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小儿发病后天脑脱髓鞘疾病患者小脑生长受损。

Impaired growth of the cerebellum in pediatric-onset acquired CNS demyelinating disease.

机构信息

McConnell Brain Imaging Centre, Montreal Neurological Institute, McGill University, Montreal, QC, Canada.

Division of Neurology, Children's Hospital of Philadelphia, University of Pennsylvania, Philadelphia, PA, USA.

出版信息

Mult Scler. 2016 Sep;22(10):1266-78. doi: 10.1177/1352458515615224. Epub 2015 Nov 9.

Abstract

BACKGROUND

Acquired demyelinating syndromes (ADS) have the potential to negatively impact cerebellar growth, given the proclivity for infratentorial lesions in pediatric-onset multiple sclerosis (MS) and ADS.

OBJECTIVE

To investigate cerebellar growth longitudinally in pediatric ADS.

METHODS

Cerebellar volumes from 472 magnetic resonance imaging (MRI) scans of 98 patients with monophasic ADS (monoADS), monophasic acute disseminated encephalomyelitis (ADEM), and MS (49 girls; mean age: 11.4 years at first scan, mean follow-up: 3.1 years) imaged serially from onset and 897 MRI scans of 418 healthy children (223 girls, mean age: 11.3 years, mean follow-up: 2.9 years) were segmented automatically, analyzed with mixed-effect models, and compared with cerebral volume.

RESULTS

Cerebellar developmental trajectories followed a U-shaped curve, showing larger volumes in boys (p < 0.001). Cerebellar volumes in all three patient groups failed to reach age-expected trajectories, leading to significantly smaller volumes, notably in the posterior lobes. Cerebellar volume reductions were of a similar magnitude to cerebral volume reductions. Cerebellar white matter volume declined in MS and ADEM patients over time, while in monoADS patients it remained similar to controls. Cerebellar volumes did not correlate either with lesion volumes at onset or with physical disability.

CONCLUSION

MonoADS, ADEM, and MS in childhood lead to impaired age-expected growth of the cerebellum.

摘要

背景

鉴于小儿发病多发性硬化症(MS)和获得性脱髓鞘综合征(ADS)中倾向于发生小脑幕下病变,获得性脱髓鞘综合征(ADS)有可能对小脑生长产生负面影响。

目的

研究小儿获得性脱髓鞘综合征(ADS)的小脑纵向生长情况。

方法

对 98 例单相获得性脱髓鞘综合征(monoADS)、单相急性播散性脑脊髓炎(ADEM)和 MS 患者的 472 次磁共振成像(MRI)扫描(49 名女孩;首次扫描时的平均年龄为 11.4 岁,平均随访时间为 3.1 年)和 418 例健康儿童(223 名女孩,平均年龄为 11.3 岁,平均随访时间为 2.9 年)的 897 次 MRI 扫描的小脑体积进行自动分割,采用混合效应模型进行分析,并与大脑体积进行比较。

结果

小脑的发育轨迹呈 U 型,男孩的小脑体积较大(p<0.001)。所有三组患者的小脑体积均未达到与年龄相关的轨迹,导致小脑体积明显较小,尤其是在后叶。小脑体积减少与大脑体积减少的幅度相似。MS 和 ADEM 患者的小脑白质体积随时间推移而减少,而 monoADS 患者的小脑白质体积与对照组相似。小脑体积与发病时的病变体积或身体残疾均无相关性。

结论

儿童期的 monoADS、ADEM 和 MS 导致小脑的预期生长受损。

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