Joint hypermobility and headache: understanding the glue that binds the two together--part 1

Headache. 2014 Sep;54(8):1393-402. doi: 10.1111/head.12418. Epub 2014 Jul 16.

Abstract

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.

Results: 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.

Keywords: Ehlers-Danlos syndrome; Marfan syndrome; connective tissue disorder; headache; joint hypermobility syndrome; migraine.

Publication types

  • Review

MeSH terms

  • Connective Tissue Diseases / complications
  • Connective Tissue Diseases / diagnosis
  • Ehlers-Danlos Syndrome / complications*
  • Ehlers-Danlos Syndrome / diagnosis
  • Headache / etiology*
  • Humans
  • Joint Instability / complications*
  • Joint Instability / diagnosis
  • Marfan Syndrome / complications*
  • Marfan Syndrome / diagnosis