Connective tissue, Ehlers-Danlos syndrome(s), and head and cervical pain

Area of Science:

• Neurology
• Genetics
• Rheumatology

Background:

• Ehlers-Danlos syndrome (EDS) encompasses hereditary connective tissue disorders characterized by joint hypermobility, skin hyperextensibility, and tissue fragility.
• Unlike other connective tissue disorders, EDS typically allows a normal lifespan but causes significant disability due to pain, fatigue, and headache.
• Headache is a prevalent and disabling symptom in EDS patients, necessitating a deeper understanding of its characteristics and underlying mechanisms.

Purpose of the Study:

• To review the existing literature on the prevalence, clinical features, and potential pathogenic mechanisms of headache in Ehlers-Danlos syndrome.
• To highlight the association between specific EDS types, particularly hypermobility EDS, and various headache disorders.
• To discuss current treatment challenges and propose avenues for future research and improved patient management.

Main Methods:

• A comprehensive review of pertinent scientific literature focusing on headache in EDS.
• Analysis of reported prevalence data, clinical presentations of headache disorders in EDS patients.
• Examination of proposed pathogenic mechanisms, including cardiovascular dysautonomia, structural abnormalities, and central sensitization.

Main Results:

• Headache is reported in at least one-third of EDS patients, with migraine being the most common type in hypermobility EDS.
• Associated headache disorders include tension-type headache, new daily persistent headache, and headaches linked to cerebrospinal fluid leakage, Chiari malformation, and cervicogenic issues.
• Pathogenesis is multifactorial, potentially involving dysautonomia, cervical spine instability, meningeal fragility, poor sleep, medication overuse, and central sensitization.

Conclusions:

• Headache is a significant and complex issue in Ehlers-Danlos syndrome, requiring further investigation.
• Subclinical cervical spine dysfunction may play a crucial role in EDS-related headaches.
• Current symptomatic treatments are often inadequate, emphasizing the need for improved diagnostic and management strategies and dedicated research.