BACKGROUND: Heritable connective tissue disorders (HCTD) present with a wide array of findings, including headache. Because of their unusual substrate, headaches in HCTD can derive from both common and uncommon circumstances.
METHODS: Literature review.
Ehlers-Danlos hypermobile type can be recognized by multiple joint findings and its tendency to progress to a multisystem chronic pain syndrome.
Ehlers-Danlos classic type also manifests joint laxity and similar pain complaints, but is differentiated by its skin laxity and fragility.
Ehlers-Danlos vascular type presents the most severe risk due to blood vessel and hollow organ rupture.
Marfan syndrome demonstrates skeletal abnormalities, lens dislocations, and aortic root dilation that can result in dissection.
CONCLUSIONS: In a headache patient, recognizing the presence of an HCTD improves the strategy for diagnosis and management. A brief review of findings related to joints, skin, and arteries may prompt further investigation into the HCTDs.
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
- 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.