Are Ehlers-Danlos Syndrome Headaches and Migraines Caused by Cervical Spine and Neck Instability?
Ross Hauser, MD
Ehlers-Danlos Syndrome Headaches and Migraines
Among the many symptoms that patients suffer from in Ehlers-Danlos Syndrome is one of headache and migraine. Like many of the other symptoms that these people suffer from, the diagnosis and treatment of their headache and migraine pain is often wrapped in confusion, treatments that are not helpful, and the continuation of the helplessness feeling that many Ehlers-Danlos Syndrome have. In this article we hope to present evidence to suggest that headaches and migraine pain in Ehlers-Danlos Syndrome can be resolved by addressing problems of damaged, weakened, loose, lax cervical spine ligaments with simple injection therapies. Certainty a simple theory for a complex problem.
Headaches: A symptom among a sea of symptoms
It is very rare that a patient diagnosed with Ehlers-Danlos Syndrome will come in with one or two symptoms or health challenges. They will have many, sometimes dozens. These are the type of things we hear from people. Very likely these people will tell a story that sounds very familiar to your own.
I suffer from severe headaches. I also have severe TMJ. I had a lot of treatments for the TMJ. My doctors thought that if I could address the TMJ I could address my headache. After years of treatment and no success, my doctors finally realized that I have hEDS Ehlers-Danlos Syndrome. So now after chasing TMJ for years my doctors are telling me that my C1-C2 are hypermobile and out of place.
I have hEDS and the “only way out,” is an anterior cervical discectomy surgery which scares me. I have so many symptoms. Terrible hip pain, knee pain, shoulder pain, wrist pain for which I had a carpal tunnel surgery that failed. I have terrible neck pain, vision problems, and migraines. The headaches and migraines are now every day. I have also developed tinnitus along with dizziness.
On our website we have a series of articles that you can refer back to help you understand neck pain, TMJ, and headaches.
- The evidence and comparisons of TMJ injection treatments
- Ehlers-Danlos syndrome, Joint hypermobility, and temporomandibular disorders in younger patients
- Cervicogenic headaches: Migraines, tension headaches and cervical spine instability
- Vestibular migraine and spontaneous vertigo – Migraine Associated Vertigo
Not enough research linking headaches to hEDS: Patients need more
The concurrent challenges patients face, are well documented, certainly we see them at our center. Here an August 2020 paper in the journal Annals of Psychiatry and Clinical Neuroscience (1) not only makes a connection between the symptoms but also says there is not enough research linking headaches to hEDS and we need more. Here is what the researchers offered:
- “Although there are various neurological manifestations that could present as headaches in hEDS, this report will discuss the prevalence and presentation of migraine, idiopathic intracranial hypertension, dysautonomia, craniocervical instability, temporomandibular joint dysfunction, and Chiari malformation in patients with hEDS.
- Although the exact etiology and pathophysiology of various types of headache seen in hEDS is not known. One of the limitations of the study is lack of enough observational studies to establish a causal association between the various types of headache discussed and hEDS. In addition to discussing previous research on these topics, this review summarizes areas in need of further investigation.”
Some of the learning points of this study include similar findings to what we see clinically:
Research on Increased intracranial pressure
- The researchers note among other things that increased intracranial pressure may play a role as it is due to excess cerebral spinal fluid production. Other theories argue that increased intracranial pressure is due to obstruction of veins that drains blood from the brain leading to increase venous sinus pressure.
- When cerebral spinal fluid flow is disrupted, it’s almost as if the brain becomes a toxic toilet. You need normal cerebral spinal fluid flow to be normal to flush all the toxins and all the waste products from all the activity of the brain out. If these is impeded flow you will suffer the problems of not being able to focus or remember. You feel like a brain fog has surrounded you. This can be caused by upper cervical instability.
In our article: How cervical spine instability disrupts blood flow into the brain, we discuss how cervical spine instability pinches on arteries and disrupts, impedes, and retards blood flow into the brain. This is one of the great challenges that face cervical spine or cervical neck instability patients. In our office, almost all the people who have upper cervical spine instability, who come in for our non-surgical treatments, have an amazing amount of brain fog, the inability to concentrate, anxiety, and depression. They can also suffer from the symptoms listed above including migraines. For more information, please continue with that article: How cervical spine instability disrupts blood flow into the brain
Treat the problems of the neck, you can treat the problems of Ehlers-Danlos and hypermobility related headaches.
