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舞蹈病:豆状核增大。

Sydenham Chorea: Putaminal Enlargement.

机构信息

Department of Pediatric Neurology, 64032Akdeniz University School of Medicine, Antalya, Turkey.

Department of Pediatric Neurology, 146991Ankara Children's Hospital Hematology-Oncology Research and Training Hospital, Ankara, Turkey.

出版信息

J Child Neurol. 2021 Jan;36(1):48-53. doi: 10.1177/0883073820951364. Epub 2020 Aug 27.

DOI:10.1177/0883073820951364
PMID:32851928
Abstract

BACKGROUND

Our aim in this study was to evaluate the efficacy of magnetic resonance imaging (MRI) studies in the detection of brain regions effected by Sydenham chorea and to determine whether they provided data regarding the pathogenesis of Sydenham chorea. To this end, we assessed basal ganglia structures in Sydenham chorea patients and control group by quantitative MRI volumetric analysis.

METHODS

Patients with a recent onset of chorea and control subjects matched for age and gender were included in the study. Medical history, laboratory tests, and physical and neurologic examinations were reviewed. All MRIs were considered within normal limits. High-resolution T1-weighted 3D magnetization-prepared rapid acquisition of gradient echo scans were used for quantitative volumetric assessment of the brain via the "volBrain" method.

RESULTS

Twenty-four subjects with Sydenham chorea (16 girls and 8 boys, aged between 7 and 16 years) and 35 control subjects were evaluated. Mean age was 11.25 ± 2.89 years for Sydenham chorea patients and 10.58 ± 2.53 years for the controls. No significant difference was found relative to globus pallidus, caudate, and thalamic volumes between patients with Sydenham chorea and controls. The relative mean total, left, and right putamen volumes were significantly larger in patients with Sydenham chorea compared to controls ( = .003, = .018, = .001, respectively).

CONCLUSION

Selective neuroanatomic differences in putamen among other basal ganglia structures and significant increases in size are consistent with a hypothesis of a cross-reactive antibody-mediated inflammation of the putamen as being the pathophysiologic mechanism for this disorder.

摘要

背景

本研究旨在评估磁共振成像(MRI)在检测被舞蹈病影响的大脑区域方面的功效,并确定其是否提供了有关舞蹈病发病机制的信息。为此,我们通过定量 MRI 容积分析评估了舞蹈病患者和对照组的基底节结构。

方法

本研究纳入了近期发病的舞蹈病患者和年龄、性别相匹配的对照组患者。回顾了病史、实验室检查以及体格检查和神经系统检查。所有 MRI 均被认为在正常范围内。采用高分辨率 T1 加权 3D 磁化准备快速获取梯度回波扫描,通过“volBrain”方法对大脑进行定量容积评估。

结果

共评估了 24 例舞蹈病患者(16 名女孩和 8 名男孩,年龄在 7 至 16 岁之间)和 35 名对照组患者。舞蹈病患者的平均年龄为 11.25 ± 2.89 岁,对照组为 10.58 ± 2.53 岁。舞蹈病患者与对照组之间,苍白球、尾状核和丘脑体积无显著差异。与对照组相比,舞蹈病患者的总、左、右壳核的相对平均体积明显增大(分别为 =.003、 =.018、 =.001)。

结论

壳核与其他基底节结构之间存在选择性的神经解剖差异,且体积显著增大,这与壳核存在交叉反应性抗体介导的炎症反应这一病理生理机制假说一致,这种炎症反应可能是导致该疾病的原因。

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J Child Neurol. 2021 Jan;36(1):48-53. doi: 10.1177/0883073820951364. Epub 2020 Aug 27.
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