Hands signs in Marfan syndrome: thin fingers, long hand shape & hypermobility!
Marfan syndrome is a connective tissue disorder characterised by a tall, slender body featured with long limbs & long thin fingers. The most serious complications are the defects of the heart valves and the aorta, which could lead to an aortic rupture (due to too much stress on the aorta), which is usually fatal. However, many people who have this disorder are not aware of it – partly because Marfan syndrome typically becomes manifest only after the age of 5. But there are hand signs that have a highly reliable diagnostic value!
Marfan syndrome is featured with many typical hand characteristics, however a combination of two specific hands signs related to a long hand shape (hand signs) & hand motorics (joint hypermobility) is often enough to identify the disorder.
The Steinberg sign & Walker-Murdoch sign.
THE STEINBERG SIGN (a):
This test is used for the clinical evaluation of Marfan patients.
Procedure:
Instruct the patient to fold his thumb into the closed fist. This test is positive if the thumb tip extends from palm of hand (see figure a).
THE WALKER-MURDOCH SIGN (b):
This test is used for the evaluation of patients with Marfan syndrome.
Procedure:
Instruct the patient to grip his wrist with his opposite hand. If thumb and fifth finger of the hand overlap with each other, this represents a positive Walker-Murdoch sign (see figure b).
JOINT HYPERMOBILITY
How to check if a person has hypermobility? You can check this easily by doing the 5 tests that are included in the so-called ‘Beighton score‘: see figure 1.
A ‘Beighton score’ of 4 or above usually indicates hypermobility.
And if a person has the Sternberg sign + Walker-Murdoch sign + hypermobility, the chances are close to 90% that the person has Marfan syndrome.
The presence of other related hand markers such as: skin quality (hyperextensiblity), a simian crease, extra digital transverse creases, or a high positioned axial triradius provide other hand signs which are indicative for a person to have a medical diagnosis for Marfan syndrome.
Marfan hands.
MultiPerspective Palm Reading
Palm Reading Research
[...] The presence of other related hand markers such as: skin quality (hyperextensiblity), a simian crease, extra digital transverse creases, or a high positioned axial triradius provide other significant hand signs which are indicative for a person to have a medical test for Marfan syndrome. [...]
The hand in Marfan syndrome: thin fingers, hand signs, hypermobility, skin quality & more! | Hand Facts: News about hands!
May 24, 2011 at 5:00 am
Good web page
Matthew Syracuse
May 29, 2011 at 3:55 pm
Thank you!
martijnvanmensvoort
May 29, 2011 at 4:13 pm
Nice to see that this post featured at:
http://marfan-syndrome.crossfingers.info/term/marfan+syndrome+pictures+hands
martijnvanmensvoort
June 1, 2011 at 4:34 am
Oh god… I just discovered I have it. Oh my god.
Hein Limmen
August 16, 2011 at 6:40 pm
Hello Hein, feel free to share more details about how you made your discovery exactly….
martijnvanmensvoort
August 17, 2011 at 12:36 pm
i just discovered i have it too.
for years i knew i could do ‘abnormal’ things that my friends couldnt.
i score a 9 on the Beighton score. and i can do the hand gestures.
although, i am not tall. i have long limbs and a small torso. i am only 5″2 and 16 years old.
bek
August 17, 2011 at 7:43 pm
Hello Bek, thank you for your report!
martijnvanmensvoort
August 17, 2011 at 7:50 pm
I think it’s important to note that a diagnosis requires more than just these findings. These are the diagnostic criteria for Marfan syndrome: http://www.ncbi.nlm.nih.gov/books/NBK1335/#marfan.Diagnosis
GCNerd
August 22, 2011 at 12:17 am
Agreed – its really important to look at the actual criteria for diagnosing Marfan Syndrome, because these tests don’t even come close to addressing the most important two (aortic dilation and ectopia lentis). In fact, most of the hypermobility issues aren’t even included in a real Marfan diagnosis.
Martin, could you please inform us where you found this data? What medical studies have shown that having these signs makes the chances of having Marfan Syndrome 90% or more? It’s important to raise awareness, but if this information has backing from real medical data and real studies, it’s also important for your readers to see it. In the meantime, I suggest readers check out this information: http://www.marfan.org/marfan/4265/Diagnostic-Criteria
ConcernedAboutTheValidityOfThisArticle
February 21, 2012 at 1:31 am
Hello … (?),
Thank you for your question!
The 90% percentage concerns only a rough estimate (my estimate), but I can share some evidence that my estimate is actually rather ‘conservative’:
The 1997 study ‘Evolving phenotype of Marfan’s syndrome‘ reports the following for a sample of 40 Marfan children:
“The presence of arachnodactyly was defined clinically by a positive Steinberg sign when the thumb protrudes from the ulnar border of the hand when apposed across the palm20 and a positive Walker-Murdoch sign when a combination of slim wrists and long digits results in noticeable overlap of the thumb and little finger when the wrist is grasped.21 This was found in all except three children and was identifiable from birth in five.
thirty one children had pes planus which in most was associated with joint hypermobility with a Beighton score of greater than 5/9.”
