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ADEM定义在MOG抗体相关疾病脑卒中之应用。

Application of the ADEM definition to cerebral attacks of MOG antibody-associated disease.

作者信息

Hooshmand Sara J, Vorasoot Nisa, Li Xiaoyang, Chen John J, Redenbaugh Vyanka, Banks Samantha A, Cacciaguerra Laura, Valencia-Sanchez Cristina, Lopez Chiriboga Sebastian, Sechi Elia, Tillema Jan-Mendelt, Tajfirouz Deena, Basso Michael, Hardy Todd A, Tisavipat Nanthaya, Guo Kai, Pittock Sean J, Flanagan Eoin P

机构信息

Departments of Neurology and Center for MS and Autoimmune Neurology, Mayo Clinic College of Medicine and Science, Rochester, MN, USA.

Laboratory Medicine and Pathology, Mayo Clinic College of Medicine and Science, Rochester, MN, USA.

出版信息

Mult Scler. 2025 Aug 19:13524585251359450. doi: 10.1177/13524585251359450.

Abstract

BACKGROUND

Acute disseminated encephalomyelitis (ADEM) is a demyelinating syndrome observed in myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) but there are limited data on application of this definition to its cerebral attacks or in adults with this disease.

METHODS

We analyzed patients from the Mayo Clinic MOGAD cohort ( = 263) with cerebral attacks. We assessed whether children (<18 years-old) and adults (⩾18 years-old) fulfilled the 2013 ADEM definition.

RESULTS

We identified 89 patients (children, 34; adults, 55) and 55/89 (62%) were of female sex. Acute magnetic resonance imaging (MRI) abnormalities included: poorly demarcated T2 lesions (white matter, 85 (96%); deep gray matter, 41 (46%); T1-hypointense lesions (42 (47%)). Forty-two patients did not fulfill the ADEM definition due to: monofocal syndrome, 9; or no encephalopathy, 33. The remaining 47 patients (53%: children, 27/34 (79%); adults, 20/55 (36%)) fulfilled the ADEM definition, and 14 had preceding attacks, and 34 had subsequent attacks relative to ADEM episode. The features in children and adults with ADEM were broadly comparable.

CONCLUSION

Applying the ADEM definition to MOGAD cerebral attacks highlighted that an update of the 2013 ADEM definition would be beneficial and could explicitly state its application to both adults and children and allow it to be applied to attacks at any time during the disease course.

摘要

背景

急性播散性脑脊髓炎(ADEM)是一种在髓鞘少突胶质细胞糖蛋白抗体相关疾病(MOGAD)中观察到的脱髓鞘综合征,但关于将该定义应用于其脑部发作或该疾病成人患者的数据有限。

方法

我们分析了梅奥诊所MOGAD队列中发生脑部发作的患者(n = 263)。我们评估了儿童(<18岁)和成人(≥18岁)是否符合2013年ADEM定义。

结果

我们确定了89例患者(儿童34例;成人55例),其中55/89(62%)为女性。急性磁共振成像(MRI)异常包括:边界不清的T2病变(白质,85例(96%);深部灰质,41例(46%));T1低信号病变(42例(47%))。42例患者不符合ADEM定义,原因如下:单灶综合征,9例;或无脑病,33例。其余47例患者(53%:儿童,27/34(79%);成人,20/55(36%))符合ADEM定义,其中14例在ADEM发作前有发作,34例在ADEM发作后有发作。儿童和成人ADEM患者的特征大致相当。

结论

将ADEM定义应用于MOGAD脑部发作突出表明,2013年ADEM定义的更新将是有益的,并且可以明确说明其对成人和儿童的应用,并允许其应用于疾病过程中的任何时间的发作。

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本文引用的文献

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