Craniocervical Instability
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What is CCI?
Craniocervical Instability (CCI) is a structural problem of the craniocervical junction. The craniocervical junction is located at the back of your head where the base of your skull (the occipital bone) and C1 (the atlas) – C2 (the axis) function together. Ligaments that hold your head upright become loose and lax.
The Upper cervical portion of the spine (C1-C2) is highly specialized. C3 to C7 are more classic vertebrae with spinous processes and facet joints.
Difficulties can result from problems with excessive motion in the first two cervical vertebrae, since this results in decreased cervical space in the brain stem and cervical spinal cord. Instability is not limited only to this region of the neck, as the impact often extends through the cervical areas to C7.
Some patients presenting with instability may have suffered physical trauma (such as a motor vehicle accident, sports accident) or be predisposed to risk (such as Ehlers-Danlos syndrome). Other patients who experience similar issues had “random onset of symptoms” with no triggering event or physical injury. (ME/CFS) Some patients lose their mobility overnight.
Upper cervical issues may include persistent misalignment, Atlantoaxial instability (AAI), and Craniocervical instability (CCI).
Biomechanical Issues 2024; Bateman Horne’s Dr. Yellman Presents a Webinar on Craniocervical Instability, Tethered Cord & Mast Cell Activation Syndrome (MCAS)
Symptoms
May include:
- Neck pain (often at the base of the skull)
- Headaches (and head pressue)
- Vertigo and dizziness
- Weakness
- Drop attacks
- Visual disturbances
- Confusion
- Sleep apnea
- Brain fog (or cognitive disturbances), concussion like symptoms
- Tinnitus (ringing in the ears)
Many patient groups have been linked to CCI as a co-morbidity.
Patient groups linked to Craniocervical issues include:
- Myalgic Encephalomyelitis/Chronic Fatigue Syndrome (ME/CFS)
- Ehler-Danlos Syndrome (EDS) and other connective tissue disorders (hEDS)
- Fibromyalgia (FM)
- Physical trauma (motor vehicle accidents) and sports injuries
- Rheumatoid arthritis
- Down syndrome
Once CCI develops, this can result in a myriad of other associated serious issues including chiari malformation, tethered cord, postural orthostatic tachycardia syndrome (POTS), Eagle’s syndrome, and other neurological disturbances.
Levels of symptom severity are wide ranging. Some patients are house bound, others are bed bound with little support and many are socially isolated.
Diagnosis in Canada is rare and most GPs are not properly trained to look for issues related to Craniocervical junction issues.
OTHER BIOMECHANICAL ISSUES may co-exist with/without CCI. These discussions are now surfacing by doctors committed to chronic illness patients and hoping greater attention and emphasis will be directed towards improved patient care. Many patients are not properly diagnosed, assessed and directed for treatment for these complex conditions. The lack of diagnosis and proper guidance for treatment exists globally.
Diagnostic Imaging
Traditional MRI and CT scans are not considered adequate for proper assessment of the Craniocervical junction. The following video explains how a 3D recumbent MRI can be requested to maximize imaging of the Craniocervical junction.
Specialized radiographic studies include:
- Upright MRI (flexion/extension)
- Rotational CT scan
- Digital motional X-ray (DMX)–the merits of DMX are detailed in this paper
- CBCT (2024, Dr. Gilete–a published article is expected to be released in the next year on the merits of CBCT imaging)
The role CCI plays in symptoms and their condition remains unknown to some patients who will never receive imaging. In Canada, 1 Upright MRI is available for patient use in BC (with no cervical imaging as of May 2024).
Treatment
Treatment approaches vary significantly from patient-to-patient since no clear guidance is available for Canadian patients experiencing craniocervical issues. Based on communication with patient groups, most instability patients are not imaged and assessed by a neurosurgeon. For more severely impacted patients, neurosurgical interventions such as fusion may be necessary and access is limited. In the interim, patients seek assistance through upper cervical chiropractors, specialized PTs, and highly skilled osteopaths.
Beware that patients with Craniocervical junction issues must avoid thrusting chiropractic manipulations since this can be dangerous. Unsupervised traction can also be dangerous.
