I currently have an integrative psychiatry and neurologic health practice in Scottsdale, Arizona, where we regularly attend to patients with "weird" or "mystery" symptoms attributable to dysautonomia (e.g., postural orthostatic tachycardia syndrome (POTS)), long COVID, and Ehler's-Danlos Syndrome (EDS). However, recently we have begun to see a particularly nasty presentation: Patients presenting with two or all three of these conditions at the same time! I'm writing this to bring awareness to this particularly tormenting combination of conditions.
Understanding Ehlers-Danlos Syndrome (EDS) and Its Role in Dysautonomia, Long COVID, and Brain Fog
Ehlers-Danlos Syndrome (EDS) is a group of inherited connective tissue disorders. It affects tissues that provide support to the skin, joints, blood vessels, and other organs. The hypermobile subtype (hEDS) is the most common form and has drawn attention for its potential connection to conditions like dysautonomia, Long COVID, and neurological symptoms like brain fog. I hope to briefly explain these sometimes complicated connections and how EDS may complicate these and other health conditions.
Let's first discuss the (often missed) symptoms and signs of EDS.
Symptoms and Signs of Ehlers-Danlos Syndrome
The symptoms of EDS can vary depending on the subtype, but common features include:
Joint Hypermobility: Joints that move beyond the normal range, often leading to pain and frequent dislocations.
Skin Hyperextensibility: Skin that stretches more than usual and feels soft or velvety.
Tissue Fragility: A tendency for easy bruising, slow wound healing, and scars that look different than normal.
Chronic Pain: Persistent pain in muscles and joints.
Heart and Blood Vessel Problems: Some people may have mitral valve prolapse or widening of the aorta (Murray et al., 2020).
Diagnostic Criteria for Hypermobile EDS (hEDS)
Diagnosing hEDS can be challenging because there is no specific genetic test. Instead, doctors use clinical guidelines. The most recent criteria (The Ehlers-Danlos Society, 2017) include:
Generalized Joint Hypermobility (GJH): Assessed with the Beighton score, which measures joint flexibility.
Systemic Signs of Connective Tissue Disorder: At least five of these features:
Soft or velvety skin.
Mildly stretchy skin.
Unexplained stretch marks.
Heel bumps (piezogenic papules).
Recurring hernias.
Atrophic (sunken) scars.
Organ prolapse (e.g., bladder or uterus).
High or narrow palate.
Long, thin fingers (arachnodactyly).
Arm span longer than height.
Heart problems like mitral valve prolapse or aortic dilation.
Family History: A close relative with hEDS.
Exclusion of Other Disorders: Other connective tissue disorders like Marfan syndrome or Loeys-Dietz syndrome must be ruled out.
EDS and Dysautonomia
Now, let's discuss dysautonomia. Dysautonomia refers to a group of conditions that affect the autonomic nervous system, which controls involuntary body functions like heart rate and digestion. Many people with EDS experience dysautonomia, including conditions like Postural Orthostatic Tachycardia Syndrome (POTS). POTS causes symptoms such as dizziness, rapid heartbeat, and fatigue, especially when standing up. Studies suggest that the connective tissue abnormalities in EDS may affect blood vessel function, contributing to dysautonomia (Rowe et al., 2021). This fact is not unknown to the medical field but diagnosing and recognizing this problematic combination is far from easy, often requiring months to years of visits to specialists, most of whom are still learning about both of these conditions.
That said, this topic becomes much more complicated with the introduction of a third medical "wild card" - Long COVID.
EDS and Long COVID
Long COVID, also called post-acute sequelae of SARS-CoV-2 infection (PASC), refers to lingering symptoms after a COVID-19 infection. Symptoms like fatigue, joint pain, and brain fog overlap with those of EDS. Research indicates that people with underlying connective tissue disorders, including EDS, may have an increased risk of experiencing prolonged COVID-19 symptoms (Knight et al., 2023). That said the novelty of COVID and of Long COVID means that the medical field has yet to fully understand this condition, and especially its interaction with other pre-existing conditions like EDS and dysautonomia.
EDS and Brain Fog
Brain fog—a term used to describe cognitive issues such as memory problems, trouble concentrating, and mental fatigue—is common in both EDS and Long COVID. People with EDS often have autonomic dysfunction, which can impair blood flow to the brain, potentially causing or worsening brain fog. Chronic pain and fatigue associated with EDS may also contribute to these cognitive symptoms (Castori et al., 2022).
Why Understanding EDS is Important
The overlapping symptoms between EDS, dysautonomia, Long COVID, and brain fog can make diagnosis and management difficult. However, recognizing these connections can help people get the care they need. While we are still learning about each of these conditions, managing them is possible with a growing range of treatment options, ranging from lifestyle changes, medications, certain medical procedures, and physical rehabilitation can significantly improve quality of life for people with EDS. The first step is proper diagnosis, which alone can be a relief.
About the Author: Dr. David George is founder of Neuregen, an Integrative Psychiatry and Neurologic Health Clinic in Scottsdale, Arizona offering a multidisciplinary approach to brain-based conditions, including brain injury, post-concussion symptoms, long covid, dysautonomia, and medical trauma.
References
Castori, M., Morlino, S., Pascolini, G., Blundo, C., & Grammatico, P. (2022). Autonomic dysfunction in Ehlers-Danlos syndrome hypermobile type: A review. American Journal of Medical Genetics Part C: Seminars in Medical Genetics, 190(4), 447-455. https://doi.org/10.1002/ajmg.c.31956
Knight, K. M., Levison, L. J., & McBride, J. M. (2023). Long COVID symptoms in patients with hypermobility spectrum disorders: A case-control study. Journal of Post-Acute Care Medicine, 15(2), 123-130. https://doi.org/10.1016/j.jpacm.2023.02.005
Murray, B., Yasin, Z., & Milner, J. D. (2020). Cardiovascular implications of Ehlers-Danlos syndrome. Frontiers in Cardiovascular Medicine, 7, 95. https://doi.org/10.3389/fcvm.2020.00095
Rowe, P. C., Barron, D. F., & Bou-Holaigah, I. (2021). POTS and joint hypermobility syndrome: A common association. Clinical Autonomic Research, 31(1), 43-48. https://doi.org/10.1007/s10286-020-00755-w
The Ehlers-Danlos Society. (2017). Diagnostic criteria for hypermobile Ehlers-Danlos syndrome. Retrieved from https://www.ehlers-danlos.com
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