Hypermobility Syndrome, AKA Joint Hypermobility Syndrome (JHS), Ehlers-Danlos Syndrome Hypermobility Type (EDS-HT), Hypermobility Spectrum Disorders (HSD)

March 29, 2016

Link: EDS, Hypermobility Type: Evolving Perspectives on a Poorly Understood Problem - Dr. Derek Neilson

"EDS, Hypermobility Type: Evolving Perspectives on a Poorly Understood Problem" is an informative talk by Dr. Neilson that touches on many important issues related to EDS-HT as well as  presents some very important updates regarding this condition that I have not seen being clarified in any other talk previously.

The video cannot be embedded here due to its privacy settings, so please visit vimeo to play the video.
To view the video on Facebook, click here.
To view his slides along with his talk, click here (only in IE or Firefox, does not play in Chrome) and play from slide#35.

I enumerate here some issues discussed in this talk and some take home points:

1. Definition. What is EDS, what are the subtypes, current estimates of prevalence rate of EDS-HT (seems to be around 1/100 - 1/500)

2. Diagnostic criteria of EDS-HT and the importance of clinical diagnosis

3. Confusion between JHS and EDS. Hypermobility syndrome was originally defined by rheumatologists, and EDS by geneticists. They are the same thing. JHS described a pain syndrome associated with joint hypermobility, and this joint hypermobility can arise from EDS-HT as well as some other conditions like Marfan's syndrome.

Source: Cincinnati Children's Hospital Medical Center

Source: Cincinnati Children's Hospital Medical Center

4. Evolution of the disease. Kids have males = females ratio for asymptomatic hypermobility condition. With age, men improve while females around menarche start worsening. Adults --- Males:Females = 1:9. Testosterone improves, Estrogen & Progesterone worsen the quality of connective tissues and therefore the symptoms. 

5. Common co-morbid conditions
  • Musculoskeletal : Chronic pain, joint dislocations, TMJ dislocation
  • Dysautonomia and POTS (discussed well) 
  • GI dysfunction
  • Chronic headaches 
  • Bruising and Bleeding
  • Anxiety and Panic disorder
  • Endocrine dysfunction, pelvic dysfunction, cardiovascular problems, immune system dysregulation incl. MCAD, chronic fatigue syndrome
6. Why are doctors not familiar with this condition yet? How is it new and why it is not mentioned in textbooks yet? Barriers to acceptance of this disease as an entity by doctors. (Jump to Slide# 69)

7. Proposed model for understanding EDS-HT - connective tissue impairment leads to sensory abnormalities related to mechanotransduction (dysfunction of blood pressure receptors, joint sense receptors, altered gait mechanics etc)

8. Management. Aerobic conditioning and strengthening exercises are extremely important for dysautonomia as well. How POTS can be better managed by hydration and salt intake. Since exercising is difficult in EDS patients, what sort of exercises they can do. (Jump to Slide#107.) Swimming is highly recommended.

9. Causes and management of headaches and importance of hydration and posture. Includes discussion about Arold Chiari malformation in EDS-HT, slide#108

10. Gastrointestinal disorders and bleeding disorders in EDS-HT.

1) EDS, Hypermobility Type: Evolving Perspectives on a Poorly Understood Problem - Dr. Derek Neilson from csfinfo on Vimeo.

2) Cincinnati Children's Hospital Medical Center