Brain injury unmasking Ehlers-Danlos syndromes after trauma: the fiber print.
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.
Department of Physical and Rehabilitation Medicine, Hôtel Dieu Hospital, APHP, Paris, France.|Department of Medicine, University Paris-East-Créteil (UPEC), Créteil, France.
Claude
C
Hamonet
Department of Neuroradiology, Saint-Anne Hospital, Paris, France.
Daniel
D
Frédy
Department of General and Digestive Surgery, Saint-Antoine Hospital, APHP, Paris, France.|University Paris VI, Paris, France.
Jérémie H
JH
Lefèvre
Laboratory of Modern Economics, Paris II University, Paris, France.
Sacha
S
Bourgeois-Gironde
Department of Gastroenterology and Nutrition, Saint-Antoine Hospital, APHP, 284, rue du Faubourg Saint-Antoine, 75012, Paris, France. jdzeitoun@yahoo.fr.|Department of Proctology, Croix Saint-Simon Hospital, Paris, France. jdzeitoun@yahoo.fr.
Jean-David
JD
Zeitoun
D000328:Adult; D001930:Brain Injuries / Q000175:diagnosis*; D004535:Ehlers-Danlos Syndrome / Q000175:diagnosis*; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D055815:Young Adult
Brain injury; Brain trauma; Ehlers-Danlos syndrome