Depending on the type of difficulty, patients may also seek upper cervical care practitioners including:
- Atlas orthogonal
- Nucca
To find an Atlas orthogonal practitioner, select your Province using the following link. It is recommended that proper imaging be obtained to ensure the use of accurate vectors to correct a misalignment. Upright MRI imaging is necessary to identify anomalies in the spine to ensure vector accuracy. Approximately 20% of the patient population has anomalies in the bone.
New Theories in CCI & Craniocervical Obstructions
Craniocervical instability may occur as a result of a physical injury including a motor vehicle accident and/or sports injury. However, what remains novel, and not well defined, is the link between infections, toxicity and inflammation playing a role in the erosion of the neck ligaments. For example, it is not unusual for ligament laxity to occur in Lyme/Multiple Systemic Infectious Disease Syndrome (MSIDS) and other patients experiencing high levels of inflammation. In his recent blog article (February 2023), Dr. Harris of the Bay Area Lyme Foundation makes the connection between Lyme and CCI.
Further, Swedish study reports on patients with Myalgic Encephalomyelitis/Chronic Fatigue Syndrome (ME/CFS) and the common finding of craniocervical obstructions, such as those seen in CCI. Scientists suggest that obstructions of blood flow to the brain are connected to the high levels of pain, neurological symptoms, and fatigue that ME/CFS patients experience. Read more from this cross-sectional study on the connection between ME/CFS and Craniocervical issues (2020).
Gez Medinger’s interview of Dr. Jen Curtin on Craniocervical issues including CCI, Chiari malformations, tethered cord, and the connection to Long-COVID, and ME/CFS
Dr. Peter Rowe Talks Biomechanical Issues
Dr. Peter Rowe (2024) discusses BIOMECHANICAL factors and how NEUROANATOMIC problems relate to ME/CFS -Thoracic outlet syndrome(TOS), Stenosis and craniocervical issues are discussed
Renegade research’s Tess Fallor hosts these excellent sessions.
VENOUS CONGESTION, BRAIN & NECK DISORDERS
This expert team of neurosurgeon/neurologist/neurointervention panelists, led by Dr. Ferdinand K. Hui, who discuss the importance of organizing the team’s work and research (2023).
Team includes Dr. Vitor Pereira (St. Michael’s Hospital, Toronto)
- Dr. Ilene Ruhoy explains the mechanics of the craniocervical junction and the connection between craniocervical instability, jugular vein compression, and intracranial hypertension. The Structural integrity of the ligaments which are clearly part of the problem.
Summary of webinar (click here):
- Dr. Hui refers to his 34 y/o old he treated and was lost to suicide. This inspires him to do better and continue to find solutions.
- Dr. Higgins describes Venous congestion as the “thing that no doctor bothers to look for” and constellations of symptoms.
- Intracranial hypertension (IIH) and cerebral venous congestion are discussed as intertwined in this complex discussion.
- Different types of Stenosis and where medications are expected to be effective. When to Stent vs when not to Stent.
- Constellation of Symptoms can include:
- Viruses can directly attack neck tissue and increase expression of genes which attack connective tissue. (at 1.01)
- Benefits of decompression vs stenting based on patient outcomes.
- The prospect of shaving C1 and craniocervical compression of the jugular vein
- Mast cell activation syndrome is a common problem among this group of patients
- Venous congestion issues tied to Long Covid.
- pain phenomenon at the base of the skull
- strange vision issues not identified by eye doctor
- problems relieved by lying down or standing up
- pre black out spells caused by rotation of the head
- tinnitus
- dizziness
- neuralgia
- migraines
- hormone difficulties
Cerebrospinal Fluid Flow
Dr. Scott Rosa (Rock Hill, NY) is a leader in the upper cervical chiropractic field with extensive experience in the treatment of Craniocervical issues. The article below describes how persistent misalignment can impair cerebrospinal fluid (CSF) flow and cause further issues (2015).
CCSVI Interviews Dr. Scott Rosa (2016)
Discussion regarding his research in the area of cerebellar tonsillar ectopia, altered cerebrospinal fluid, arterial/venous flow dynamics and the correlation to the craniocervical junction.
