The B & B Scores

If you’re suspected of having Ehlers-Danlos Syndrome you’re likely to be tested via the Beighton Score.  This looks at certain joints in the body and how hypermobile they are:

  • Elbow bends backwards more than 10 degrees (1 point per elbow)
  • Knee bends backwards more than 10 degrees (1 point per knee)
  • Thumbs bend inwards to touch your forearm (1 point per thumb)
  • Little finger bends up more than 90 degrees from the back of your hand (1 point each pinky)
  • Hands can be placed flat on the floor with your knees straight (1 point)

I have big issues with the Beighton Score.  My knees don’t bend backwards, my thumbs don’t bend anywhere near my forearms and I’ve never been able to place my hands flat on the floor with straight legs.  So according to this I’d only score 4/9 and not really be classed as hypermobile at all!

Why do they only look at these particular joints?  What about floppy wrists (which was one of the tests my Rheumatologist did on me), hypermobile shoulders, a hypermobile neck (before mine got stiff and painful I could turn my head round like the Exorcist 😉 ), hypermobile spine (ie ability to do backflips), hypermobile pelvis (ie ability to do the splits), hypermobile ankles or toes – all of which I have.  Plus my thumbs might not bend inwards to touch my forearms, but they do bend outwards at 45 degrees and my wrists bend outwards at 90 degrees.

I have no idea why all the joints in the body aren’t tested, and in various directions.  If you come across a Doctor, Physio or even Rheumatologist not particularly clued up on EDS they stick rigidly to the Beighton Score and are probably missing people, like myself, who are obviously hypermobile but just not in the particular joints listed.

The Beighton Score has to be coupled with the Brighton Score to diagnose EDS:

Major criteria

  • Beighton score greater than 4 (if there has been an injury or surgery affecting range of movement, this can be considered historically)
  • Arthralgia (joint pain) in more than 4 joints for more than 3 months.

Minor criteria

  • Beighton score 1-3 (or 0-3 if over 50 years)
  • Arthralgia in 1-3 joints for more than 3 months
  • Back pain, spondylosis (spinal arthritis) or spondylolisthesis (spinal subluxation) for more than 3 months
  • Dislocating/Subluxating (partial dislocation) in more than 1 joint, or the same joint more than once.
  • More than 3 soft tissue injuries, tenosynovitis (inflammation of sheath around tendons) or bursitis (inflammation of the fluid-filled sac in a joint)
  • Marfanoid Habitus; a characteristic appearance including being tall and slim and having long, thin fingers.
  • Thin, stretchy skin or abnormal scarring (cigarette paper scarring or easy scarring)
  • Droopy eyelids, short-sightedness, double vision.
  • Varicose Veins (particularly at a young age)
  • Hernia
  • Rectal or uterine prolapse

In order to be diagnosed with EDS you should have:

  • Two major criteria or
  • One major and two minor criteria or
  • Four minor criteria or
  • Two minor criteria and a first-degree relative (parent/child/sibling) who has been diagnosed with EDS-HT.

To be honest, I also take issue with the Brighton Criteria because I barely fulfill the Beighton score and don’t have “joint pain” in the traditional sense of the word – my pain……..actually there’s so much to say about my pain it needs a blog post of its own.  At the time of diagnosis I’d never had a prolapse, hernia, varicose veins, droopy eyelids, am 5ft 2″ with short arms so hardly a Marfans body type and no dislocations or subluxations (although some of these have manifested in recent years) so I only really fitted 3 minor criteria.

The Score doesn’t take into account my:

  • Positive Gorlin’s sign
  • GI issues (reflux, nausea, early satiety, constipation, GI pain)
  • Chronic Fatigue
  • Migraines
  • Dysautonomia
  • Low blood pressure
  • Dental issues
  • Easy bruising
  • Excessive bleeding & poor wound healing
  • Menstrual pain
  • Blue Sclera or
  • Mast cell disease

which, coupled with my history and hypermobility, point very strongly towards EDS.  All of which are the reasons I was careful to choose a Rheumatologist who knew what to look for in a suspected EDS patient.

I know good Rheumatlogists who specialize in hypermobility will do a full history, know what questions to ask, not stick rigidly to the Beighton Score and expand on the Brighton Criteria but many doctors will not – they tick boxes on a form and if you don’t tick the box then you can’t possibly have EDS.  It has to change.  I fell through the net for 42 years because a full clinical history from birth wasn’t taken and no-one joined the dots of my mish-mash of symptoms.  When I was finally diagnosed my Rheumy said I was such a “text book” case of EDS she was amazed it hadn’t been picked up sooner.  But looking at the diagnostic criteria it shouldn’t surprise anyone because they’re really quite flawed.

