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Ehlers-Danlos Syndrome (EHD) and dizziness.

Timothy C. Hain, MD. Return to Index. Content last updated: February 27, 2019

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.

Diagnosis of EHD.

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);

Beighton Score

Image is from https://www.physio-pedia.com/Beighton_score

  1. extension of fifth finger >90°;
  2. apposition of thumb to forearm;
  3. hyperextension of elbows beyond 190°;
  4. extension of knee beyond 190°;
  5. forward flexion of trunk, legs straight, and palms touching floor (1 point);

cutoffs vary with age, namely

This is because people get stiffer with age.

Association with Dizziness

EHD is found more commonly in several types of dizziness --

According to Bendik et al (2011), migraine is found in 75% of persons with the joint hypermobility variant of EHD.

Cases

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.

Treatment:

There is no medical treatment for EHD, and all treatments are based on managing the complications. When spine stabilization is being conteplated, Dr. Fraser Henderson is a EHD specialist neurosurgeon in Maryland.

References:

 

 

 

Copyright February 27, 2019 , Timothy C. Hain, M.D. All rights reserved. Last saved on February 27, 2019

Copyright February 27, 2019 , Timothy C. Hain, M.D. All rights reserved. Last saved on February 27, 2019