover, Germany

[14] Univ Munster, Dept Neurol, D-4400 Munster, Germany

[15] Hosp Martha Maria Halle, Dept Neurol, Halle, Germany

[16] Univ Med Ctr, Dept Neurol, Hamburg, Germany

[17] Univ Med Ctr, Inst Neuroimmunol & Clin Multiple Sclerosis Res, Hamburg, Germany

[18] Asklepios Hosp Teupitz, Dept Neurol, Teupitz, Germany

[19] Kliniken Landkreis Sigmaringen GmbH, Dept Neurol, Sigmaringen, Germany

[20] Univ Leipzig, Dept Neurol, Leipzig, Germany

[21] Klinikum Gorlitz, Dept Neurol, Gorlitz, Germany

[22] Klinikum Bayreuth, Dept Neurol, Bayreuth, Germany

[23] Euroimmun Lubeck, Inst Expt Neuroimmunol, Lubeck, Germany

[24] Dietrich Bonhoeffer Klinikum Neubrandenburg, Dept Neurol, Neubrandenburg, Germany

[25] Rheinhessen Fachklin Alzey, Dept Neurol, Alzey, Germany

[26] Dr Horst Schmidt Hosp Wiesbaden, Dept Neurol, Wiesbaden, Germany

[27] Univ Rostock, Dept Neurol, Rostock, Germany

[28] Klinikum Dortmund, Dept Neurol, Dortmund, Germany

[29] Univ Gottingen, Dept Neuropathol, Gottingen, Germany

[30] Hanse Klinikum Stralsund, Dept Neurol, Stralsund, Germany

[31] Helios Vogtland Klinikum Plauen, Dept Neurol, Plauen, Germany

来源：

JOURNAL OF NEUROINFLAMMATION | 2012年 / 9卷

关键词：

MULTIPLE-SCLEROSIS; ANTI-AQUAPORIN-4; ANTIBODY; AQUAPORIN-4; AUTOANTIBODIES; CEREBROSPINAL-FLUID; IMMUNE-RESPONSE; NMO-IGG; INTRACTABLE HICCUP; IMMUNOGLOBULIN-G; NATURAL-HISTORY; FOLLOW-UP;

D O I：

10.1186/1742-2094-9-14

中图分类号：

R392 [医学免疫学]; Q939.91 [免疫学];

学科分类号：

100102 ;

摘要：

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.

引用

页数：17

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