Cerebrospinal fluid findings in aquaporin-4 antibody positive neuromyelitis optica: Results from 211 lumbar punctures

被引:271
作者
Jarius, S. [1 ]
Paul, F. [2 ]
Franciotta, D. [3 ]
Ruprecht, K. [4 ]
Ringelstein, M. [5 ]
Bergamaschi, R. [3 ]
Rommer, P. [6 ]
Kleiter, I. [7 ]
Stich, O. [8 ]
Reuss, R. [9 ]
Rauer, S. [8 ]
Zettl, U. K. [10 ]
Wandinger, K. P. [11 ,12 ]
Melms, A. [13 ]
Aktas, O. [5 ]
Kristoferitsch, W. [14 ]
Wildemann, B. [1 ]
机构
[1] Heidelberg Univ, Dept Neurol, Div Mol Neuroimmunol, Heidelberg, Germany
[2] Charite, NeuroCure Clin Res Ctr, D-13353 Berlin, Germany
[3] Natl Neurol Inst C Mondino, IRCCS, Pavia, Italy
[4] Charite, Dept Neurol, D-13353 Berlin, Germany
[5] Univ Dusseldorf, Dept Neurol, D-4000 Dusseldorf, Germany
[6] Med Univ Vienna, Dept Neurol, Vienna, Austria
[7] Univ Med Ctr Regensburg, Dept Neurol, Regensburg, Germany
[8] Univ Freiburg, Dept Neurol, D-7800 Freiburg, Germany
[9] Hosp Hohe Warte, Dept Neurol, Bayreuth, Germany
[10] Univ Rostock, Dept Neurol, Rostock, Germany
[11] Euroimmun, Inst Expt Immunol, Lubeck, Germany
[12] Univ Med Ctr Eppendorf, Inst Neuroimmunol & Clin MS Res, Hamburg, Germany
[13] Univ Tubingen, Dept Neurol, D-7400 Tubingen, Germany
[14] Sozialmed Zentrum Ost Donauspital, Dept Neurol, Vienna, Austria
关键词
Neuromyelitis optica (Devic disease); Longitudinally extensive transverse myelitis; Recurrent optic neuritis; Cerebrospinal fluid; Lumbar puncture; NMO-IgG; Antibody to aquaporin-4; Oligoclonal bands; IgG; IgM; IgA; Albumin Total protein; Lactate; EXTENSIVE TRANSVERSE MYELITIS; MULTIPLE-SCLEROSIS; NEUROLOGICAL DISEASES; IGG; DIAGNOSIS; CSF; IMMUNOGLOBULIN; PATHOGENESIS; MECHANISMS; RELEVANCE;
D O I
10.1016/j.jns.2011.03.038
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Background: Neuromyelitis optica (NMO, Devic disease) is a severely disabling autoimmune disorder of the CNS, which was considered a subtype of multiple sclerosis (MS) for many decades. Recently, however, highly specific serum autoantibodies (termed NMO-IgG or AQP4-Ab) have been discovered in a subset (60-80%) of patients with NMO. These antibodies were subsequently shown to be directly involved in the pathogenesis of the condition. AQP4-Ab positive NMO is now considered an immunopathogenetically distinct disease in its own right. However, to date little is known about the cerebrospinal fluid (CSF) in AQP4-Ab positive NMO. Objective: To describe systematically the CSF profile of AQP4-Ab positive patients with NMO or its formes frustes, longitudinally extensive myelitis and optic neuritis. Material and methods: Cytological and protein biochemical results from 211 lumbar punctures in 89 AQP4-Ab positive patients of mostly Caucasian origin with neuromyelitis optica spectrum disorders (NMOSD) were analysed retrospectively. Results: CSF-restricted oligodonal IgG bands, a hallmark of MS, were absent in most patients. If present. intrathecal IgG (and, more rarely, IgM) synthesis was low, transient, and, importantly, restricted to acute relapses. CSF pleocytosis was present in around 50% of samples, was mainly mild (median, 19 cells/ill; range 6-380), and frequently included neutrophils, eosinophils, activated lymphocytes, and/or plasma cells. Albumin CSF/serum ratios, total protein and CSF L-lactate levels correlated significantly with disease activity as well as with the length of the spinal cord lesions in patients with acute myelitis. CSF findings differed significantly between patients with acute myelitis and patients with acute optic neuritis at the time of LP. Pleocytosis and blood CSF barrier dysfunction were also present during remission in some patients, possibly indicating sustained subclinical disease activity. Conclusion: AQP4-Ab positive NMOSD is characterized by CSF features that are distinct from those in MS. Our findings are important for the differential diagnosis of MS and NMOSD and add to our understanding of the immunopathogenesis of this devastating condition. (C) 2011 Elsevier B.V. All rights reserved.
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页码:82 / 90
页数:9
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