What is CCI? Craniocervical Instability

by Chris Centeno, MD /

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What is CCI? This is a term that means craniocervical instability. It’s a problem that impacts many people with neck pain and headaches as well as a host of other symptoms. Let’s dig in.

What is CCI Illness?

cci instabilityCCI means that the strong ligaments that hold the head to the upper neck are lax or loose (1). These ligaments include the alar, transverse, accessory, apical dens, and others. I have outlined these ligaments in yellow in the image here.

This isn’t an illness like the flu or usually due to an infection as much as it’s a mechanical problem. If these ligaments are loose or injured, the upper neck joints, nerves, muscles, and tendons take a beating and begin to cause pain and a host of weird symptoms.

What is CCI?

Cervical means your neck and instability means that a joint or spinal segment is moving around too much (2). Realize that every joint in your body has ligaments that constrain its movement. When a ligament is stretched out or torn, that joint moves too much in the wrong directions (instability) and that can wreak havoc on the joint and cause arthritis.

In terms of the neck, you have 7 different numbered levels from C0 (skull) to C7 (base of the neck) and all of these have ligaments and other structures that keep them stable. So instability happens when those ligaments are too loose (like in Ehler-Danlos) or normal ligaments have been injured. Given that the spine protects the spinal cord and nerves, these structures can also get irritated or damaged.

For more information, see my video below:

What Causes Craniocervical Instability?

Loose ligaments where the skull meets the spine (CCI or craniocervical instability) can be caused by a number of things. The two biggest causes are naturally loose ligaments or trauma. Let’s review.

There are several diseases that people are born with that can lead to loose ligaments. The most commonly diagnosed is Ehlers Danlos Syndrome or EDS. This is when the body produces too much of a specific type of collagen which causes the ligaments to be too stretchy. CCI is much more common in these patients, especially as they age (13).

Another cause of craniocervical instability is trauma (3). These patients generally have normal ligaments, but trauma in an EDS patient can be a double risk for developing CCI. This can include a car crash, a hit on the head, falling on the head, manipulation of the neck, or other causes.

What are the Symptoms of CCI?

The symptoms of CCI include headaches, usually upper neck pain near the skull, dizziness or imbalance, visual disturbances, brain fog, rapid heart rate, and others. Let’s take each of these:

  1. Headaches can be caused by a number of things including upper neck joints like C0-C1, C1-C2, or C2-C3 that get injured or arthritic, irritated occipital nerves at the back of the skull, irritated spinal or cranial nerves, (5,6), or tendons pulling on the covering of the brain (7).
  2. Upper neck pain near the skull is usually caused by the upper neck joints, muscles, and tendons in this area getting beat up by the instability.
  3. Dizziness or imbalance is a feature related to the fact that the upper neck is a major contributor to balance (4). The upper neck provides position sense that has to be coordinated with balance information from the eyes and inner ear.
  4. Visual disturbances can happen because the upper neck supplies information to the brain to guide eye position and vice versa (9).
  5. Brain fog is something that has long been reported in patients with upper neck disorders and may be linked to the Barre-Lieou Syndrome (8) which involves irritation of the upper neck arteries or sympathetic nerves. A brain injury also needs to be ruled out if the patient was hit on the head.
  6. Rapid heart rate can happen as the vagus nerve gets irritated by the extra motion where the skull meets the neck.

Diagnosing Craniocervical Instability

First, patients with craniocervical instability usually fall into two categories. The minority of patients have a huge dislocation of one of the upper neck bones that is often diagnosed on x-ray, CT Scan, or MRI (10). This type of CCI is easier to diagnose, hence it’s usually picked up early. However, the majority of patients with this problem have some or all of the symptoms above, don’t have a seriously dislocated bone, and often struggle to get a diagnosis for months or years.

The patients who have symptoms without severely dislocated upper neck bones usually get diagnosed by one of the following types of imaging:

  • Specialized neck MRI using a head coil. To learn more, see my video below:
  • Upright MRI:
  • DMX or movement-based x-rays:

There are also several different measurements that can be used to make the diagnosis that patients may hear about:

  • Grabb-Oakes measurement:
  • Powers Ratio:

Treatments for CCI

First, as discussed above, most patients with CCI do not have severe dislocations of the upper neck bones that require immediate surgery. When this does happen, this requires immediate surgical fusion (11). Instead, many patients have smaller amounts of instability that can cause severe disability, but can often be managed with:

  • Upper cervical low force chiropractic (NUCCA)
  • Physical therapy
  • Bracing
  • Curve restoration therapy (CBP)

However, if these options don’t work, the next level of treatment based on my experience is cervical ligament injections (12) or upper cervical facet injections. However, when patients don’t respond to this kind of care, the PICL procedure that involves direct injection of the damaged ligaments (alar. transverse, and accessory) with the goal of healing the damage is another option. See my video below for more information:

There are many different surgical fusion options that involve bolting together upper neck bones to each other or the skull. However, in my experience, these procedures have a very high complication rate. Common problems post-surgery that I have noted:

  • Eventual arthritis and pain above or below the fusion
  • Misplaced screws damaging joints or nerves
  • Failure to fuse (to grow bone between a joint)

Having said that, for the right patients, fusion may be the only option once all other less invasive procedures have failed. In addition, in selected patients, it can be life-changing.

The upshot?  What is CCI? Craniocervical instability for many patients is a confusing tour through a medical system that is often ill-equipped to make the diagnosis. In addition, it’s also confusing to find an effective treatment. I hope this deep dive into this diagnosis helps CCI patients understand what’s happening and how best to find answers.

