Acute brain ischemia as a complication of the Ehlers–Danlos syndrome, the case series

Pająk, Michał; Majos, Marcin; Szubert, Wojciech; Stefańczyk, Ludomir; Majos, Agata

doi:10.1177/1708538113505519

Cited by 1 publication

(1 citation statement)

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“…Our findings are novel, given that CNS lesions have only exceptionally been reported in EDS [ 10 ]. We categorized the observed lesions as six imaging signs that were thought to be sufficiently frequent and specific for claiming a high degree of validity.…”

Section: Discussionmentioning

confidence: 72%

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

Hamonet

Frédy

Lefèvre

et al. 2016

Orphanet J Rare Dis

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BackgroundThe 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.ResultsFifty-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).ConclusionsPatients 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.

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Section: Discussionmentioning

confidence: 72%