This implicates:
- the combination of the Steinberg sign and the Walker-Murdoch sign was found in 37 out of 40 Marfan children (=92%);
- and additinonally in (almost) 30 of the 40 children (=75%) a Beighton score higher than
five
was found.
Combining these two figures confirms that my 90% estimate mentioned in my article is sensible + realistic. And as a matter of fact, my estimate could even be described as ‘conservative’ – especially since
I have used in my article a Beighton score higher than four
, which indicates that in an arbitrary Marfan population the 90% percentage could very well turn out to be even higher in practice.
How does this sound to you? Have I answered your question?
Greetings from The Netherlands!!
martijnvanmensvoort
February 21, 2012 at 4:03 pm
[...] • 5 Hand signs in Marfan syndrome [...]
Can hand signs signal disease & psychiatric disorders? | Hand Facts: News about hands!
August 25, 2011 at 12:40 am
I got a seven on the Beighton score :O
kat
October 14, 2011 at 1:52 am
if I only scored 2, and couldn’t do A or B does this mean I do not have marfan sydrome? bare in mind that i have a sunken chest, a moderately arched pallete, close teeth, long slender finger, and 6ft 135 pounds, i also have stretch marks on my back when i had a growth spurt.
Would really appreciate if someone would reply as I’m extremely worried. also I’m 22 years old
Terry
December 16, 2011 at 11:54 pm
Yes Terry, you probably don’t have Marfan syndrome – since you don’t have the Steinberg sign, nor the Walker-Murdoch sign, nor do you qualify for hypermobility.
If you have more worries regarding your body, please consult your general-practitioner in order to ease your mind.
Thank you for your comment.
martijnvanmensvoort
December 31, 2011 at 2:55 am
Wow… I think I have it. I can do both a and b and got a 7 on the 3rd test. But I’m not tall. I just have long thin fingers and arms.
Bori
February 11, 2012 at 5:44 pm
Hello Bori,
While many individuals who have Marfan syndrome grow to above height, many have normal height. So, you do appear to have most of the typical characteristics.
Do you have any problems with your wrist, spine or eyes?
(At wikipedia you can find the most common problems in Marfan syndrome for these body parts: http://en.wikipedia.org/wiki/Marfan_syndrome#Signs_and_symptoms )
Anyway, thank you for your report so far!
Greetings from The Netherlands.
martijnvanmensvoort
February 11, 2012 at 7:28 pm
My son has long slender fingers does that mean he has it he didnt pass the Steinberg sign but passed the walker murdoch sign im so worried he didnt pass some of the other test either cant you just have long slender hands without have Marfan please put my mind at ease shall i do the test of hand span when you measure the distance to your height is thst good indicator as i have read this is another test boy am i worried thanks susan
susan
February 12, 2012 at 11:38 am
Hello Susan,
First of all, ‘long slender fingers’ & the ‘hand span when you measure the distance to your height’ should not be considered as representing reliable indicators for Marfan syndrome.
Second, I think your son doesn’t show (m)any signs of joint hypermobility; and because he basically only passes the Walker-Murdoch sign… therefore I think you son has a pretty high chance that he DOES NOT have Marfan syndrome.
I hope this will easy your mind (permanently).
Thank you for sharing your question!
martijnvanmensvoort
February 12, 2012 at 4:52 pm
There are many differential diagnosis for long fingers/positive wrist or thumb signs. Also people can have Marfan without being tall or have long fingers. I think it is appalling that you are on here diagnosing people? when you are not a doctor but a psychologist (albeit with an interest in hand therapy!).
Do you have any idea of the implications for someone to believe but not KNOW that they may have Marfans? Insurance? Self esteem? body image? no cure? etc etc . I cannot believe the arrogance and naive stupidity/cynical manoeuvring that is on here!
The only way to diagnose/rule out marfan is to see a PROPER doctor – and then only if there is a reason why you feel you need to! if something has no cure perhaps it is better to not know? and take your chances? and live a happy life? rather than one ruled by possibly endless/needless fear and anxiety! (and thereby keep charlatans out of the equation) :s
Mag
February 25, 2012 at 5:22 am
Hello Mag,
I think you are confusing the process of observing/discussing the chances (based on scientific evidence) that an individual has Marfan syndrome with the process of diagnosing Marfan syndrome.
There is no clear cut (blood) test available for diagnosing Marfan syndrome, and even the genetic test are arbitrary due to the fact that the proces of diagnosis Marfan syndrome is still a matter of ‘clinical evaluation’. Which implicates that even when consulting a doctor for diagnosing Marfan syndrome, the result will depend on which criteria he/she decides to use.
Basically, I have only been discussing here the perspective of the hand… but I have reported in the last sentence of my article additional (hand related) criteria – which could also make the difference of whether it would be appropriate for an individual to have a check-out for a Marfan diagnosis.
In the article & my responses to questions I have talked about chances (in the perspective of ‘diagnostic value’) – I never claimed to be able to make a permanent diagnosis.
(By the way, you forget to mention whether you have yourself any experience in the perspective of Marfan syndrome – which could indicate that you are judging my article/responses without knowing much about this topic…?)
You are welcome to respond & share your additional views/experience!
martijnvanmensvoort
February 25, 2012 at 2:29 pm