Head and Neck Concerns in EDS/HSD

Sep 30, 2022 | Samantha Campbell | @samanthacmaa | Comments (17)

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

Interested in more newsfeed posts like this? Go to the Ehlers-Danlos Syndrome blog.

Colleen Young, Connect Director | @colleenyoung | Nov 2, 2023

In reply to @nial1n "I’m looking for peer reviewed research/studies/clinical literature regarding how hEDS can weaken bones OR the joints..." + (show)

@nial1n

I’m looking for peer reviewed research/studies/clinical literature regarding how hEDS can weaken bones OR the joints around the bone plates in the skull. 5 years I’ve had slight rocking back & forth of the right side temporal bone. Only I could hear the shifting, so was dismissed by the ever popular “that’s impossible” & “what you think is happening isn’t what’s happening”. It’s now evolved to the point of snapping so violently that it rattles my teeth, shakes my eyes & the sound is clearly audible outside of my body to anyone else in the room. When it snaps, it also pops my inner ear bone, sending pain thru my inner ear, down my jaw joint, down the side of my neck adjacent to my throat. On really bad days, when the bone is micro snapping all day on top of the more violent macro snapping that’s audible outside my body, the sharp constant pain in my inner ear is accompanied by the feeling of having cotton stuffed in my ear. It also snaps against surrounding plate joints, which puts force against them, causing shooting pains into my right eye when I bend over & a myriad of different headaches WHEN THE TEMPORAL BONE ROCKS OR SNAPS. This isn’t pain that exists unless the bone is moving. Meaning, none of these symptoms exist outside of this phenomenon; to blamed on other sources of pain. When I stay bedridden to keep this bone stable, these symptoms disappear. But, even rolling over can cause the slightest rocking or popping of the temporal bone, so this isn’t a sustainable “management of symptoms” or “cure”. It causes severe occipital migraines w/tachycardia, autonomic dysfunction (I stop breathing in my sleep w/the worst migraine episodes), weird changes to my vision; that feel like I’m not getting blood or enough oxygen to my brain. The only cure is for me to purposely snap it until it gets aligned enough that the migraine instantly disappears. Like a light switch being turned off. The snap of a finger! I can’t get any dr to believe me even though they can hear the very loud snapping of the temporal bone, behind my right ear. Not only is it excruciatingly painful (it feels like a broken bone, the same sort of deep, sharp ache around the outline of the bone joints), I’m also terrified that this bone is gonna get loose enough to irrevocably harm me, whether or not that’s “possible” in the general population. I mean, this is my skull. This can’t be good. Drs keep treating my cervical spine for the pain, (I just had a diagnostic procedure of a medial C2-C5 anesthetic block yesterday which actually triggered a migraine, triggered left side occipital & neck pain I DIDN’T have before & I felt 0 effects from the injection; might as well have been water). This does absolutely nothing to resolve the myriad symptoms that it’s causing, nor does it resolve the pain in the temporal bone joints & inner ear. They simply won’t accept it. I’m in desperate need of any credible sources regarding issues w/this specific skull bone; primarily in hEDS (my dx), but even in the general pop would be helpful. I’m stuck in a small town where absolutely no doctors know anything about this disease. I’m going into my 50s & I gotta say, it’s an extremely exhausting & hopeless situation. Reaching out to strangers publicly is my last straw. Thank you for any sources in advance.

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Hi @nial1n, I'm not a doctor, so take this with a grain of salt. You might consider talking to your doctor about temporomandibular joint (TMJ) disorders. Here's some information from Mayo Clinic:
https://www.mayoclinic.org/diseases-conditions/tmj/symptoms-causes/syc-20350941
According to the Ehlers-Danlos Society article "Mouth and jaw symptoms have been observed in all types of EDS patients." Read more here:
- Oral and Mandibular Manifestations in the Ehlers-Danlos Syndromes https://www.ehlers-danlos.com/2017-eds-classification-non-experts/oral-mandibular-manifestations-ehlers-danlos-syndromes/

nial1n | @nial1n | Nov 3, 2023

In reply to @colleenyoung "Hi @nial1n, I'm not a doctor, so take this with a grain of salt. You might..." + (show)