Neck pain as a cause of headaches is a subject we have written about for nearly 30 years. Validation of our own clinical observations and scientific papers have come in the form of independent and published findings in the medical literature. Simply all this means is that there is a connection between your diagnosis of hEDS, your headaches, and a problem of cervical spine instability.
Here is a study with a December 2020 publication date. It comes from the Hospital for Special Surgery, Weill Cornell Medical College, and the Icahn School of Medicine at Mount Sinai Hospital in New York.(2)
This is what these researchers said:
“Headache and neck pain (cervicalgia) are frequently reported among patients with joint hypermobility but the prevalence and scope of these symptoms has not been studied in the era of contemporary Ehlers-Danlos and hypermobility disorder nosology.”
In other words a connection between headaches and hEDS has not been fully understood or made. To help this understanding, this research team looked at 140 patients with hypermobility disorders. They followed the patients progress over two years. Here are their observations:
- Overall, 93 patients (66%) reported either headache or neck pain with 49 of those (53%) reporting both.
- Migraine (83%) was the most common headache type among those with headache disorders and cervical spondylosis (61%) the most common pathology among those with neck symptoms.
- Fifty-nine percent of spondylosis patients who underwent cervical facet procedures reported significant improvement in neck and head symptoms.
- Of patients with both head and neck complaints, 82% had both migraine and spondylosis, which, when combined with the high response rate to injections raises the possibility of cervicogenic headache.
- Findings: “In this large multidisciplinary retrospective study of patients with hypermobility disorders, head and neck symptoms were highly prevalent, with migraine and cervical spondylosis common, often coexisting, and frequently responsive to targeted therapy for the cervical spine suggesting that degenerative spinal pathology may cause or contribute to headache symptoms in some patients with hypermobility disorders.”
Simply: Treat the problems of the neck, you can treat the problems of Ehlers-Danlos and hypermobility related headaches.
People with hypermobile EDS are more likely to have migraine, and their migraine disease is more frequent and disabling.
Some of the examples we give of what people talk to us about may mimic what is happening to you, so many problems causing so much pain that the amount of pain is making all the pain worse. The same is true for the intensity of migraines.
Migraines are worse in hEDS and joint hypermobility patients. Your headaches are really that bad
As mentioned in the study above “the prevalence and scope of these symptoms has not been studied in the era of contemporary Ehlers-Danlos and hypermobility disorder nosology,” This means that there is not a lot of research offering answers to the problems of headaches and migraine (which I will get to below). There are however observations. One observation is that Migraines are worse in hEDS and joint hypermobility patients.
A study in the journal Neurological sciences (3) suggested that Migraines are worse in hEDS and joint hypermobility patients and offered these observations:
- “Joint hypermobility syndrome (JHS) and Ehlers-Danlos syndrome, hypermobility type (hEDS) are two clinically overlapping heritable connective tissue disorders strongly associated with musculoskeletal pain, fatigue and headache.
- Migraine with or without aura is considered the most common form of headache in JHS/hEDS.
When symptoms and intensity were compared between patients suffering from migraines and JHS/hEDS and patients suffering from migraines without JHS/hEDS, the researchers observed:
- Results showed that in JHS/hEDS patients:
- (1) migraine has an earlier onset (12.6 vs 17 years of age)
- (2) the rate of migraine days/month is higher (15 vs 9.3 days/month);
- (3) accompanying symptoms are usually more frequent;
- (4) Headache and disability pain scores were higher
- (5) effectiveness of rescue medication is almost identical, although, total drug consumption is significantly lower
- Joint hypermobility syndrome and Ehlers-Danlos syndrome, hypermobility type patients have a more severe headache syndrome with respect to the migraine only group, therefore demonstrating that migraine has a very high impact on quality of life in this disease.
The last sentence is probably something you already knew but have a study behind it may help convince your doctors that your headaches are really that bad.
The researchers suggest “extracranially-directed treatments such as occipital nerve blocks, cervical trigger point injections, botulinum toxin, and monoclonal antibodies may provide more substantial relief for unremitting head and neck pain; additionally, decompression of the occipital nerves from muscular and fascial compression is effective for some patients and may result in enduring pain relief.” To this list we would add Prolotherapy. This treatment is discussed below.
