Now we come at the point of the rare complication of EDS. The instability of the neck with CCI, AAI, Chiari malformation and brain stem compression as a consequence. Luckily this doesn’t occur often because this complication is seriously invalidating. It takes away the patients pleasure in live and, eventually, is even life threatening. Both Jane and Rosa suffer from this complication.
The cause of these problems is the extreme flexibility of the neck. Amongst other things the symptoms of CCI are: headache, neck ache, double vision, memory loss, loss of concentration, dizziness, problems of speech and swallowing and over-sensitive for light, sound and smell. It is diagnosed with the help of an upright MRI. If live gets unbearable an operation to stabilise the neck is induced. In the Netherlands they don’t perform this operation with EDS patients. In Europe, Barcelona, there is one specialist who regularly performs this operation with success.
With AAI the ligaments around the atlanto axiale vertebrae are too stretched; therefore the vertebrae move and create a hypermobility of the neck vertebrae. This complication is seen more often with people with a deformation of the connective tissue. This complication is also diagnosed with an upright MRI, or with a CT-scan with 3D technology. With people that don’t have a connective tissue disorder, the CCI/AAI is limited to C0-C1-C2; with people that do have a connective tissue disorder like EDS, the lower vertebrae are usually involved. In Jane’s case until C5, Rosa got it until C7. Symptoms are severe headache, neck ache and narrowing of the spaces in the neck. The damage done to the ligaments are painful, progressive and permanent.
Additional complications in persons with EDS are Chiari malformations; actually it is not allowed to call it this way cause the origin is different in EDS patients. In patients with EDS it is called a hindbrain herniation. It means that the hindbrain sink in the neck; you can call it a hindbrain herniation is the brain exceed the limit of 5 mm. Jane has got a deviation of 12 mm, Rosa has got a milder form. Symptoms are loss of power or paralysation of legs and arms, disorders of sensation, disorders of balance and coordination, wobbly or trembling eyes, disorders of speech, decreased reflex to swallow and problems swallowing, changed voice, difficulties with breathing, sleep apnoea and incontinence.
Beside the hindbrain herniation there can also be of brain stem compression. Rosa is only 0,9 mm away. The brain stem controls and regulates the autonomic nervous system; this means that it regulates the breathing, the heart rate, blood pressure and blood circulation, reflexes of seeing and hearing, sweating, digestion, the body temperature and the pupils. Furthermore the brain stem influences the alertness, tasting, chewing and swallowing, the forming of saliva, vomiting, peeing, sleep, sexual functions, balance and feeling gravity. In compression of the brain stem all off the above mentioned functions can be damaged or fall out which is a direct threat to life. .