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多发性硬化症非交通性脊髓空洞症病例系列的 6 年随访。

Six-year follow-up of a case series with non-communicating syringomyelia in multiple sclerosis.

机构信息

Department of Neurology, University Hospital Basel, Switzerland.

Division of Diagnostic and Interventional Neuroradiology, Department of Radiology and Nuclear Medicine, University Hospital Basel, Switzerland.

出版信息

Eur J Neurol. 2013 Mar;20(3):578-583. doi: 10.1111/ene.12052. Epub 2012 Dec 17.

Abstract

BACKGROUND

Non-communicating syringomyelia (NCS) has occasionally been described in case reports and small case series as an incidental finding of spinal cord (SC) pathology in patients with multiple sclerosis (MS), but only little is known on the clinical course and progression of NCS, and in more general terms on the prognosis of patients with MS and NCS.

METHODS

Nine patients with MS with known NCS at baseline and a control group of 18 age-, sex- and disease course-matched patients with MS without NCS were recruited for a follow-up visit after 6 years. All 27 patients underwent clinical examination and brain magnetic resonance imaging (MRI), and 8/9 patients with NCS were additionally studied with MRI of the SC. MRI data were analysed for changes in length and maximal cross-sectional area of the NCS, lesion volumes of the brain and cord as well as for volumetric metrics of the whole brain (using SIENAX), the cerebellum and medulla oblongata (using ECCET).

RESULTS

NCS did not significantly change in size when corrected for multiple comparisons. The clinical data (annual relapse rate, EDSS and disease duration) and MRI metrics (T2 and T1 lesion load; whole brain, cerebellar and medulla oblongata volumes as well as their percentage volume change per year) did not significantly differ between patients with MS with or without NCS.

CONCLUSION

The stable findings regarding size and shape of the syrinx and lack of distinguishing MRI and clinical features support the assumption that NCS is not defining a prognostically or pathogenetically distinct subgroup of patients with MS.

摘要

背景

非交通性脊髓空洞症(NCS)偶尔在病例报告和小病例系列中被描述为多发性硬化症(MS)患者脊髓(SC)病理学的偶发发现,但对 NCS 的临床病程和进展知之甚少,更广泛地说,对 MS 和 NCS 患者的预后知之甚少。

方法

在基线时已知有 NCS 的 9 例 MS 患者和 18 例年龄、性别和病程匹配的无 NCS 的 MS 对照患者被招募进行 6 年后的随访。所有 27 例患者均接受了临床检查和脑磁共振成像(MRI),8/9 例 NCS 患者还接受了 SC 的 MRI 检查。分析 MRI 数据以了解 NCS 的长度和最大横截面积、脑和脊髓病变体积以及全脑(使用 SIENAX)、小脑和延髓的容积指标(使用 ECCET)的变化。

结果

在进行多次比较校正后,NCS 的大小没有显著变化。临床数据(年复发率、EDSS 和病程)和 MRI 指标(T2 和 T1 病变负荷;全脑、小脑和延髓体积以及它们的每年体积变化百分比)在有或没有 NCS 的 MS 患者之间没有显著差异。

结论

关于空洞大小和形状的稳定发现以及缺乏鉴别 MRI 和临床特征支持这样的假设,即 NCS 并不是定义 MS 患者具有预后或发病机制上不同亚组的特征。

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