Craniocervical instability (CCI) is an instability of the cervical vertebrae, because the ligaments are relaxed. The muscles normally keep everything in place. When it is relaxed, there is too much movement and the cervical vertebrae can shift, causing nerves, like the brainstem, to become trapped.
It mainly occurs in patients with Ehlers-Danlos Syndrome(EDS) and other hereditary connective tissue disorders. About 1 in 15 people with EDS later develop CCI due to a lack of connective tissue support. Some EDS patients get CCI after a head and neck injury (such as a whiplash). But often this is gradually manifested by the damage to the bands from stretching, through movements as simple as turning your head.
Unfortunately, there is still very little knowledge about CCI, but it does have major consequences.
In addition to the severe headache, Zjuul also suffers from concentration problems, fatigue, muscle weakness, ringing in his ears, heat intolerance, vision problems and extreme thirst. He has only been going to school for half a day since December 2019. School is very tiring for him, mainly because of the sounds in the environment, sitting in a chair, but also because of his concentration problems. He only has 10-15 minutes of concentration and then has to take a half hour break to be able to work again for 10-15 minutes. During such a break, he sits in a beanbag at school that supports him completely so that sitting does not cost him any strength. You can imagine that playing with a friend is not possible for him.
If Zjuul is not treated, he will only get more symptoms, and these will only get worse. CCI usually starts in the late teens and after a few years these young people lie in bed 24 hours a day in a low-stimulus dark room. They can no longer tolerate light, sound, touch, emotions. We really hope that we are intervening early enough and that Zjuul does not have to experience this.
The longer his condition is left untreated, the more likely the symptoms will be permanent after treatment.
What is the treatment for CCI?
Craniocervical Fusion: An operation in which the skull is pulled upwards (cervical traction) and placed in the correct position. Then the skull is attached to the upper vertebrae of the spine to maintain the correct position.
Craniocervical fusion is a risky operation and is performed only when there is a medical need.