Living with Ehlers-Danlos Syndrome, Type III – Hypermobile

Have You Ever Felt Like A Bobbly-Headed Dashboard Dog?

More than 30 years ago when I was first married, my husband and I lived in a tiny, four-room apartment above his cousin’s garage. One of the rooms was a bathroom, thankfully. But since it was upstairs, the water pressure was non-existent. So, to take a bath, we sat on a one-step footstool in the middle of the tub with a 32-ounce plastic cup from a convenience store. We slowly filled the cup and poured the water on our heads and bodies, one cup at a time.

Once when I was taking a bath, my husband was in the bathroom talking to me. I leaned forward to fill the cup, and the footstool flipped out from under me. It tossed me in the air, and I landed with a thud on the bottom of the tub. It was jarring, to say the least. As I sat there dumbfounded, trying to understand what just happened to me, my husband was dying laughing, so hard that he could barely catch his breath. I looked at him for a clue as to what was so funny. He gaspingly sputtered that, when I fell, my head bounced around “like a bobbly-headed dashboard dog.”

Do you know what he was talking about? Today, bobble-head figurines come in the likenesses of nearly every celebrity and fictional character. You can even get a bobble-head made in your own image. But the original bobble-head creatures were dogs that would sit on vehicle dashboards. I recently discovered that one in 15 people with EDS3 have the signature feature in common with these little plastic ornaments. Apparently, because the connective tissue surrounding our cervical spines does not sufficiently support the weight of our heads, surgeons have described the condition as “bobble-head.” It is a funny name for a painful, and in many cases, humorless problem.

According to The Pain Relief Foundation,

“Craniocervical Instability (CCI), also known as the Syndrome of Occipitoatlantialaxial Hypermobility, is a structural instability of the craniocervical junction which may lead to a pathological deformation of the brainstem, upper spinal cord, and cerebellum. It primarily occurs in patients with Ehlers-Danlos Syndrome and other hereditary disorders of connective tissue.”

[I do want to mention, though, that CCI is NOT the same as Bobble Head Syndrome (BHS). BHS is usually found in three-year-old children and is often caused by cysts and other non-EDS issues.]

A number of resources stipulate that an MRI must be conducted with the patient in the upright position. There are several reasons for that which can be explored outside of this website. CCI can be caused by damage such as whiplash, or by something as seemingly benign as stretch injuries from repeatedly turning your head.

Because of the serious problems caused to the brain and spinal fluid, people with EDS3 should see a neurologist or other specialist for examination and diagnosis.

Since I am just discovering this, I am in no position to expound upon the information I am finding. So, the following is a reprint from The Pain Relief Foundation’s website.

The symptoms of CCI are:

  • a heavy headache: a constant to near constant headache that can be described as feeling like the head is too heavy for the neck to support (feeling like a “bobble-head”);
  • a pressure headache: an impairment of cerebral spinal fluid (CSF) flow causes intracranial pressure which would be aggravated by “valsalva maneuvers,” such as yawning, laughing, crying, coughing, sneezing or straining;
  • Dysautonomia: brainstem compression can lead to a dysfunctional autonomic nervous system (the involuntary regulator of all body functions). Symptoms of this include, but are not limited to:
    • tachycardia (rapid heart);
    • heat intolerance;
    • orthostatic intolerance (low blood pressure when standing);
    • syncope (fainting);
    • polydipsia (extreme thirst);
    • delayed gastric emptying; and
    • chronic fatigue.

Other symptoms include:

  • neck pain;
  • central or mixed sleep apnea;
  • facial pain or numbness;
  • balance problems;
  • muscle weakness;
  • dizziness and vertigo;
  • vision problems;
  • reduced gag reflux and difficulty swallowing;
  • ringing in the ears and hearing loss;
  • nausea and vomiting;
  • impaired coordination;
  • downward nystagmus (irregular eye movements);
  • paralysis; and more.

I had many of the above-listed symptoms already, but I recently slipped in the shower and hit the back of my head on the ceramic tile above the tub, in my apartment, by myself. I blacked out very briefly and came to when I experienced that familiar jarring sensation as I landed flatly on the bottom of the tub once again. The Emergency Department ordered a CT-scan and found “no damage” from the accident. But they were not looking for evidence of CCI. They were looking for brain bleeds, evidence of a possible aneurism, cracked skull bones, etc. Although their neurologist reviewed my scan, they recommended that I see a neurologist. They found that most of my frontal lobe was atrophied. From the little I have seen, I do not believe that could be a result of CCI. But what do I know? I know that I am trying to get an appointment with a neurologist! (If I were to highlight each symptom I have been battling for years, the whole page would be bright.)

↑ Back to Top