If I hadn’t read up about EDS online, realized myself I had it, researched the most knowledgeable Rheumy to see and paid for a private appointment because my GP practice wouldn’t fund an out of county referral on the NHS I’d still be undiagnosed.  It shouldn’t be that hard.

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10 thoughts on “The B & B Scores

  1. Jan Groh

    I can’t agree more about the shortcomings of the 9 pt Beighton hypermobility scale which in a plot to confuse is only 1 letter off from the better Brighton diagnostic *criteria* (not score or scale, sigh). I like to say we “got Bright in Brighton England” right! That said, you don’t have to be tall to have a marfanoid habitus. If you read the original Brighton criteria closely, height is not required. It’s more the ratio of your limbs to your height that matters. Many do happen to be tall and slim, but another friend of mine is also Marfanoid at 5’2″. (I’m 5′ 4″ myself with a long wing span.) I admit to thinking this myself initially until she corrected me. (She is properly diagnosed.) They use a Steinberg wrist sign plus the height/span ratio to really nail it I think.

    Further, you can score a 0 (yes, zero) on the Beighton 9 pt scale and *still pass* the Brighton. I help many do this by starting from the bottom up and ticking off minor points. I stop at 7 (before hitting the Beighton scale) and ask, and almost invarialy folks have ticked off 4 points and usually more. Meaning they’ve passed. The bottom 4 have nothing to do with your joints, and the next 3 up have nothing to do with being bendy. (I’ve written about this at length on my blog http://ohtwist.com.) I’m willing to bet you’d still pass. And, note that you can be less bendy when you use the minor criteria Beighton score too. So yes, you would have passed that point with your score of 4 if you passed any 3 otehr minor criteria also. (Hope that makes sense.)

    I know some doctors think it’s too inclusive actually, and almost no one gets the names straight. I’ve also yet to meet a fibromyalgia patient in real life since studying this deeply who does not pass the Brighton (with an “r”) diagnostic criteria (and quite often also the Beighton score as well) for EDS. Their jaws usually drop and they confess their family has a history of pain and trouble once gently probed. I’m pretty sure it’s just a manifestation of a CTD now, but I’m not a doctor. Just a pedantic and passionate patient!

    Anyway, we can only hope and pray they come up with at least a better and less confusing name if not system during this year’s International EDS Symposium in NYC where the Professional Advisory Network is meeting to hash out this very thing again in hopes of making it easier for all involved. Toes carefully crossed! http://ednf.org/eds-international-symposium-2016

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  2. The Phoenix

    I agree, doctors need to be more aware of ALL of the signs of EDS and other connective tissue disorders. Like you, if I had not researched hypermobility on my own I never would’ve gotten to a doctor who knew enough about it to diagnose me. I was surprised to find that the reason I do so poorly on the Beighton and Brighton tests is because my connective tissue disorder is from the Marfan family of connective tissue disorders, not from the EDS family. Since Marfan-type connective tissue disorders and some types of EDS can have life-threatening complications without proper precautions, why aren’t all doctors aware of all of the signs?

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    1. Jak Post author

      It’s all so complicated isn’t it? I strongly suspect Marfan in my family – my brother is 6ft 2″ with very long arms, much stretchier skin than me yet no hypermobility. My Mum has pectus cavinatum (ie pigeon chest) and also had a collapsed lung for no reason in her 40s. All of which suggest Marfans. Of course, getting to see someone knowledgeable about Marfan in the north of england is impossible so we all just go by not knowing – scary being as though, as you say, it can be fatal.

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      1. The Phoenix

        Yes, that is scary. My brother and I kind of freaked out when he got sent to be checked for Marfans even thought he seems perfectly healthy and I’m the one with the serious hypermobility symptoms.

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  3. Elizabeth Milo

    This was eye-opening for me; I didn’t realise I was so like you in terms of EDS diagnostic criteria. I only have 1 or 2 on the Brighton Scale, bursitis in my shoulders, prolapses and, like you, not typical joint pain–mine travels. I also have all the other atypical symptoms you mentioned bar excessive bleeding and a positive Gorlin’s sign (but my Mum does). And I do have some hypermobility in my ankles, neck, shoulders, hips, but not enough that I paid attention to it. Now I just have to figure out how this knowledge can help me in the future since I’m currently so mild.

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    1. Jak Post author

      Glad you found the post enlightening EM. Marfans is about ratio of arm span to height – you can measure it at home, so it’s worth doing. My only hope is that they can gene sequence Classical EDS better in the near future and of course find the gene responsible for Hypermobile EDS – it would take all the guesswork out! I’d also love my family to be tested for the Marfans gene though have no clue how to go about this where I live x

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