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具有复发临床病程的特发性急性横贯性脊髓炎中的抗水通道蛋白4(AQP4)抗体:阳性率及其对预后的影响

Anti-AQP(4) antibody in idiopathic acute transverse myelitis with recurrent clinical course: frequency of positivity and influence in prognosis.

作者信息

Alvarenga Marina Papais, Alvarenga Regina Maria Papais, Alvarenga Marcos Papais, Santos Adriano Miranda, Thuler Luiz Claudio Santos

机构信息

Pós graduação em Neurologia, Universidade Federal do Estado do Rio de Janeiro, Brazil.

出版信息

J Spinal Cord Med. 2012 Jul;35(4):251-5. doi: 10.1179/2045772312Y.0000000019.

Abstract

BACKGROUND

The anti-aquaporin4 (anti-AQP4) antibody is specific for neuromyelitis optica (NMO), but is also found in limited forms. The presence of this antibody in acute transverse myelitis (ATM) has been associated with recurrence and conversion to NMO, but the influence on disability has not yet been described.

OBJECTIVE

To describe the frequency of anti-AQP4 in ATM and analyze the influence in long-term prognosis.

DESIGN

Cross-sectional and retrospective study.

METHODS

Consecutive ATM cases in a multiple sclerosis center in Rio de Janeiro, Brazil, from 2000 through 2009 were reviewed. Recurrent cases tested for anti-AQP4 were selected. ATM with magnetic resonance imaging spinal cord lesions extending over three or more vertebral segments was classified as longitudinally extensive transverse myelitis (LETM); Kurtzke scale was applied at last evaluation.

OUTCOME MEASURES

Frequency of anti-AQP4; severity of spinal cord dysfunction at last follow-up.

RESULTS

Twenty six patients (21 female:5 male; 17 white:9 African descent) were studied. The first ATM occurred at 38.04 ± 12.7 years. The interval between the first and the second ATM was eight months (1-150) and the number of ATM varied from two to seven. After 40.5 months (12-192) of disease, the median Expanded Disability Status Scale (EDSS) score was three (0-9). Anti-AQP4 antibody was positive in 26.9%. LETM was found in 65.4%. LETM presented later onset, higher disability and higher positivity to anti-AQP4 (LETM 41.2% versus no-LETM 0%, P = 0.024). Dysfunction at long-term follow-up was similar in anti-AQP4 positive and negative cases.

CONCLUSION

The frequency of anti-AQP4 in recurrent ATM was 26.9%, increasing to 41.2% among LETM. Presence of the antibody had no influence on morbidity.

摘要

背景

抗水通道蛋白4(anti-AQP4)抗体是视神经脊髓炎(NMO)的特异性抗体,但也存在于有限的形式中。该抗体在急性横贯性脊髓炎(ATM)中的存在与复发及转化为NMO有关,但对残疾的影响尚未见描述。

目的

描述ATM中抗AQP4的频率并分析其对长期预后的影响。

设计

横断面及回顾性研究。

方法

回顾了2000年至2009年巴西里约热内卢一个多发性硬化中心的连续ATM病例。选择检测了抗AQP4的复发病例。脊髓磁共振成像显示病变累及三个或更多椎体节段的ATM被分类为长节段横贯性脊髓炎(LETM);最后一次评估时应用Kurtzke量表。

观察指标

抗AQP4的频率;最后一次随访时脊髓功能障碍的严重程度。

结果

研究了26例患者(21例女性:5例男性;17例白人:9例非洲裔)。首次ATM发生于38.04±12.7岁。首次与第二次ATM之间的间隔为8个月(1 - 150个月),ATM发作次数从2次到7次不等。患病40.5个月(12 - 192个月)后,扩展残疾状态量表(EDSS)评分中位数为3分(0 - 9分)。抗AQP4抗体阳性率为26.9%。LETM见于65.4%的患者。LETM起病较晚,残疾程度较高,抗AQP4阳性率较高(LETM为41.2%,非LETM为0%,P = 0.024)。抗AQP4阳性和阴性病例在长期随访中的功能障碍相似。

结论

复发性ATM中抗AQP4的频率为26.9%,在LETM中增至41.2%。该抗体的存在对发病率无影响。

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