To treat you must find the cause
Below we summarize our article “Digital Motion X-ray: Finding the missing cause of headaches, dizziness, and facial pain.”
One of the problems in hEDS sufferers is the long journey they took to get to a diagnosis. Then once they had the diagnosis another journey began to find a treatment. Sometimes the treatment and the diagnosis did not match.
At our center we use a Digital Motion X-ray machine or DMX. This helps us visually see disorders in the cervical spine in real time and in real motion. This is not a static image. For many people, a digital motion x-ray can reveal a series of missing diagnoses and possibly end your chase for that elusive diagnosis that finally responds well to treatment.
Again, a digital motion x-ray is exactly what it is sounds like. It is a moving picture x-ray. In this video, Dr. Brian Hutcheson, DC shows how digital motion x-ray helped reveal a series of missing diagnoses in the case of a woman in her mid-30’s who happened to be a mother of three.
Video transcript summary and explanatory notes:
Headaches, neck pain, lightheartedness, dizziness, facial pain, and trigeminal neuralgia.
- This is the case of a 35 year-old mother-of-three suffering from headaches, neck pain, lightheartedness, dizziness and facial pain that can be known as trigeminal neuralgia. We are going to show you her digital motion x-ray.
- Digital motion x-ray allows us to examine your neck as your head and neck travel through different ranges of motion. This provides us more information on what is happening in your neck than what we would see in a conventional or static image “snap shot.”
What this mom’s neck reveals
At 0:36 of the video the motion image of her neck
- The first thing we see in her digital motion x-ray is that she has lateral C1 tilting. Some of you may understand this as lateral head tilting. Lateral head tilting has not only been linked to these following problems, but these following problems have also been shown to be difficult to accurately diagnose. This is why the we believe in the value of the digital motion x-ray in uncovering the missing diagnoses in the cervical spine.
Head, neck, jaw and face pain
In our many years helping people with chronic pain of the head, neck, jaw and face we have seen many people with a diagnosis of Trigeminal neuralgia. For some of these people, possibly including yourself, it was not easy getting this diagnosis as this problem can be confusing and frustrating to understand. Not only for the patient but the doctor alike. The problems of diagnosis to determine where you source of facial, head and jaw pain is coming from is covered in our article: The evidence for Trigeminal Neuralgia non-surgical treatments.
Painful, involuntary muscle spasms in the neck
- Lateral head tilting is linked to: Torticollis, painful, involuntary muscle spasms in the neck with a resulting head rotation and tilting to one side. In the 2020 medical publication STAT Pearls, the authors noted, “There may be several presenting positions, including flexion, extension, right or left tilt (in Torticollis.) (1) See our article Cervical dystonia and spasmodic torticollis treatment.
Dizziness, loss of balance, sometimes nausea, sometimes vomiting
- Lateral head tilting is linked to: Benign paroxysmal positional vertigo or BPPV. Some of you reading this article may have been offered this diagnosis with a somewhat reassuring tone that your dizziness, loss of balance, sometimes nausea, sometimes vomiting is something that can be managed, may go away on itself, and this will be more of a nuisance than anything. That is why it is called “Benign.” For some people indeed, this does happen, for many patients we see, the dizziness did not go away and it continues on. Please see our article Cervical Vertigo and Cervicogenic Dizziness.
You can continue with this article here: Digital Motion X-ray: Finding the missing cause of headaches, dizziness, and facial pain.
Patients with migraines and chronic headache have a cervical neck instability problem
As mentioned above at Caring Medical, we have been seeing headache patients for a long time. In 2009, our research team lead by Ross Hauser, MD., published our findings and recommendations for the treatment of chronic headaches and migraines in patients where clear cervical neck instability was suspected. This was based on already 15 years of clinic observation.
In our research, published in the journal Practical Pain Management, (4) we described the problems of patients with headaches that were not being helped by traditional drug-based medicine. Not much has changed in the last ten plus years.
