Chiari, Craniocervical Instability, and Headaches

As has been mentioned in the past on this blog, I was diagnosed with Chiari Malformation (8.5mm herniation) and Craniocervical Instability (C1-C2) in 2011. Chiari and craniocervical instability are both seen in EDS patients, though the connection between EDS and Chiari is not fully understood.

Chiari Malformation is a neurological disorder where part of the brain (the cerebellar tonsils) herniates or descends out of the skull into the spinal canal. This results in compression of the spinal cord and brain stem. As a result, this compression disrupts the normal flow of cerebrospinal fluid (CSF) leading to disabling neurological symptoms (headaches, occipital neuralgia, numbness and tingling in hands and feet, weakness, nausea/vomiting, dizziness, low blood pressure, and many more).

In December 2014, I underwent a posterior fossa decompression with C1 laminectomy and partial duraplasty in order to relieve the compression on my brain, brain stem, and spinal cord. We had hoped that this procedure would improve my quality of life greatly by decreasing or eliminating my headaches and other neurological symptoms. Unfortunately, that is not what happened.

I am not going to review all of the details of recovery as that can be seen here (update 1, update 2, update 3, update 4, update 5), but it became clear that there were multiple factors contributing to my headaches (something I did not want to consider prior to the decompression). Brain surgery addressed the brain stem compression, and while that didn't eliminate my headaches entirely, it was still necessary. However, what was not addressed in that surgery was craniocervical instability or an unstable cervical spine.

Craniocervical instability (CCI) is a structural instability of the craniocervical junction (where the skull and neck meet), which can lead to deformation of the brain stem, upper spinal cord, and cerebellum (lower part of the brain). It is primarily seen in patients with EDS and other hereditary disorders of connective tissue. In severe cases a cranoicervical or cervical fusion may be necessary.

Symptoms of Craniocervical Instability include (but are not limited to):

• Headaches
• Often pressure-like--typically the result of impaired CSF flow
• Occipital neuralgia 
• Sharp, knife-stabbing, shooting pain often one sided (can be bilateral) that travels from the base up the skull up to behind the eye
• Neck pain
• Difficulty holding one's head up (bobble-head)
• Vision changes 
• Dysautonomia
• Facial pain/numbness
• Sleep apnea
• Nausea/vomiting 
• Nystagmus 
• Difficulty swallowing
• Balance problems 
• Hearing loss/tinnitus/auditory changes
• Impaired coordination 
• Brain fog and cognitive challenges

Not every individual with craniocervical instability (CCI) experiences all of the above symptoms (and some may experience symptoms not listed). Symptoms may be constant or intermittent depending on the position of the cervical spine.

I know patients with CCI (myself included) that have a few symptoms that almost always mean there is a rotation and many individuals can tell you where that rotation is. For example, when I have left sided vision changes and occipital neuralgia we typically find that C2 is rotated. Of course I also usually have a headache and other symptoms but those 2 in combination usually leads us to believe there is a rotation that needs to be addressed.

I have been in physical therapy for craniocervical instability for nearly two years. PT consists of manual therapy to reduce the muscle tension and spasms in the upper back, scapula, shoulder, and neck in order to allow the vertebrae to sit in a better position. Because of EDS and my weak connective tissue, the muscles in my neck and upper back are forced to work extremely hard in order to stabilize the c-spine. This causes severe muscle tension which pulls on the vertebrae and can lead to rotations, subluxations, and imbalances. Cervical spine PT can be very frustrating. Progress is slow and seemingly overnight any progress we do make can be undone. We may take a step forward, only to take 5 steps back on any given week. However, without PT my neck pain, headaches and other neurological symptoms would be significantly worse.  When my symptoms are unmanageable I use a cervical collar (Miami J) to prevent any more subluxations/rotations and to reduce worsening muscle tension until I can be assessed by my physical therapist. I try to avoid the cervical collar as much as possible because my neck muscles tend to "forget" to hold my head up when I use the cervical collar, which makes weaning out of it very difficult.

Headaches and other neurological symptoms are a constant in my life and management is difficult, but as of now my goal is to avoid a cervical fusion and continue with physical therapy and my cervical collar as needed.