____________________________________________

References:

(1) Offiah CE, Day E. The craniocervical junction: embryology, anatomy, biomechanics and imaging in blunt trauma. Insights Imaging. 2017;8(1):29–47. doi:10.1007/s13244-016-0530-5

(2) Klein GN, Mannion AF, Panjabi MM, Dvorak J. Trapped in the neutral zone: another symptom of whiplash-associated disorder?. Eur Spine J. 2001;10(2):141–148. doi:10.1007/s005860100248

(3) Yang SY, Boniello AJ, Poorman CE, Chang AL, Wang S, Passias PG. A review of the diagnosis and treatment of atlantoaxial dislocations. Global Spine J. 2014;4(3):197–210. doi:10.1055/s-0034-1376371

(4) Thompson-Harvey A, Hain TC. Symptoms in cervical vertigo. Laryngoscope Investig Otolaryngol. 2018;4(1):109–115. Published 2018 Nov 28. doi:10.1002/lio2.227

(5) Hall T, Briffa K, Hopper D. Clinical evaluation of cervicogenic headache: a clinical perspective. J Man Manip Ther. 2008;16(2):73–80. doi:10.1179/106698108790818422

(6) Antonaci F, Bono G, Chimento P. Diagnosing cervicogenic headache. J Headache Pain. 2006;7(3):145–148. doi:10.1007/s10194-006-0277-3

(7) Enix DE, Scali F, Pontell ME. The cervical myodural bridge, a review of literature and clinical implications. J Can Chiropr Assoc. 2014;58(2):184–192.

(8) Zeigelboim BS, Fonseca VR, Mesti JC, Gorski LP, Faryniuk JH, Marques JM. Neurotological Findings at a Health Unit for Adults with Cervicalgia. Int Arch Otorhinolaryngol. 2016;20(2):109–113. doi:10.1055/s-0036-1572563

(9) Ischebeck BK, de Vries J, Van der Geest JN, et al. Eye movements in patients with Whiplash Associated Disorders: a systematic review. BMC Musculoskelet Disord. 2016;17(1):441. Published 2016 Oct 21. doi:10.1186/s12891-016-1284-4

(10) Radcliff K, Kepler C, Reitman C, Harrop J, Vaccaro A. CT and MRI-based diagnosis of craniocervical dislocations: the role of the occipitoatlantal ligament. Clin Orthop Relat Res. 2012;470(6):1602–1613. doi:10.1007/s11999-011-2151-0

(11) Joaquim AF, Patel AA. Craniocervical traumatic injuries: evaluation and surgical decision making. Global Spine J. 2011;1(1):37–42. doi:10.1055/s-0031-1296055

(12) Centeno CJ1, Elliott J, Elkins WL, Freeman M. Fluoroscopically guided cervical prolotherapy for instability with blinded pre and post radiographic reading. Pain Physician. 2005 Jan;8(1):67-72. https://www.ncbi.nlm.nih.gov/pubmed/16850045

(13) Castori M, Voermans NC. Neurological manifestations of Ehlers-Danlos syndrome(s): A review. Iran J Neurol. 2014;13(4):190–208.

Category: Neck Procedures

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7 thoughts on “What is CCI? Craniocervical Instability

  1. Dan Semrad

    Excellent post Dr. Centeno. Putting the CCI description, diagnosis and treatment information all in one place. It is very helpful. I know how difficult it used to be for those of us with CCI injuries to find such information just few years back. I’ve been there. Here it is all in one place. Thank you for putting this together.

  2. Beth Istre

    Would this condition also cause shoulder pain and pain down to and including the elbow. Terrific pain about 1/2 way between shoulder and elbow.

    1. Regenexx Team

      Hi Beth,
      More commonly, shoulder/elbow pain would involve lower cervical nerves. Please see: https://regenexx.com/blog/a-colorado-regenexx-patient-review-pain-free/ and https://regenexx.com/blog/why-is-my-arm-throbbing/ To determine what’s going on in your particular case, we’d need more information. To do that, please give us a call at 855 622 7838, or submit the Candidate form here: https://regenexx.com/conditions-treated/spine/

  3. Stacey Kaufman

    Great article about CCI. I was sustained CCI from a car accident, have many of the symptoms noted, plus many others. Surgeons do not understand nor do they even recognize this as injury or understand it’s associated symptoms, and they have no protocol for treatment of lateral laxity from trauma. I will be eternally grateful for the doctors and staff at the Centeno-Schultz Clinic for developing the PICL procedure and for their compassionate and professional care. While I have a long road of healing ahead of me, and while we may not as yet have stats on how much healing can be attained or how long it will hold or how susceptible we are to re-injury if we don’t attain 100% healing so may need ongoing care, I am improving. Thank you!

    1. Regenexx Team

      Great to hear, Stacey!

  4. Melissa

    Just another issue I’ll have to talk to Dr. Jackson about, I have a majority of the symptoms. Along with my shoulder, knee, hip, and ankle, I should keep him pretty busy. I always find Dr. Centeno’s blogs very informative, but perhaps I’m self-diagnosing too much without an actual consultation LOL

    1. Regenexx Team

      Hi Melissa,
      Thanks, Melissa. Dr. Jackson is a great Doctor, so great to keep him busy! But we only treat Craniocervical Instability in CO. You may find some helpful info at the CCJ Instability Institute: https://ccjinstability.com/

Chris Centeno, MD

Regenexx Founder

Chris Centeno, MD is a specialist in regenerative medicine and the new field of Interventional Orthopedics. Centeno pioneered orthopedic stem cell procedures in 2005 and is responsible for a large amount of the published research on stem cell use for orthopedic applications.
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