Craniocervical instability (CCI) is a condition that affects the upper part of the spine, where the cranium (skull) and cervical spine (neck) meet. It is commonly seen with hypermobility with a range of symptoms that can significantly impact a person’s quality of life. This blog is about understanding CCI and hypermobility, how they are diagnosed, and the common treatments available.

diagram of the upper neck showing different portions of the anatomy

Courtesy of TrialEx

What Is Craniocervical Instability (CCI)?

Craniocervical instability is a structural disorder where the ligaments and connective tissues that support the junction between the skull and the neck are weakened or damaged. This instability can lead to excessive movement between the skull and the spine, causing compression or stretching of the spinal cord, brainstem, and nerves.

Symptoms Of CCI

The most common symptoms of CCI are:

  • Severe headaches, particularly at the base of the skull
  • Neck pain
  • Dizziness or vertigo
  • Visual disturbances
  • Tinnitus (ringing in the ears)
  • Dysautonomia (autonomic nervous system dysfunction)
  • Cognitive issues like brain fog
  • Sleep disturbances

As you can see, these are common and you don’t have to have CCI to have these symptoms. This is one of the reasons why CCI is missed often. In my opinion, anyone with joint hypermobility should be assumed to have craniocervical instability. The rehabilitation for their joint hypermobility should exclude mobilization or manipulation of the upper cervical spine.

How Is Hypermobility Related To CCI?

Hypermobility is a connective tissue disorder that impacts all joints. While there are different levels of hypermobility, from Ehlers Danlos Syndrome (EDS) to Hypermobility Spectrum Disorder (HSD) and beyond, all joints are somewhat impacted. The joint between the skull and neck is of no exception and I err for caution and stay clear of manipulating or mobilizing the upper cervical spine.

Does Hypermobility Contribute To CCI?

In individuals with hypermobility disorders, the ligaments and tissues that stabilize the craniocervical junction may be too lax, predisposing them to instability. This increased range of motion can exacerbate the symptoms and complications associated with CCI.

How Is CCI Diagnosed?

Craniocervical instability relies heavily on the clinician recognizing the chances of its existence by listening to the patient, recognizing that the individual has hypermobility, and then ordering the right studies.

Unfortunately, connective tissue disorders are not taught in professional schools and are considered to be rare findings which is not my experience!! I firmly believe this population is underserved and misdiagnosed regularly.

Clinical Evaluation

A thorough medical history and physical examination are critical first steps. Signs of hypermobility, assessing range of motion, and evaluating symptoms suggestive of CCI all lead to ordering further studies to confirm the diagnosis.

Imaging Studies

  • MRI (Magnetic Resonance Imaging)

Provides detailed images of soft tissues, allowing doctors to assess the condition of the ligaments, brainstem, and spinal cord.

  • CT (Computed Tomography) Scan

Detailed images of the bones and can help detect structural abnormalities.

  • X-rays

Particularly dynamic X-rays (taken in different positions) can show excessive movement at the craniocervical junction.

Specialized Tests

  • DMX (Digital Motion X-ray)

A specialized type of X-ray taken while the patient moves their head can reveal instability that static images might miss.

  • Upright MRI

Performed while the patient is in an upright position, an upright MRI shows how gravity affects the craniocervical junction, potentially revealing instability not seen in a traditional MRI.

Common Treatments For CCI And Hypermobility

The treatment for CCI and hypermobility often involves a multidisciplinary approach, including conservative management and, in severe cases, surgical intervention.

Conservative Management

  • Functional Mobility and Stability

This is primarily done by physical therapists, chiropractic, and occupational therapists but it is SO important to only see rehab clinicians who know how to treat hypermobility. What we do for the non-hypermobile population can hurt the bendy. Look for resources such as the EDS Society for the providers near you.

  • Bracing

Wearing a cervical collar can help limit neck movement and provide support but I only suggest that for moderate to severe cases. Wearing braces continuously can lead to the weakness of the muscles whose function becomes limited by the brace. Lack of strength in those muscles means further instability of the CCI.

Pain Management

  • Medications, nerve blocks, or other pain management techniques can be helpful.
  • Lifestyle Modifications and I suggest you follow me on social media for tips I provide.
  • Avoiding activities that worsen the symptoms, using ergonomic supports, and pacing activities

Surgical Intervention

For severe cases of CCI where conservative treatments fail, surgery may be necessary. The goal is to stabilize the craniocervical junction and alleviate pressure on the spinal cord and brainstem.

  • Fusion Surgery

Involves the use of rods, screws, and bone grafts to fuse the affected vertebrae, providing stability.

  • Decompression Surgery

May be performed to relieve pressure on the spinal cord or brainstem caused by the instability.

If you are hypermobile, make sure to seek care from those providers who treat patients with hypermobility. If you suspect having craniocervical instability, contact me.

Dr. Shakib

Recommended Reading:

How To Treat Hypermobility On a Budget

Can Hypermobility Be Cured?