Head and Neck Concerns in EDS/HSD
We have recently posted information on headaches from our neurological headache expert, Dr. Todd Rozen. Headaches are a significant and common contribution to the pain suffered daily by patients with EDS/HSD; however, you would be right to think that there are many different causes to headaches that originate not only within the skull but also associated with neck issues as well. We will summarize some common ones here.
First, let us start with the head, and intracranial conditions. Those that are more common in EDS/HSD can usually be identified by presenting features. Is there a certain position that makes it worse? Is there a trigger such as poor sleep, stress, noise, light, too much or too little caffeine, alcohol, etc? These can lead to migraine headaches for some people. Migraines are an episodic disorder that can also be associated with nausea and thought to be caused by a neuronal (nerve) dysfunction with 4 phases: premonitory, aura (visual disturbance), headache, and postdrome (nausea, fatigue). In most instances there is an inherited basis that is likely complex – similar to the complex genetic nature of hEDS/HSD. Sensitization can make features of migraines worse with time if they go unaddressed. It’s also important to note, as Dr. Rozen mentioned there are other types of headaches common in hEDS/HSD. These include New Daily, Cervicogenic, and Tension type Headaches. “Coat Hanger” Headaches can happen in patients with POTS. An anatomical problem that can occur within the skull is Chiari malformation. This can be seen in patients with hEDS/HSD at a younger age than the general population and occurs when a portion of the brain (brainstem / cerebellum) moves down and out of the opening to the spinal canal called foramen magnum. Symptoms include headache with cough, dizziness, difficulty speaking, and unsteady gait; diagnosis is confirmed by imaging, and surgery may be required for treatment. Temporal Mandibular Joint Disorders (TMD) can result from increased stress (hypermobility, psychological, or postural) on the jaw joint. Associated symptoms of TMD can be tinnitus, toothache, earache, limited mouth opening, and trouble swallowing. Treatment includes physical therapy (PT), TMJ exercises, stress management, and dental appliances. Pseudotumor Cerebri, also known as Idiopathic Intracranial Hypertension, is another cause of headaches, more frequent in females - especially if overweight and can also have visual disturbances, tinnitus, and nausea connected to it. There is still much more to learn about this condition, but treatment may include weight loss, and surgical placement of a shunt to reduce the pressure of spinal fluid (CSF) within the skull.
Moving down to the neck and cervical spine there several conditions that can happen in hEDS/HSD. Instability of joints connecting the head to neck can potentially lead to symptoms of headache, neck pain, limb weakness / poor coordination. Atlantoaxial (AAI) or Craniocervical Instability CCI) diagnosis is made by imaging, dynamic XR, or upright MRI, and treatment can include bracing, PT, or surgery depending on the severity. Neck muscle injuries are also be seen and can occur with some frequency especially the more active and hypermobile a patient is. Come muscle groups include capitis, cervicis, frontalis, suboccipital, and upper trapezius, among others. Pain can be noted with head movement, rotation, laying head on pillow, stiffness, numbness, and is best treated with PT. Some types of massage and craniosacral (along with heat) therapy can be useful as well. CSF leaks are less common, but may happen in a patient with hEDS/HSD who has had lumbar puncture or other intervention or injury breaching the dura (tissue enclosing spinal fluid). Headaches are the most likely symptom, worse with cough, and can be associated with vision changes, tinnitus, dizziness, and nausea. A blood patch may be used for treatment. Tethered cord, like CSF leaks, can affect the entire spine. This happens rarely but occurs because of abnormal spinal cord attachment to surrounding tissue limiting cord movement and causing stretching; when present it can lead to debilitating symptoms of low back pain, urinary incontinence, leg weakness, and sensory loss. Surgery is the best treatment and needs to be conducted with an experienced specialist. As always, discuss with your primary care doctor if you have new features or changes in your symptoms related to head, neck and spine. The sooner any of these are diagnosed, the better.
What similar conditions have you suffered from? What has helped? Share in the comment section below!
