Headaches in Ehlers-Danlos Syndromes

Chronic pain Hypermobility Lifestyle

by Jeannie di Bon, March 28th, 2024

Estimated reading time: 5 minutes

Headaches are a frequent complaint among my hypermobile clients and The Zebra Club community. In a recent retrospective study of patients with either hEDS/HSD or another form of EDS, 66% had head and neck problems including headaches, neck pain, facial pain, neck instability, or a combination of these (1). Headache has also been characterized as “one of the most common and disabling types of pain in hEDS (2).

The recent National Health Service guidelines on migraine management report that migraines may be responsible for many more symptoms than simply a headache. It is in fact considered an invisible and common disability for many people. These non-headache symptoms can include fatigue, coathanger pain, dizziness, poor quality sleep and brainfog. These are also all symptoms often reported by people with EDS or HSD. We will explore this connection further on in this article.

The spectrum of headaches and migraines in EDS

Headaches seem to vary from a frequent occurrence, to chronic and debilitating. Headaches in EDS can be linked to a wide range of conditions. However exactly why EDS is related to these is not always understood. Some of these conditions include (1,2,3):

  • Migraine
  • Idiopathic Intracranial Hypotension (increased pressure cerebrospinal fluid)
  • Chiari malformation – spinal cord and brain compression
  • Upper Cervical Instability
  • Dysautonomia – Such as Orthostatic Hypotension (OH) or Postural Orthostatic Tachycardia Syndrome (POTS)
  • Temporomandibular Joint Disorders (TMJD)
  • Muscle tension
  • Cervicogenic headaches from other neck issues like Occipital Neuralgia

Anecdotally, we could probably add sleep issues, menopause and mast cell activation syndrome (MCAS) as common issues that likely contribute to headaches.

Migraines and hypermobility

A migraine is more than a headache. This condition is characterized by frequent moderate to severe attacks that last 4 -72 hours. They are often one-sided, pulsating and can be associated with nausea, light and noise sensitivity, and can be disabling (3). Migraines and hypermobility are both more frequent in women (3).

It was not surprising to learn that migraine was found to be 3 times higher in patients with joint hypermobility syndrome (4). It was interesting to learn that migraines in hypermobile people were found to actually be more severe than in non-hypermobile patients. This was both in how frequent the headaches occurred, but had more severe symptoms, were more at risk for having chronic migraines, and were usually not adequately treated (4).

A note on vestibular migraines

I have a handful of patients who experience another type of migraine called vestibular migraines that complicate the headache picture even more. This type of migraine is often characterized as migraines with vertigo. The vestibular system’s job is to detect where the head is in space and how it is moving. This is done through nerve signals between the vestibular system in the inner ear to and from the brain. Vestibular migraine is also rare, affecting up to 2.7% of the general population (5). However, we could find no numbers of its prevalence in hEDS/HSD specifically.

Officially, diagnostic criteria include having episodes of moderate to severe vestibular symptoms lasting 5 minutes to 72 hours, with a history of migraines, and having one of these symptoms with 50% of the vestibular episodes: one-sided, pulsating headache; light or sound sensitivity; and/or aura. Additionally, other reasons for having vestibular symptoms have been ruled out (5).

Some examples of vestibular symptoms include (5):

  • Internal vertigo (sensation that body is in motion)
  • External vertigo (sensation that the room is spinning)
  • Positional vertigo with changes in head position
  • Visually induced vertigo
  • Vertigo with head motion
  • Head motion-induced dizziness with nausea

Vestibular migraines, like other migraines, are more common in women. They also seem to have an autosomal dominant inheritance pattern similar to that seen in hEDS, meaning a child of a parent has a 50% chance of inheriting the condition (6). It was also interesting to learn that in postmenopausal people, typical migraines are sometimes replaced by these episodes of vertigo, dizziness, or feeling off balance (6).

This type of migraine may need to be treated by a vestibular migraine experienced specialist or a Neuro-otologist, sometimes an otolaryngologist (ENT). Vestibular Rehab with a specialised physical therapist may also help.

Investigating the link between upper cervical instability, hEDS/HSD, and headaches

Recently the scientific advisor to The Zebra Club, Dr. Leslie Russek, published the first expert consensus and recommendations for screening for and managing Upper Cervical Instability (UCI). This was done along with an international team of physical /physiotherapy clinicians and researchers and a hypermobility specialist rheumatologist. They state that mild UCI is relatively common among people with hEDS/HSD impacting between 52-66%. However, severe UCI is thought to be uncommon impacting only 5% of people with hEDS/HSD (7).