- “While medicine carries 150 diagnostic headache categories, the vast majority of recurring headaches are classified as either migraine or tension. The most common headache types among adults and adolescents are tension headaches, chronic daily headaches or chronic non-progressive headaches. These muscle contraction headaches cause mild to moderate pain and come and go over a prolonged period of time.
- Migraine headache pain is often moderate to severe and described as a pounding, throbbing pain lasting from four hours to three days, and usually occur one to four times per month. Migraines are associated with symptoms such as light sensitivity, noise or odor sensitivity, nausea or vomiting, loss of appetite, and stomach upset or abdominal pain.
- Typical medical treatments for tension or migraine headaches involve the use of medications such as nonsteroidal anti-inflammatory drugs (NSAIDs), triptans or muscle relaxants. Despite the advances in migraine-specific drugs, only 50% of patients with migraine headaches attain more than 50% reduction of headache frequency after three months of treatment.
Treatments that do not work, do not work because they do not address the problems of cervical spine instability from cervical ligament damage.
Among some of the reasons that patients do not get headache or migraine relief is that pharmaceutical-based management of the patient’s headaches does not address the problem of headaches coming from neck pain caused by weakened or damaged cervical ligaments.
In our 2014 research led by Danielle R. Steilen-Matias, MMS, PA-C, and published in The Open Orthopaedics Journal (5) our research team was able to demonstrate that when the neck ligaments are injured, they become elongated and loose, which causes excessive movement of the cervical vertebrae. In the upper cervical spine (C0-C2), this can cause a number of other symptoms including, but not limited to, nerve irritation and vertebrobasilar insufficiency with associated vertigo, tinnitus, dizziness, facial pain, arm pain, and migraine headaches.
In this video, Ross Hauser, MD explains and demonstrates the use of Digital Motion X-ray (DMX) to help identify cervical neck and spine instability in the C1-C2 region in a patient with severe migraines.
The summary transcript of this video, with explanatory notes, is below the video.
One of the more common conditions we see at Caring Medical is severe migraine headaches. I am going to demonstrate how we diagnosis and plan treatments for a patient we suspect of upper cervical spine instability as the cause of migraine headaches. In this video Digital Motion X-ray DMX is utilized to demonstrate the clues and signs of cervical spine instability, especially that surrounding the Dens or the C2 vertebrae.
- The patient in this video is a physician who flew in from Europe to visit us here in Ft. Myers, Florida. He suffered a whiplash injury from snowboarding and windsurfing in 2011.
- The patient has migraine headaches every day, horrible pain behind the eyes, terrible neck pain, he has clicking, grinding, and crunching in his neck.
At the 1:00 mark of the video, the Digital Motion X-ray DMX of the patient’s cervical spine instability is demonstrated.
- As seen in the video, the DMX shows tilting of the C1 vertebrae. It is tilted through the full range of motion and this is one of the first signs that we see that a person has upper cervical instability.
- In the DMX open mouth view (AT 1:18) the video we’re looking for several things the first thing is the symmetry of the C2 vertebrae this is where the Dens and the spinous process, the bony protrusion at the back of the vertebrae, should be aligned with the Dens. In this particular patient, the C2 is shifted to the left. This misalignment, caused by cervical spine instability, is the reason that the person has migraine headaches primarily on their left side. That shift can be corrected with Prolotherapy. (Prolotherapy is a series of injections of simple dextrose. Below we will discuss the treatment further as well as provide medical research findings supporting its use in selected patients).
- (At 1:40 of the video_ We also see in the C1-C2 facet joint that there is a misalignment, there is an overhang of one vertebra over the other. We also examine changes in the periodontoid space. In this patient the periodontoid space is more narrow on one side than the other, demonstrating misalignment.
This patient had 4 Prolotherapy treatments, his headaches are at a minimum. He will need a few more treatments to stabilize his cervical spine and further reduce eliminate his migraines.
Headache researchers are understanding that patients with migraines and chronic headache have a neck pain/instability problem and it is more common than thought
Researchers representing Lund University in Sweden and the University of Copenhagen in Denmark published a December 2017 study in The Journal of Headache and Pain (6) showing that the prevalence of migraines with co-existing tension-type headache and neck pain is high in the general population.
They also acknowledge the problem that there is very little literature on the characteristics of these combined conditions.
