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MOG抗体在获得性脱髓鞘综合征患儿中的预后相关性。

Prognostic relevance of MOG antibodies in children with an acquired demyelinating syndrome.

作者信息

Hennes Eva-Maria, Baumann Matthias, Schanda Kathrin, Anlar Banu, Bajer-Kornek Barbara, Blaschek Astrid, Brantner-Inthaler Sigrid, Diepold Katharina, Eisenkölbl Astrid, Gotwald Thaddäus, Kuchukhidze Georgi, Gruber-Sedlmayr Ursula, Häusler Martin, Höftberger Romana, Karenfort Michael, Klein Andrea, Koch Johannes, Kraus Verena, Lechner Christian, Leiz Steffen, Leypoldt Frank, Mader Simone, Marquard Klaus, Poggenburg Imke, Pohl Daniela, Pritsch Martin, Raucherzauner Markus, Schimmel Mareike, Thiels Charlotte, Tibussek Daniel, Vieker Silvia, Zeches Carolin, Berger Thomas, Reindl Markus, Rostásy Kevin

机构信息

Author affiliations are provided at the end of the article.

出版信息

Neurology. 2017 Aug 29;89(9):900-908. doi: 10.1212/WNL.0000000000004312. Epub 2017 Aug 2.

Abstract

OBJECTIVE

To assess the prognostic value of MOG antibodies (abs) in the differential diagnosis of acquired demyelinating syndromes (ADS).

METHODS

Clinical course, MRI, MOG-abs, AQP4-abs, and CSF cells and oligoclonal bands (OCB) in children with ADS and 24 months of follow-up were reviewed in this observational prospective multicenter hospital-based study.

RESULTS

Two hundred ten children with ADS were included and diagnosed with acute disseminated encephalomyelitis (ADEM) (n = 60), neuromyelitis optica spectrum disorder (NMOSD) (n = 12), clinically isolated syndrome (CIS) (n = 101), and multiple sclerosis (MS) (n = 37) after the first episode. MOG-abs were predominantly found in ADEM (57%) and less frequently in NMOSD (25%), CIS (25%), or MS (8%). Increased MOG-ab titers were associated with younger age ( = 0.0001), diagnosis of ADEM ( = 0.005), increased CSF cell counts ( = 0.011), and negative OCB ( = 0.012). At 24-month follow-up, 96 children had no further relapses. Thirty-five children developed recurrent non-MS episodes (63% MOG-, 17% AQP4-abs at onset). Seventy-nine children developed MS (4% MOG-abs at onset). Recurrent non-MS episodes were associated with high MOG-ab titers ( = 0.0003) and older age at onset ( = 0.024). MS was predicted by MS-like MRI ( < 0.0001) and OCB ( = 0.007). An MOG-ab cutoff titer ≥1:1,280 predicted a non-MS course with a sensitivity of 47% and a specificity of 100% and a recurrent non-MS course with a sensitivity of 46% and a specificity of 86%.

CONCLUSIONS

Our results show that the presence of MOG-abs strongly depends on the age at disease onset and that high MOG-ab titers were associated with a recurrent non-MS disease course.

摘要

目的

评估髓鞘少突胶质细胞糖蛋白(MOG)抗体在获得性脱髓鞘综合征(ADS)鉴别诊断中的预后价值。

方法

在这项基于医院的前瞻性多中心观察性研究中,回顾了ADS患儿的临床病程、磁共振成像(MRI)、MOG抗体、水通道蛋白4抗体(AQP4抗体)以及脑脊液细胞和寡克隆带(OCB)情况,并进行了24个月的随访。

结果

纳入210例ADS患儿,首次发作后诊断为急性播散性脑脊髓炎(ADEM)(n = 60)、视神经脊髓炎谱系障碍(NMOSD)(n = 12)、临床孤立综合征(CIS)(n = 101)和多发性硬化(MS)(n = 37)。MOG抗体主要见于ADEM(57%),在NMOSD(25%)、CIS(25%)或MS(8%)中较少见。MOG抗体滴度升高与年龄较小(P = 0.0001)、ADEM诊断(P = 0.005)、脑脊液细胞计数增加(P = 0.011)和OCB阴性(P = 0.012)相关。在24个月随访时,96例患儿无进一步复发。35例患儿出现复发性非MS发作(发病时63%为MOG抗体阳性,17%为AQP4抗体阳性)。79例患儿发展为MS(发病时4%为MOG抗体阳性)。复发性非MS发作与高MOG抗体滴度(P = 0.0003)和发病时年龄较大(P = 0.024)相关。MS由类似MS的MRI表现(P < 0.0001)和OCB(P = 0.007)预测。MOG抗体临界滴度≥1:1280预测非MS病程的敏感性为47%,特异性为100%;预测复发性非MS病程的敏感性为46%,特异性为86%。

结论

我们的结果表明,MOG抗体的存在强烈依赖于疾病发病年龄,且高MOG抗体滴度与复发性非MS病程相关。

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