Craniocervical instability (CCI) is ligament laxity in the joint between the atlas (Cranial vertebra 1 or C1) and the occiput or base of the skull (C0). There can also be instability in the atlas (C1) and the axis (C2), this is called atlantoaxial stability (AAI). Together these can be called Upper Cervical Instability (UCI).

Symptoms of UCI exist on a spectrum and can include headaches, neck or facial pain, dizziness, vertigo, nausea, numbness/tingling, shortness of breath, difficulty speaking, vision changes (blurred, tunnel vision, auras), hearing changes, difficulty swallowing, choking, sleep apnea, memory deficits, pre-syncopal episodes (feeling like you’re going to faint).

Determination Criteria for UCI

Russek et al classify patients as having low, moderate, or high “irritability.” This is a measure of mechanical irritability. This is based on how severe the condition is, how easily it is flared, and how long it takes for symptoms to calm down after being flared. Importantly, this may change from day to day and progress towards lower irritability as patients improve. These are important to keep in mind when looking at recommended interventions.

Three criteria must be met:

  • Symptoms are consistent with musculoskeletal and/or neurological UCI.
  • Symptoms are altered by neck movement or position.
    • For example: Increased symptoms when leaning forward or looking down
    • Increased symptoms with neck motions like flexing, extending, or rotating
  • Mechanical irritability with a physical exam

Useful assessment tools are provided for use by physical/physiotherapists as well as recommendations for when to make referrals. I don’t recommend making this determination yourself.

Exploring self-treatment for neck-related headache issues

Importantly, some strategies can help with UCI. The authors recommended numerous interventions organized by levels of irritability. Russek et al provide several very helpful intervention suggestions to help with UCI symptoms. Many of these strategies are things I already found useful with my clients and strategies I made sure were included in The Zebra Club App

  • Sitting, standing, and sleep posture, positioning, and body support
  • Body awareness and mindfulness in various positions (sitting, standing, lying down).
  • Relaxation and autonomic nervous system balancing (not requiring neck movement).
    • The Head Meditation Audio in The Zebra Club is reportedly very helpful for headache pain management.
  • Breathing (diaphragmatic or slow breathing)
    • Try one of the breathing classes in The Zebra Club or this one
  • Education in pain neuroscience
  • Mindful use of language to enhance feelings of safety
  • Self-care “toolbox”
  • pain management strategies like TENS, topicals, relaxation, etc.

In this video I discuss more about my experience working with clients who have neck pain and headaches. I then teach a movement class. Please note – these exercises may not be suitable for everyone. They should not cause increased pain or neurological symptoms. Please seek medical advice if they do. They are not designed to replace any medical guidance you have been given. If you are highly irritable in the neck, please work one-on-one with a physical therapist.

Works Cited

  1. Malhotra, et al (2020) Headaches in hypermobility syndromes: A pain in the neck? PMID: 32940405
  2. Levy, et al (2020) An investigation of headaches in Hypermobile Ehlers-Danlos Syndrome. Annals of Psychiatry and Clinical Neuroscience.
  3. Henderson, et al (2017) Neurological and Spinal Manifestations of the Ehlers-Danlos Syndromes PMID: 28220607
  4. Puledda et al (2015) A study of migraine characteristics in joint hypermobility syndrome a.k.a. Ehlers-Danlos syndrome, hypermobility type.
  5. Lempert et al, (2022) Vestibular Migraine: Diagnostic criteria (Update).
  6. Stolte et al, (2015) Vestibular Migraine. PMID: 24847169
  7. Russek et al (2023) Presentation and physical therapy management of upper cervical instability in patients with symptomatic generalized joint hypermobility: International expert consensus recommendations.

Literature review by Catherine Nation, MSC, PhD


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Martina Ingemarsson - 10th April 2024

This article was very helpful to me, thank you! Very pedagogical. I will now make sure to watch the YouTube episode and class.


    Jeannie Di Bon - 11th April 2024

    Thank you – I am so glad you enjoyed this headache article.


Kylie - 29th March 2024

My daughter often tells us she is seeing one colour could be pink, blue, red or green then after a little while states she has a headache. Just interested what category this may fall into


    Jeannie Di Bon - 2nd April 2024

    Thank you for taking the time to comment. I cannot give any medical advice on here but do recommend checking with your medical practitioner.