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香港华人特发性炎性脱髓鞘疾病中的视神经脊髓炎免疫球蛋白G

Neuromyelitis optica-IgG in idiopathic inflammatory demyelinating disorders amongst Hong Kong Chinese.

作者信息

Chan K H, Ramsden D B, Yu Y L, Kwok K H H, Chu A C Y, Ho P W L, Kwan J S C, Lee R, Lim E, Kung M H W, Ho S L

机构信息

University Department of Medicine, Research Center for Heart, Brain, Hormone and Healthy Aging, LKS Faculty of Medicine, The University of Hong Kong, Hong Kong, China.

出版信息

Eur J Neurol. 2009 Mar;16(3):310-6. doi: 10.1111/j.1468-1331.2008.02376.x.

DOI:10.1111/j.1468-1331.2008.02376.x
PMID:19138340
Abstract

BACKGROUND

Idiopathic inflammatory demyelinating disorders (IIDD) affect the central nervous system. In classical multiple sclerosis (CMS), brain, optic nerves [optic neuritis (ON)] and spinal cord [acute transverse myelitis (ATM)] are affected. In neuromyelitis optica (NMO), optic nerves and spinal cord are predominantly affected. NMO-IgG, an autoantibody targeting aquaporin-4, is a marker for NMO. We studied the frequency and clinical relevance of NMO-IgG seropositivity in IIDD patients.

METHODS

Neuromyelitis optica-IgG was detected by indirect immunofluorescence using primate cerebellum.

RESULTS

Neuromyelitis optica-IgG was detected in six of 10 NMO patients (60%), six of 10 idiopathic relapsing transverse myelitis (IRTM) patients (60%), two of nine idiopathic relapsing ON patients (22%), one of 11 patients (9%) having single ON attack, one of 30 CMS patients (3%), and none of patients having single ATM attack or controls. Comparing NMO-IgG seropositive (n = 12) with NMO-IgG seronegative (n = 8) patients having NMO or IRTM, NMO-IgG seropositivity was associated with a higher relapse rate in first 2 years, 1.5 and 0.6 attacks/year for seropositive and seronegative groups respectively (P = 0.006), and non-significant trend towards more severe ON and myelitis with poorer clinical outcome.

CONCLUSION

Neuromyelitis optica -IgG facilitates diagnosis of NMO spectrum disorders. NMO-IgG seropositivity is associated with higher relapse rate in first 2 years.

摘要

背景

特发性炎性脱髓鞘疾病(IIDD)累及中枢神经系统。在经典型多发性硬化(CMS)中,脑、视神经[视神经炎(ON)]和脊髓[急性横贯性脊髓炎(ATM)]均会受累。在视神经脊髓炎(NMO)中,主要累及视神经和脊髓。NMO-IgG是一种靶向水通道蛋白4的自身抗体,是NMO的标志物。我们研究了IIDD患者中NMO-IgG血清阳性的频率及其临床相关性。

方法

采用灵长类动物小脑通过间接免疫荧光法检测视神经脊髓炎-IgG。

结果

10例NMO患者中有6例(60%)检测到视神经脊髓炎-IgG,10例特发性复发性横贯性脊髓炎(IRTM)患者中有6例(60%),9例特发性复发性视神经炎患者中有2例(22%),11例单次发作视神经炎患者中有1例(9%),30例CMS患者中有1例(3%),而单次发作急性横贯性脊髓炎患者或对照组中均未检测到。将患有NMO或IRTM的NMO-IgG血清阳性患者(n = 12)与血清阴性患者(n = 8)进行比较,NMO-IgG血清阳性与前2年较高的复发率相关,血清阳性组和血清阴性组的年复发率分别为1.5次和0.6次发作/年(P = 0.006),并且在视神经炎和脊髓炎严重程度更高、临床结局更差方面存在不显著的趋势。

结论

视神经脊髓炎-IgG有助于视神经脊髓炎谱系疾病的诊断。NMO-IgG血清阳性与前2年较高的复发率相关。

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