- In this study, the researchers were able to conclude that migraine with co-existing tension-type headache and neck pain was highly prevalent and that persons with migraine and co-existing tension-type headache and neck pain may require more individually tailored interventions to increase the level of physical activity, and to improve psychological well-being, perceived stress, and self-rated health.
Now let’s examine a study from a diverse team of Canadian researchers from medical universities and hospitals throughout Canada. Here doctors writing in the European Spine Journal (7) explored treatments for managing patients who suffered from chronic tension-type headaches with constant neck pain and muscle spasm. The muscle spasms should have been a clue that the headaches were being caused by cervical neck instability. Muscles spasm in unstable joints because they are being overworked trying to help or replace the function of damaged ligaments and tendons in stabilizing the joint.
“Emerging evidence of occipital nerve compression in unremitting head and neck pain”
That is the title of recent research that appears in the July 2019 issue of The Journal of Headache and Pain (8) It comes from researchers at the University of Texas and Harvard Medical School
The cause of Unremitting head and neck pain in some patients may be compression of the lesser and greater occipital nerves by the posterior cervical muscles and their fascial attachments at the occipital ridge (where the base of the skull meets the spine) with subsequent local perineural inflammation (nerve inflammation). The resulting pain is typically in the sub-occipital and occipital location, and, via anatomic connections between extracranial and intracranial nerves, may radiate frontally to trigeminal-innervated areas of the head. Migraine-like features of photophobia (light sensitivity) and nausea may occur with frontal radiation.
Occipital allodynia (a super sensitivity to pain) is common, as is spasm of the cervical muscles. Patients with Unremitting head and neck pain may comprise a subgroup of Chronic Migraine, as well as of Chronic Tension-Type Headache, New Daily Persistent Headache, and Cervicogenic Headache.
Centrally acting membrane-stabilizing agents (local anesthetics), which are often ineffective for Chronic Migraine, are similarly generally ineffective for Unremitting head and neck pain.”
The researchers suggest extracranially-directed treatments such as occipital nerve blocks, cervical trigger point injections, botulinum toxin, and monoclonal antibodies may provide more substantial relief for unremitting head and neck pain; additionally, decompression of the occipital nerves from muscular and fascial compression is effective for some patients and may result in enduring pain relief.
Prolotherapy and the neck element in headaches
Do weakened ligaments in the neck cause an unnatural head posture which can cause headaches? Can strengthening these neck ligaments resolve the problem of chronic headaches and migraines by resolving the problem of cervical instability?
We are going to return to our research published in the journal Practical Pain Management, (4)
Prolotherapy is based on the theory that the cause of most chronic musculoskeletal pain is ligament and/or tendon weakness (or laxity). This retrospective pilot study was undertaken to evaluate the effectiveness of dextrose prolotherapy on tension and migraine headache pain and its associated symptoms.
Typical areas treated during Prolotherapy sessions for chronic headaches and neck pain are the base of the skull, cervical vertebral ligaments, posterior-lateral clavicle, where the trapezius muscle attaches, as well as the attachments of the levator scapulae muscles. Because there is an anesthetic in the solution, generally the neck or headache pain is immediately relieved. This again, confirms the diagnosis both for the patient and the physician.
- Prolotherapy, by strengthening cervical ligaments and tendons, treats very common trigger and pain locations of the posterior neck that can cause headaches.
- Prolotherapy will likely become an increasingly useful treatment for aging patients who experience an increase in cervical pain as a trigger for tension and migraine headache pain.
- 15 patients
- Of the 15 patients, five reported daily tension or migraine headaches.
- Another five participants experienced three to six tension or migraine headaches per week.
- Taken together, 66% of study participants had tension or migraine headaches multiple times each week.
- All study participants experienced headaches at least monthly prior to treatment with Prolotherapy
- After Prolotherapy treatments to the cervical spine, 60% reported the frequency of their headaches as less than once per month
- Only one patient continued to have daily headaches, although all respondents reported a decrease in the level of pain overall.
- Intensity Level and Length of Headaches
- Patients were asked to rate the intensity level of their headaches prior to receiving Prolotherapy and after their last Prolotherapy treatment, using a scale of 1 to 10 (1 being non-noticeable and 10 being severe).
- Prior to treatment, 67% reported a pain level of 10 out of 10.
