Craniocervical Instability (CCI) / Atlantoaxial Instability (AAI)
Craniocervical instability and atlantoaxial instability are conditions that often occur simultaneously and may have a major impact on quality of life.
CCI refers to instability in the area where the skull and upper cervical vertebrae meet.
AAI refers to instability between the first and second cervical vertebrae (atlas and axis).
Upper Cervical Instability (UCI) is an umbrella term that includes both cranio-cervical instability and atlanto-axial instability. It may affect the nerves, brain, and spinal cord, which together make up the nervous system. The tissue surrounding these nerves and structures is there to protect, support, and connect. If there is a change in how these structures are loaded or how well they are supplied with blood, it may affect the nervous system.
CCI (Craniocervical Instability) after trauma and its relationship to brain injury
Craniocervical instability (CCI) is a condition in which the stability in the area between the skull and the upper cervical vertebrae is disturbed. The instability may lead to a reduced ability to properly support the head and the neck.
Although not all cases of CCI immediately cause brain damage, untreated instability may contribute to long-term complaints that strongly overlap with symptoms of brain damage.
It is therefore important to seek medical advice in a timely manner and to have a diagnosis performed in the event of persistent complaints after a trauma.
Causes
The causes of CCI may vary.
A common cause is trauma, such as whiplash, a rear-end collision, a fall or another accident. Such an accident may also result in a traumatic brain injury. The ligaments and other supporting structures may be stretched or damaged.
In addition, conditions such as Ehlers-Danlos syndrome (EDS), in which connective tissue is naturally weaker, may contribute to the development of CCI. Inflammatory diseases such as rheumatoid arthritis, which affect the joints and supporting tissues, may also play a role.
In some cases, CCI is caused by degenerative changes, such as wear and tear of the intervertebral discs or ligaments with aging. Finally, congenital abnormalities, such as an abnormal shape of the skull base or vertebrae, Chiari malformation type I, may increase the risk of CCI.
Consequences
CCI may cause various symptoms including dizziness, balance problems and neurological symptoms, such as tingling, muscle weakness, or difficulty speaking and swallowing (dysphagia).
In severe cases it may even lead to breathing problems or severely reduced mobility or paralysis.
Common symptoms include severe headache, a feeling of head being too heavy for the neck, and neck pain.
Headaches may be aggravated by activities that increase pressure, such as coughing, sneezing, yawning, or laughing.
Other neurological symptoms include sleep apnea (both centrally from the brain and from obstruction in the neck), tingling, pain or numbness in the face, and problems with (double) vision.
In addition, tinnitus, nausea and vomiting, and irregular downward eye movements (nystagmus) may also occur. These symptoms may have a major impact on quality of life.
Autonomic nervous system dysfunction (dysautonomia or POTS) may cause a wide range of symptoms. Some common complaints include an irregular or rapid heartbeat (tachycardia), heat intolerance, dizziness, which is sometimes caused by orthostatic hypotension (impaired blood pressure regulation upon standing). Other symptoms include fainting, bowel problems such as constipation, excessive thirst, and chronic fatigue.
In addition, pressure on the brainstem and surrounding structures may also contribute to increased intracranial pressure, which may result in symptoms such as headaches, blurred vision, nausea and neurological deficits.
The connection with brain injury therefore lies in the possible impact of instability on the surrounding nerves, blood vessels and brainstem. The blood flow to the brain may be disrupted.
The relationship between Idiopathic Intracranial Hypertension (IIH) and CCI
Idiopathic Intracranial Hypertension, also known as a cerebrospinal fluid pressure problem, is a condition in which the pressure in the brain is not properly regulated.
This may lead to symptoms such as headaches, vision problems and difficulty with balance.
One often overlooked possible cause is a link to craniocervical instability (CCI). This means that the joints between the skull and the upper cervical vertebrae are not strong enough, which may put pressure on the spinal cord or brainstem. Although not yet fully understood, studies show that CCI may contribute to changes in cerebrospinal fluid pressure and thus cause or worsen IIH.
The Relationship Between Atlantoaxial Instability (AAI) and Brain Injury
Atlantoaxial instability (AAI) refers to an abnormal or extreme range of motion at the junction of the atlas vertebra (C1) and the axis vertebra (C2), which compromises the stability of the upper cervical vertebrae.
This instability may lead to compression or damage to the spinal cord and surrounding structures, which in some cases may cause brain damage.
Causes
The instability may have various causes, such as congenital abnormalities, trauma or inflammatory diseases. Although a brain tumor, cerebral hemorrhage or cerebral infarction occur in the brain and do not directly affect the spine, these conditions may lead to AAI indirectly. This may happen, for example, through changes in muscle tone, neurological complications or secondary effects on the structures surrounding the cervical vertebrae.
Although AAI is more common in conditions such as Down syndrome, rheumatoid arthritis, or Ehlers-Danlos syndrome (EDS), it may also occur as a result of brain injury or trauma.
In such cases, damage to the ligaments, bones, or other structures around the upper cervical vertebrae may contribute to the problem.
Consequences
The impact of AAI ranges from mild symptoms such as neck pain and stiffness to more serious complications such as neurological deficits or even life-threatening conditions.
The cranial nerves, which are responsible for functions such as vision, motor control and reflexes, may become pinched or irritated by the increased pressure or abnormal movements in this area. This may lead to symptoms such as headaches, neck pain, difficulty swallowing, dizziness, speech problems or even loss of certain neurological functions. In severe cases, AAI may also damage the spinal cord, which may cause further complications.
Experience story of someone with CCI and AAI
I think my brain definitely suffered damage during my rear-end collision.
That same day I had a lot of pain in my back and ribs and had trouble breathing, so I called in sick and went to the GP, who diagnosed at least 2 ribs and vertebrae bruised.
After a few days I started losing my speech, my sentences made no sense and I couldn't remember anything. I asked my girlfriend the same question every minute. I got really bad migraine attacks, up to twelve a day. My girlfriend bought a whiteboard to write down the information I kept asking for. I couldn't stand any stimuli, no warmth, crowds, noise and light, but also no movement, not even bending over. Just getting up from the couch made me throw up. That lasted for years and caused quite a bit of damage, including to my teeth.
I worked hard for years to be able to have a decent conversation again, that people understood me. Unfortunately, all this also cost me the relationship with the love of my life, my job, so income and everything.
Most cognitive complaints started about a week after the accident.
Cognitive complaints: remembering, word finding problems. You think that you are able form a correct sentence while you are just spouting incomprehensible sentences.
- Concentration problems and not being able to keep this up for long
- Not being able to focus on one specific subject / easily distracted
- The familiar stimuli: light, sound, crowds and heat niet kunnen verdragen
- Always being warm accompanied by very warm, dry hands. So warm that I sometimes had to cool them down.
- Not being able to tolerate stress
- Feeling as if my neck was being squeezed together with pliers
- Dancing letters when you want to read something
- Totally unable to cope with movement
- Many migraine attacks every day
- Problems falling asleep
- Difficulty with balance
- Due to the weight gain after the accident, also sleep apnea
- Depressive complaints
There are probably things that I have forgotten.
The medical examiner indicated that I have symptoms of CCI/AAI, of idiopathic intracranial hypertension (IIH), dysautonomia and dysfunction of the limbic system.
I have never had a NeuroPsychological Examination or any other cognitive test.