Author: Dacre Knight, MD, MS; Olga Fermo, MD
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I haven’t been diagnosed with non of this so far but my MRI shows Magna Cisterna or Arachnoid Cyst that was discovered when my vague symptoms started 2017. I had blurry vision, shining spots, imbalances, dizziness, sensory issues, uncontrollable episode of swallowing and the HR alterations. No body has kept measuring it but there’s no significant tonsil ectopia in my last brain MRI. However, I’m now waiting for a vascular surgeon from CT for his opinion specially for the abdominal pain to check any compression etc. I only have 3 X rays standing done by my chiropractor when I tried to see what could help but I never got to start treatment since I got deteriorated or worse (ER in out admission) with symptoms that are attributed so far with HSD and POTS. I do see in my X Ray some tilting but I also had severe scoliosis surgery at 17yo so I dunno if this is all connected. I’m still working on the etiology of my situation. Thanks for the valuable information. Blessings and healing for all 🙏🏻❤️
Here’s a picture of me attempting to illustrate my situation back in 2017. I’m no artist so excuse my anatomical inaccuracy lol 😆
I would like to see prolotherapy added to possible treatments list. While it is still considered an alternative treatment. prolotherapy can tighten ligaments that have become stretched, the ligaments that hold joints in place. I am not a practitioner of any kind but know that tightening cervical joints 4-7 stopped my night awakenings when joint movement was needed to allow full blood flow to return. Cervical 1 and 2 are actually made up mainly of ligament, but they are difficult to treat. A Digital Motion Xray can determine which cervical joints are hypermobile and which extend the most. A chin to the chest works as a one-time realignment.
Finally, prolotherapy is a regenerative medicine technique. By poking the ligament with a needle and sugar water, the internal healing system is turned on. Notably it can take 10 days for a fix.
I'm diagnosed with hEDS, uncertain significance cEDS, mEDS, TAAD syndrome, and Loeys-Deitz. I suffer from unbelievable amount of symptoms such as Autonomic Small Fiber Neuropathy, POTS Syndrome, Positional migraines, silent migraines w/ beautiful auras etc. I had a stroke from a tear in the Vertebrobasilar artery that over clotted due to a blood clotting disorder that was not caused by trauma. Making everything worst by losing strength in my ligaments resulting multi-direction partial shoulder dislocation doing anything, unstable spine, and inability to turn my head all the way to one side. After getting covid (I was a long hauler before covid long hauler existed:) made a crappy problem even worst.
I added x-ray photos of my neck where the radio findings was: Straightening of the normal cervical lordosis, may be positional. No findings of dynamic instability on flexion-extension views. The vertebral body heights are maintained. The disc heights are preserved. No significant osteophytosis or facet arthropathy. My response was I disagreed with the findings while acknowledging I don't know anything about x-rays just what I feel. Judge for yourself but radio still wins. lol When I was younger being hyper mobile was fun it was my super power but now that I older I keep saying "Take it back"
edsasfnf5….I too enjoyed the 'tricks' I could do and had no trouble until my old age when my flexibility began to cause problems. The latest is my jaw which creaks when chewing! I would love to have someone do some scientific comparisons of changes in the components of cell making that could point us toward supplements. Collagen seems to help.
Re Xrays … Chiropractors have something called a digital motion Xray that measures the hyper-extensions of various joints. It can be read my certified radiologists too. My cervical spinal DM Xray showed that Cervical 4 was the worst stretcher, so I took that to a Regenerative Medicine doctor who tightened the ligaments of Cervicals 4-7. Today, I no longer have the issue with an occasional slow flow of blood to my brain. A chin to the chest helps too…. like a palace guard posture!
Regenerative Medicine Drs. do prolotherapy, a procedure that tightens stretched out, "lax", ligaments. A treatment on the ligaments attached to my SI Joint allows me to walk properly again and the leg stopped subluxing when I moved it a certain way. The Dr. said I may need to have it done again, as the joint can still hyperextends, but it has been 5 years so far.
