Background: Past studies have reported that connective tissue disorders (CTDs) are more common in patients with specific types of headache disorders.
Objectives: The objectives of this study are (1) to review and critique the clinical studies reporting an association between joint hypermobility, CTDs and headache and (2) to postulate mechanisms though which CTDs might predispose to headache disorders.
Methods: PubMed was searched for relevant articles with search terms that included joint hypermobility, Ehlers-Danlos syndrome, Marfan syndrome, and specific headache disorders. A narrative review was performed of these articles as well as those identified from the bibliography of these articles.
Results: Case reports and case control studies confirm an association between CTDs and migraine, coat-hanger headaches, carotid arterial dissections, intracranial hypotension, Arnold Chiari malformations-type 1, cervical spine disorders, and temporomandibular joint disorders.
Conclusions: Observational cross-sectional studies suggest that the prevalence of CTDs is increased in patients with specific types of headache disorders. It is unknown if the CTDs directly cause these headaches disorders or are associated with them through other mechanisms.
Keywords: Ehlers-Danlos syndrome; Marfan's syndrome; connective tissue disorder; headache; joint hypermobility syndrome; migraine.
© 2014 American Headache Society.