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Brain injury unmasking Ehlers-Danlos syndromes after trauma: the fiber print

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  • معلومة اضافية
    • Contributors:
      Hôpital Hôtel-Dieu [Paris]; Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP); Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12); Service de Neuropathologie [Sainte-Anne]; Hôpital Sainte-Anne; Université Pierre et Marie Curie - Paris 6 (UPMC); CHU Saint-Antoine [AP-HP]; Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU); Laboratoire d'économie mathématique et de microéconomie appliquée (LEMMA); Université Panthéon-Assas (UP2); Dpt proctologie [CHU Croix St Simon]; Groupe Hospitalier Diaconesses Croix Saint-Simon; Service de chirurgie générale et digestive [CHU Saint-Antoine]; Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Saint-Antoine [AP-HP]; Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU); Laboratory of modern economics; Service de Gastroentérologie et nutrition [CHU Saint-Antoine]; Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)
    • بيانات النشر:
      Springer Nature
    • نبذة مختصرة :
      International audience; Background: 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. Results: 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). Conclusions: 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.
    • ISSN:
      1750-1172
    • الرقم المعرف:
      10.1186/s13023-016-0428-9
    • Rights:
      OPEN
    • الرقم المعرف:
      edsair.doi.dedup.....6375808afc7a835b22de52aac062c6dd