Upper Cervical Instability in Hypermobile Patients
People with hypermobility can experience instances where the increased mobility begins to overlap with pain provoking disorders. For some individuals with Hypermobility Spectrum Disorder (HSD) or hypermobile Ehlers-Danlos Syndrome (hEDS), the increased mobility can contribute to neck pain, headaches, muscle fatigue, and difficulty maintaining neutral/comfortable head and neck postures.
One condition which produces neck symptoms in these populations is upper cervical instability (UCI). This condition is characterized by excessive or abnormal movement in the upper cervical spine (neck) near the base of the skull. Although a clinically diagnosed “instability” can be significant, it is important to understand that the term “instability” does not have a consistent definition across all professions. For example, a physician may use this term only if a medically emergent finding is present, versus other allied health professions may use this term on a spectrum to describe movement above average. It is important to understand that neck pain, headaches, dizziness, or other symptoms do not automatically mean that a true clinical instability is present.
Recognizing concerning symptoms, and understanding how upper cervical instability is assessed/diagnosed/managed, can help people with hypermobility make informed decisions about their health.
What is Upper Cervical Instability?
The neck consists of seven cervical vertebrae that support the head, house the spinal cord, and allow the neck to move. The upper cervical spine includes the connection of the head, with the first (C1) and second (C2) cervical vertebrae. These joints participate in important neck movements, like nodding and rotation. Bone structure, muscles, ligaments and connective tissue help maintain stability while allowing these normal movements to still occur.
Upper cervical instability occurs when movement at these joints is excessive or poorly controlled, which in certain circumstances can affect the surrounding neurological structures. Terminology around instability is used to describe the specific joints affected.
Craniocervical instability (CCI): Abnormal movement or instability at the junction of the head and the upper cervical spine.
Atlantoaxial instability (AAI): Abnormal movement or instability between the first cervical vertebrae (C1 – known as the “Atlas”), and the second cervical vertebrae (C2 – known as the “Axis”).
Hypermobile Individuals and Upper Cervical Instability: What’s the Connection?
Hypermobility describes joints that move through above average range of motion. For some people this may be a normal variation and does not present any problems. In other individuals the increased movement availability can contribute to altered movement patterns, altered joint structures, and reduced movement control resulting in neck pain, fatiguability, or injury.
In conditions such as hypermobility spectrum disorder or hypermobile Ehlers-Danlos Syndrome connective tissue characteristics contribute to the increased joint mobility. The connective tissue and ligaments of the upper cervical spine are important protectors from excessive movement. When someone has symptomatic hypermobility, there are several factors that may be relevant to their neck pain presentation:
Increased ligamentous laxity: reduces available passive support
Muscle fatigue: muscles have to work harder when the passive restraint system is compromised
Altered proprioception: proprioception can be described as our body’s understanding of what position a joint is in and how it is moving – an important monitoring system
Previous traumas: high force injuries, such as motor vehicle accidents/whiplash, can contribute to UCI
Being hypermobile or having hEDS does not mean a person will have UCI, however it can be a contributing factor, and may warrant further investigation when neck pain is paired with other symptoms.
Common Symptoms of Upper Cervical Instability
Neck pain and headaches: persistent neck pain, often near the base of the skull, with associated headaches
A feeling of head heaviness: some people will describe that their head feels heavy to hold up, and that they easily fatigue, resulting in wanting to support their head (on a pillow, with their hands)
Dizziness or balance deficits: while dizziness can commonly be benign and associated with less emergent neck conditions, if severe dizziness and balance deficits present a medical assessment is warranted
Sensory or neurological changes: tingling and numbness can be common in neck conditions (ex: travelling into the arm), however some neurological findings warrant immediate medical assessment (such as difficulty swallowing, changes in speech, facial numbness – particularly around the mouth)
Note: No single symptom can reliably diagnose upper cervical instability, however a grouping of findings warrants investigation, and some symptoms (as mentioned above) warrant immediate medical attention.
How is Upper Cervical Instability Diagnosed?
Diagnosis requires taking into consideration the entire clinical presentation of a person. Assessment that leads to UCI diagnosis involves a combined approach.
Medial History and Symptom assessment: including location/severity/duration of neck pain and headaches, whether symptoms change with movement/positions, history of prior traumas, presence of dizziness or neurological symptoms, history of generalized joint hypermobility or diagnosed connective tissue disorder
Physical and Neurological Examination: assessing neck motion, muscle function, coordination, balance, sensation, reflexes, and other manual tests may be used
Medical Imaging and Specialist Assessment: referral for imaging and specialist assessment is considered when history and exam suggests that structural abnormalities could be present. This may include x-ray, CT scan, or MRI of the head and neck.
When Should You Seek Medical Attention?
Although most causes of neck pain, headaches, and dizziness are not emergencies, some circumstances warrant immediate attention.
Seek emergency medical care if you experience sudden or rapidly worsening neurological symptoms, such as difficulty swallowing or speaking, sudden loss of coordination/balance, fainting, visual changes, or severe headache. This is especially pertinent when the symptoms are new or simultaneous.
Physical therapy assessment should be initiated if suspect you have hypermobility and are presenting with neck pain and headaches, but do not have the severe symptoms that require immediate medical care. In the case of more severe symptoms, physiotherapy can be initiated once you are cleared by a physician.
What is the Role of your Physiotherapist in Upper Cervical Instability?
It is important to remember that not all neck pain and headaches are linked to clinically diagnosed “instability” and that hypermobility can be a spectrum. Hypermobile individuals can present with neck pain even in the absence of a clinical “instability” due to their increased range of motion. As such, physiotherapy can be initiated once any emergent medical conditions of the upper neck are cleared by a physician, or if you are presenting with more mild symptoms. Your physiotherapist will provide a thorough assessment of your history and physical presentation in order to come up with an individualized plan to reduce pain and restore function.
Treatment often involves a combination of:
Modalities for symptom relief (manual therapy, acupuncture and dry needling approaches, etc)
Proprioception exercises
Motor/movement control exercises
Strengthening exercises
Posture training
Education on condition and self-management strategies
I have hypermobility and ongoing neck symptoms. What can I do?
Keep a record of your symptoms: track when symptoms occur, how long they last, and associated activities/positions
Avoid forcing your neck into end-ranges of motion: do not repeatedly stretch your neck into the extremes of range of motion to test it’s flexibility.
Modify aggravating activities: based on your symptom log, you can start to modify positions/postures that create symptoms, such as changing positions or reducing duration
Seek individualized advice: this is where following up with your doctor and physiotherapist can help assess, diagnose, and create an individualized program based on your unique presentation
Not all hypermobile individuals have clinical upper cervical instability, however hypermobility can contribute in many ways to neck pain and headache presentation. Every person with hypermobility will present in their own unique way, as such the management of neck pain is best supported by an individual care plan to improve movement control, proprioception, and overall function. An individualized physiotherapy assessment can help guide you on this path.
References
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Mao, G., Kopparapu, S., Jin, Y., Davidar, A., Hersh, A., Weber-Levine, C., & others. (2022). Craniocervical instability in patients with Ehlers-Danlos syndrome: Controversies in diagnosis and management. The Spine Journal, 22(11), 1944–1952. https://doi.org/10.1016/j.spinee.2022.08.008
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