Ehlers- Danlos syndrome is a connective tissue disorder, characterized by joint hypermobility. It has 13 subtypes. Many are associated with distinct gene mutations. The most common syndrome, the hypermobile type, is autosomal dominant but the gene is yet unknown.
Here we are discussing only the joint hypermobility variant of EHD, and we are not considering the other variants.
Hypermobility is scored using the Beighten score. Cutoffs vary by age.
5 elements, and 1 point for each side (so could have 9 points max);
Image is from https://www.physio-pedia.com/Beighton_score
cutoffs vary with age, namely
This is because people get stiffer with age.
EHD is found more commonly in several types of dizziness --
- Chiari malformation
- Basilar impression (or invagination)
- CSF leak
- Cervical spine disorders
- Carotid dissections (more of a cause of stroke than dizziness).
- TMJ disorders (not a big cause of dizziness, but still a correlation)
One would expect that there would be more PLF as well.
According to Bendik et al (2011), migraine is found in 75% of persons with the joint hypermobility variant of EHD.
Many of these disorders are related to the fragility of collagen (Henderson et al, 2017).
We have encountered several patients to date with dizziness, eventually traced to EHD. Two of these patients underwent a cervico-occiptal fusion for an unstable upper cervical spine. This is a heroic procedure where bolts are put into the skull and upper cervical vertebrae to keep the head stable on the upper spine.
We have also had several patients with POTS as well as EHD together.
There is no medical treatment for EHD, and all treatments are based on managing the complications. When spine stabilization is being contemplated, Dr. Fraser Henderson is a EHD specialist neurosurgeon in Maryland.