Hope you can find some answers too
@rjmtwit…I have a family member that has the same jaw issue. I read articles of them being able to make a mouse hypermobile but have not released details on the find as of yet still a ways. The way I understood what they found is that it is a production problem that's why they couldn't find a gene but will have to wait for the research release. Cortney Gensemer, a postdoctoral scholar, is one of the researchers that may be able to answer your question in the near future. Also there is some other researchers that are working on the same area. If you go to the Statnews website there is an article on 12/12/2022 ehlers-danlos-syndrome-patients-turned-researchers Titled "Revenge of the gaslit patients: Now, as scientists, they’re tackling Ehlers-Danlos syndromes." Even though they are working on EDS there research would also answer other connective tissue issues across the board. (My opinion)
Thanks for the chiro info will definitely ask about the digital motion X-ray. I have vascular connective tissue issues on top of hypermobile along with an anesthesia allergy and a blood clotting disorder. (Mouth full I know) So I'm not cleared for any surgeries unless it is a threat to do nothing. I'm to the point I will try anything to improve but I avoid doing anything on maybes. So thank you! for sharing your experience that makes me feel more comfortable looking for a Regen dr and getting my ligaments tightened. It would me great if my ligaments in my neck are tighten and it stops my positional migraines as well as stop my imbalance. 🙂
@edsasfnf5, I noticed that you wished to post a URL to an article with your post. You will be able to add URLs to your posts in a few days. There is a brief period where new members can't post links. We do this to deter spammers and keep the community safe. Clearly the link you wanted to post is not spam. Please allow me to post it for you.
– Revenge of the gaslit patients: Now, as scientists, they’re tackling Ehlers-Danlos syndromes https://www.statnews.com/2022/12/12/ehlers-danlos-syndrome-patients-turned-researchers/
@colleenyoung Thank you for posting the URL. I believe keeping up with new research is very important to future treatments and understanding so thank you for allowing me to share. But I do completely understand about deterring spammers and keeping the community safe. Thank you!
Samantha, CCI … craniocervical instability can cause all kinds of symptoms but I would like to add a bit about diagnosing and treating CCI that are still part of 'complementary' medicine, but have worked for me. First, the digital motion Xray that can be done at some chiropractors offices and read by licensed radiologists. They are a great diagnostic technique. My own SI Joint hypermobility extended up my spine to affect the cervical joints. The DMX can measure the hyperextension of each joint. For me joint 4 showed the worse extension, although others did measure excess mobility. My Dr. elected to do prolotherapy on the ligaments in 4-7. It worked and I no longer feel the blood flow into my brain diminished and the awful feelings that causes. Nor do I have to "do the chin thing". That means that by moving my neck into a position the palace guards stand in. A chin to an up straight neck can realign those cervical joints.
Hypermobile joints probably cannot be completely fixed, but prolotherapy on the internal and connecting ligaments of the joints will hold them in place and make living life a whole lot better.
Your symptoms sound very much like mine. I spent years trying to get properly diagnosed and I finally did at 51 yo. So grateful. I’m feeling so much better now. So there’s light at the end of the tunnel.
I have hEDS (Hypermobile Ehlers Danlos Syndrome) and POTS (Postural Orthostatic Tachycardia Syndrome). They go hand in hand often enough and that’s the case with me.
First step would be to get to a cardiologist, who’ll do a tilt table test, an EKG, and some other tests. That’ll and questions you answer will easily help determine POTS or not. They can also help diagnose hEDS, but you have to make sure they understand the symptoms. They should.
I’m complementing my doctor’s treatment plan (prescription meds, compression garments , supplements), healthy eating (dairy free, gluten free, whole foods), with alternative treatments. I’ve recently been addressing a possible chronic Epstein Barr viral infection. EBV’s been discussed as being a culprit for triggering POTS and the associated dysautonomic symptoms. I chose acupuncture and traditional Chinese medicine. Specifically, my practitioner suggested Lightning Pearls (Su He Tang Jiawei II; Classical Pearls brand). The extreme fatigue I’ve felt over the last few years has FINALLY lifted!!!
So good luck, feel better, stay the course, and please reach out with any questions. You have a lot of support. Get ready to feel well.