Head Pain in Ehlers-Danlos Syndrome (EDS)

Headache is one of the most common pain complaints among individuals with Ehlers-Danlos Syndrome (EDS) (Jacome). Due to the multisystemic nature of EDS, headaches can arise from various underlying causes, including structural abnormalities, vascular issues, neurological dysfunctions, and musculoskeletal complications. Understanding these causes is crucial for proper diagnosis and management. The more common causes of headaches in EDS are listed in Table 1, and this article will discuss several of the more pertinent ones in detail.

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Common Causes of Headaches in EDS
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Detailed Discussion of Common Causes

Arnold Chiari Malformation

Arnold Chiari Malformation (ACM) is a common comorbidity in EDS, characterized by herniation of the cerebellar tonsils through the foramen magnum. This displacement disrupts cerebrospinal fluid (CSF) flow, potentially leading to increased intracranial pressure or non-communicating hydrocephalus.

Cervicogenic Headaches

Cervicogenic headaches stem from instability and excessive movement in the cervical spine. Many EDS patients present with excessive intersegmental vertebral motion, which can contribute to persistent daily headaches. This form of headache is often mistaken for migraines but can be differentiated by its origin in neck dysfunction.

Spontaneous Cerebrospinal Fluid Leak

EDS patients are predisposed to spontaneous CSF leaks due to connective tissue fragility, leading to intracranial hypotension and postural headaches that worsen when upright. Approximately two-thirds of patients with spontaneous CSF leaks have underlying connective tissue disorders. The precise cause is unclear but may involve a combination of dural thinning and minor trauma.

Cerebrovascular Diseases

EDS, particularly the vascular subtype, increases the risk of cerebrovascular complications such as intracranial aneurysms, subarachnoid hemorrhage, and spontaneous arterial dissections. Patients with spontaneous carotid-cavernous sinus fistula may experience sudden-onset unilateral headaches, pulsatile tinnitus, or proptosis.

Vision-Related Headaches

EDS patients often present with keratoconus, high myopia, retinal detachment, and lens subluxation. Poorly corrected vision can contribute to head and neck posture imbalances, exacerbating headaches. Additionally, fluctuating vision due to dysautonomia complicates the ability to maintain consistent corrective lenses, leading to strain-induced headaches.

Temporomandibular Joint Dysfunction

Temporomandibular joint (TMJ) dysfunction is reported in over 70% of EDS patients. TMJ hypermobility leads to jaw misalignment, muscle strain, and chronic headaches. Symptoms include jaw pain, clicking sounds, and limited mouth opening.

Conclusion

Head and neck pain in EDS can have diverse etiologies, requiring a multidisciplinary approach for effective management. Identifying the underlying cause is critical for targeted therapy, which may include physical therapy, bracing, medications, and, in some cases, surgical intervention. Further research is needed to improve diagnostic techniques and treatment options for EDS-related headaches.

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