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Saturday 5 November 2011

Latest Diagnosis of Ehlers Danlos / Benign Hypermobility Syndrome


This is the latest in my journey to find out what is happening to me and to explain the symptoms i am experiencing.
I went to see my Pain managment consultant in September 2011. I explained to him that i was experiencing constant dizziness , brain fog, severe pains in my hips, ankles , neck , hands , wrists , knees etc. I told him that my head felt too heavy for my neck and that i had to keep resting my head in my hands and keep mostretching it out to give me some type of relief.
He ask me to do a series of tests called the Beighton test .Helps to determine the degree of joint hypermobility. Currently it is used as part of the Brighton Criteria. For a positive Beighton Score, 4 or more points out of nine are required (i.e. two elbows and two hypermobile knees):

1. More than 10º hyperextension of the elbows

2. Passively touch the forearm with the thumb, while flexing the wrist.

3. Passive extension of the fingers or a 90º or more extension of the fifth finger (Gorling’s sign). This is used as a “Screen Test”.

4. Knees hyperextension greater than or equal to 10º (genu-recurvatum)

5. Touching the floor with the palms of the hands when reaching down without bending the knees. This is possible as a result of the hypermobility of the hips, and not of the spine as it is commonly believed.

I scored a total of 7 points and he told me that he believed i was suffering with Benign Hypermobility Syndrome / Ehlers Danlos syndrome type III. He then made me a referal to see a Rheumatologist who specialises in these syndromes.
I attended the appointment with the Rheumatologist a couple of weeks ago where she asked me to do the Beighton test again, ( again i scored 7 points ) This time the Rheumatologist looked at scarring in various places on my body to see how they had healed ( papyraceous scarring ). She made comment of how my eye lids had drooped. She even tested how flexible my ears were.
She told me that i had BHS / EDS type III and that she would arrange for me to see a specialist Physiotherapist to help me with strengthening my muscles. She told me that it explains why i have cranial instability in my neck which explains the pains i am having in that area. I am now taking 400mg of Tramadol a day to help with the pain and 40mg baclofen before bed.
I am awaiting now to see a Cardiologist to see if he can test me for POTS as this may explain the dizziness. My Journey continues.

Major Criteria

· A Beighton score of 4/9 or greater (either currently or historically)

· Arthralgia for longer than 3 months in 4 or more joints

Minor Criteria

· A Beighton score of 1, 2 or 3/9 (0, 1, 2 or 3 if aged 50+)

· Arthralgia (> 3 months) in one to three joints or back pain (> 3 months), spondylosis, spondylolysis/spondylolisthesis.

· Dislocation/subluxation in more than one joint, or in one joint on more than one occasion.

· Soft tissue rheumatism. > 3 lesions (e.g. epicondylitis, tenosynovitis, bursitis).

· Marfanoid habitus (tall, slim, span/height ratio >1.03, upper: lower segment ratio less than 0.89, arachnodactily [positive Steinberg/wrist signs]).

· Abnormal skin: striae, hyperextensibility, thin skin, papyraceous scarring.

· Eye signs: drooping eyelids or myopia or antimongoloid slant.

· Varicose veins or hernia or uterine/rectal prolapse.

The Joint Hypermobility Syndrome (JHS) is diagnosed in the presence two major criteria, or one major and two minor criteria, or four minor criteria. Two minor criteria will suffice where there is an unequivocally affected first-degree relative.

JHS is excluded by presence of Marfan or Ehlers-Danlos syndromes (other than the EDS Hypermobility type (formerly EDS III) as defined by the Ghent 1986 and the Villefranche 1998 criteria respectively).



Criteria Major 1 and Minor 1 are mutually exclusive as are Major 2 and Minor 2.