@colleenyoung, thank you for your reply & links. While I’d love to be able to speak to a Mayo EDS clinician, I don’t discount the knowledge of fellow EDS patients. We’ve had to become our own experts. I have suspected that the TMJoint is involved w/this. If not the tympanic bone. Possibly both. There’s several bones/joints that come off the temporal plate, especially in my problem area of right behind, inside & below my right ear. I have the dental manifestations (my teeth have 3 roots not 2 that are extremely long & the teeth are literally cracking & disintegrating w/out any cavities or other obvious, normal causes) & TMD symptoms. I have intermittent facial numbness & facial drooping on the right side w/negative stroke eval. So your links have merit. I have an ER visit follow up w/a hospital neuro, so hopefully this dr will listen to me & consider this research. God willing, he knows about EDS. It’s exhausting being complex, isn’t it? Thank you very much for giving me your time. I sincerely appreciate it.

Colleen Young, Connect Director | @colleenyoung | Nov 3, 2023

In reply to @nial1n "@colleenyoung, thank you for your reply & links. While I’d love to be able to speak..." + (show)

@nial1n, to consult with Mayo EDS experts, please submit an appointment request here http://mayocl.in/1mtmR63

Do you know about Dr. Knight and the EDS Clinic at Mayo? https://connect.mayoclinic.org/blog/ehlers-danlos-syndrome/tab/history/#ch-tab-navigation

jrgraves1 | @jrgraves1 | Dec 20, 2023

Hello, my son 17yrs, has had a headache that has not gone away for 2 years. it goes up and down but is never gone. He was recently diagnosed with hEDS. I am trying to find treatment options or even a support group to help him cope. A 15–17-year-old boy is not prepared to deal with this. I have been pushing for 2 years to figure out why he has had this headache, I am hopeful this new diagnosis may open doors to help. Any guidance or support you can give me would be much appreciated. We live in southern California.

Colleen Young, Connect Director | @colleenyoung | Dec 21, 2023

In reply to @jrgraves1 "Hello, my son 17yrs, has had a headache that has not gone away for 2 years...." + (show)

Hi @jrgraves1, this must be so hard as a mom. You've come here to a good place to start. If you're not familiar with it, you'll also want to know about The Ehlers-Danlos Society https://www.ehlers-danlos.com/

It's a wealth of information and support, including a directory of professionals.
- Healthcare Professionals Directory https://www.ehlers-danlos.com/healthcare-professionals-directory/

You may wish to join the discussion in the Bones, Joints & Muscles support group. Start here in this discussion:
- Ehlers Danlos Syndrome (EDS) & HSD, calling all types!
https://connect.mayoclinic.org/discussion/edshsd-calling-all-types/

julievandenh | @julievandenh | Jan 11 5:01pm

In reply to @jrgraves1 "Hello, my son 17yrs, has had a headache that has not gone away for 2 years...." + (show)

I'm 55 and have had headaches my whole life. My sister was diagnosed with hEDS and has had multiple surgeries to correct joint instabilities. I haven't been diagnosed, but have almost all of the symptoms of the disorder. I saw a dietician to get my dehydration (which causes headaches) under control and physical therapists for lax joints. The last therapist was familiar with eds and various treatments. I was having issues holding up my head at my desk job and thought it could be another reason for my headaches. She gave me stretches for my neck, strengthening exercises, did dry needling in my trapezius muscles. She also did a technique called counterstrain. I'm not sure which of these activities helped the most, but I only get headaches on occasion and they're a lot less debilitating than they used to be. I found that a good physical therapist is amazing. Good luck!

ersingh | @ersingh | 4 days ago

Has anyone developed nystagmus or other eye movement problems from their hEDS (not sure if it is nystagmus, intrusive saccades, or some weird intermittent strabismus)? I am being worked up for weird neuro symptoms but my imaging doesn't show a frank chiari malformation and only mild cervical instability which the neurosurgeon doesn't think is causing my symptoms. I am waiting to see a neuro-ophthalmologist but I find people my doctors, even specialists, are not familiar with some of the symptoms that hEDS can cause in their fields. I like to have some idea of whether hEDS might be the cause before I see specialists so they don't ignore unusual things or go down insane diagnostic pathways for other crazy diagnoses. Over the years, various specialists have told me they thought I might have everything from ALS to lupus before I was finally diagnosed with hEDS and MCAS in my mid 40s (2 diagnoses that weren't even on my radar when they were finally made).