- The remaining 33% of study participants rated their pain between 8 and 9 out of 10.
- All of the participants reported that their pain was at least 8 out of 10 on the pain scale prior to Prolotherapy treatment.
- Following treatment, significant decreases in intensity level were noted for 100% of the patients.
- Forty-seven percent were able to state that the intensity level following treatment was at level 1.
- Patients were asked to rate the intensity level of their headaches prior to receiving Prolotherapy and after their last Prolotherapy treatment, using a scale of 1 to 10 (1 being non-noticeable and 10 being severe).
Light sensitivity in the study patients
Sensitivity to light is a common complaint associated with tension or migraine headaches. Study participants reported on light sensitivity both prior to and following completion of the
Prolotherapy treatments, rating them on a scale of 1 to 10 (with 10 being the most severe).
- Sixty-seven percent reported a 10 out of 10 light sensitivity prior to treatment.
- After Prolotherapy, 67% reported sensitivity levels of 1, indicating very little sensitivity to light during a headache.
- Improvement continued for most patients, with 73% reporting reduced sensitivity that had at least somewhat continued well after final treatment. (On average, 22 months after their last Prolotherapy treatment.)
Our study followed patients, on average, 22 months after their last Prolotherapy treatment and all 100% still had benefit.
Clinically significant improvements were reported including:
- decreased headache intensity level,
- number of associated symptoms and light sensitivity in patients with tension and migraine headache pain.
Summary and contact us. Can we help you? How do I know if I’m a good candidate?
We hope you found this article informative and it helped answer many of the questions you may have surrounding Ehlers-Danlos Syndrome Headaches and Migraines. Just like you, we want to make sure you are a good fit for our clinic prior to accepting your case. While our mission is to help as many people with chronic pain as we can, sadly, we cannot accept all cases. We have a multi-step process so our team can really get to know you and your case to ensure that it sounds like you are a good fit for the unique testing and treatments that we offer here.
1 Levy AR, Nnam M, Gudesblatt M, Riley B. An Investigation of Headaches in Hypermobile Ehlers-Danlos Syndrome. Ann Psychiatr Clin Neurosci. 2020; 3(3): 1034.
2 Malhotra A, Pace A, Ruiz Maya T, Colman R, Gelb BD, Mehta L, Kontorovich AR. Headaches in hypermobility syndromes: A pain in the neck?. American Journal of Medical Genetics Part A. 2020 Sep 17. [Google Scholar]
3 Puledda F, Viganò A, Celletti C, Petolicchio B, Toscano M, Vicenzini E, Castori M, Laudani G, Valente D, Camerota F, Di Piero V. A study of migraine characteristics in joint hypermobility syndrome aka Ehlers–Danlos syndrome, hypermobility type. Neurological Sciences. 2015 Aug 1;36(8):1417-24. [Google Scholar]
4 Hauser RA, McCullough H. Dextrose Prolotherapy for recurring headache and migraine pain. Practical Pain Management. 2009:58-65. [Google Scholar]
5 Steilen D, Hauser R, Woldin B, Sawyer S. Chronic neck pain: making the connection between capsular ligament laxity and cervical instability. The open orthopaedics journal. 2014;8:326. [Google Scholar]
6 Krøll LS, Hammarlund CS, Westergaard ML, Nielsen T, Sloth LB, Jensen RH, Gard G. Level of physical activity, well-being, stress and self-rated health in persons with migraine and co-existing tension-type headache and neck pain. The journal of headache and pain. 2017 Dec 1;18(1):46. [Google Scholar]
7 Varatharajan S, Ferguson B, Chrobak K, Shergill Y, Côté P, Wong JJ, Yu H, Shearer HM, Southerst D, Sutton D, Randhawa K. Are non-invasive interventions effective for the management of headaches associated with neck pain? An update of the Bone and Joint Decade Task Force on Neck Pain and Its Associated Disorders by the Ontario Protocol for Traffic Injury Management (OPTIMa) Collaboration. European Spine Journal. 2016 Jul 1;25(7):1971-99. [Google Scholar]
8. Blake P, Burstein R. Emerging evidence of occipital nerve compression in unremitting head and neck pain. The journal of headache and pain. 2019 Dec;20(1):76. [Google Scholar]