CCI Resources
Craniocervical Junction Syndrome; Anatomy of the Craniocervical and Atlantoaxial Junction and the Effects of Misalignment on Cerebrospinal Fluid Flow
Scientific paper by Rosa et al.
Dr. Centeno’s interview with Dr. Rosa, including future attention in the area of C0-C1.
Dr. Maxwell’s Spiky Leaky Syndrome (2024)
Dr. Andrew Maxwell notes in the past decade that a combination of genetically vulnerable hosts in a chronic inflammatory state involve mast cell activation and the development of a hypermobility state including instability and anatomical changes in the cranio-fascio cervical region.
Dr. Amy Proal (from PolyBio) interviews Dr. Ilene Ruhoy on Craniocervical issues
Gez Medinger’s CCI Series with Dr. Jen Curtin, 2023
Gez Medinger interviews Dr. Jen Curtin, who refers to the common connection between CCI in ME/CFS (estimated to be potentially 50%) which is now being investigated. Long COVID may be triggering inflammation and resulting in similar outcomes. This is now being further researched. This video series explains the difference between multiple complex chronic illnesses often associated with spinal cord injury: CCI, ME/CFS, Chiari malformation, and more. The occurrence of these conditions post-COVID (and in association with long-COVID) are also explored.
Centeno Schultz “CCI 101-Understanding CCI”
The following publication is available to print for your doctor. Many Canadian GPs may not be familiar with conditions of the Craniocervical junction.
A 3- minute video in which Dr. Centeno describes the anatomical problems leading to Cranial Cervical Instability.
This link provides symptom guidance and current treatment options including stem cell therapy and PICL by Centeno Schultz. The analysis of patient outcomes is recent (2022) and are available on YouTube.
Current Scientific Papers in CCI
The two following papers are crucial to read in the area of craniocervical instability. The first consists of craniocervical junction (CCJ) anatomy landmarks, and may provide further context to specific areas/connective tissue layers implicated in CCI.
The second paper documents improved outcomes on a small group of myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS) patients following surgery of cervical spinal stenosis. It contributes to literature that makes the connection between global physiological issues and a narrowed spinal canal.
Interview with Dr. Scott Rosa
Dr. Centeno from Centeno Schultz interviews Dr. Scott Rosa on upright MRI for CCI and ME (2024).
Dr. Rosa describes how craniocervical misalignment affects CSF flow.
American Syringomyelia & Chiari Alliance Project
ASAP.org’s Not-for-Profit website, focused on Chiari malformations and syringomyelia.
ASAP 2023 conference video with Dr. Bolognese discusses craniocervical syndrome & instability.
Excerpt from 2021 International ME/CFS Conference
Topic: Craniocervical Instability in varying patient groups (Q submitted by A. Hall)
Q: There appear to be a number of ME/CFS patient groups who are bringing forward Upright MRI/DMX diagnostic imaging identifying Craniocervical Instability (CCI) (including physical injury, viral, EDS patient populations). In your opinion, is there a percentage of the ME/CFS population, where this may in fact be driving the problem?
Additionally, Multiple MDs are suggesting CIRS in the EDS patient population is resulting in CCI in a pediatric/adult population. Some neurosurgeons are in agreement, identifying this as novel. Please speak about CCI and these possibilities.
Response by Dr. Kaufmann
A: I cannot give good percentages and we do not have this available yet.
It is a huge, growing new issue, and newly recognized, that is the incidence of Craniocervical instability.
I will speak to this question in the context of connective tissue disorders. Obviously patients with EDS have genetically driven connective tissue disorder, many/majority of whom will never get sick or develop a problem.
Those that go to develop illness, chronic inflammation results in further injury to the connective tissue (as result of cytokine production, enzyme release, proteases). What is also becoming clear is you do not have to have EDS.
If you have chronic inflammation, many patients are presenting with similar imaging.
I am NOT saying that Craniocervical instability is the CAUSE of ME/CFS, however, it is one of the PILLAR parts of the infrastructure and we are the on the frontier of analysis. It is a very big deal and we are on the leading edge of trying to understand it.
— Dr. Kaufmann, 2021 International ME/CFS conference
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