Brain injury unmasking Ehlers-Danlos syndromes after trauma: the fiber print.
Brain injury unmasking Ehlers-Danlos syndromes after trauma: the fiber print.
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DOI：
10.1186/s13023-016-0428-9
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发表时间：
2016-04-22
影响因子：
3.7
通讯作者：
Zeitoun JD
中科院分区：
文献类型：
--
作者：
Hamonet C;Frédy D;Lefèvre JH;Bourgeois-Gironde S;Zeitoun JD
物理创伤在Ehlers-Danlos综合征（EDS）症状发作中的作用从未被描述过。我们试图寻找和描述脑病变EDS患者也有个人的历史，身体创伤。我们系统地进行了脑磁共振成像在第一个队列的患者与高流动性类型的EDS，描述了他们的疾病发作或恶化后的身体创伤。意外但一致的发现被认为与报告的创伤有关，导致对所有后续具有类似症状的患者进行脑成像，无论是否有创伤史，并通过主动询问寻找既往创伤。招募并分析了59例患者，其中53例（89.8%）为女性。总体而言，26例（44.1%）报告了身体创伤的个人病史。确定了6个与皮质下病变有关并影响白色物质束的体征。包括网状结构、两个豆状核、胼胝体和弓状束的病变。36例患者（61.0%）至少有6种影像学体征中的5种。在18岁之前发生创伤的情况下，患者的网状结构病变明显更多（100% vs. 50%; p = 0.0035）。EDS患者，过度活动型，被发现有一致的和特定的脑病变涉及白色物质束。此外，在相当大比例的情况下，身体创伤的记录表明，这些病变可能是创伤后的后果。因此，身体创伤可能是EDS的触发因素。
The role of physical trauma in the onset of symptoms in Ehlers-Danlos syndrome (EDS) has never been characterized. We sought to search and describe brain lesions EDS patients also having personal history of physical trauma. We systematically performed brain magnetic resonance imaging in a first cohort of patients with a hypermobility type of EDS which described the onset of their disease or its worsening after a physical trauma. Unexpected yet consistent findings that were thought to be related to the reported traumas led to perform brain imaging in all subsequent patients with similar symptoms regardless of a history of trauma and to search for a prior trauma by active questioning. Fifty-nine patients were recruited and analyzed, among which 53 (89.8 %) were women. Overall, 26 (44.1 %) reported a personal history of physical trauma. Six signs pertaining to subcortical lesions and affecting white matter tracts were identified. Those included lesions of the reticular formation, the two lenticular nuclei, the corpus callosum and the arcuate fasciculus. Thirty-six patients (61.0 %) had at least 5 of the 6 imaging signs. In case of a trauma before 18, patients had significantly more lesions of the reticular formation (100 % vs. 50 %; p = 0.0035). Patients with EDS, hypermobility type, were found to have consistent and specific brain lesions involving white matter tracts. Moreover, the record of a physical trauma in a substantial proportion of cases suggests that these lesions could be post-trauma consequences. Therefore, physical trauma could be a triggering factor in EDS.