does dance predispose us to dysautonomia? or are dysautomaniacs predisposed to dance?

That’s me in 1985, before it all went badly wrong

Hypermobility

clinical features

In the syndromic forms of hypermobility there are other features of the relevant syndrome. In the idiopathic form clinical features include:

• joint pains, particularly in the knee following exertion
• three or more signs of ligamentous laxity:
• elbow hyperextension of greater than or equal to 10 degrees.
• ability to push back thumb to touch the anterior surface of the forearm.
• flexion of 5th metacarpophalangeal joint to 90 degrees
• knee hyperextension of greater than or equal to 10 degrees.
• ability to put palms on floor with knees extended

joint laxity

• Hypermobility is common, and is more frequent in girls than boys. There are some specific causes, but mild hypermobility can be idiopathic.
• Management is by avoidance of activity that causes pain.
• Prognosis is good, with reduction of hypermobility with age. However, in severe instances secondary arthritis may develop.

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Dysautonomia Common in Joint Hypermobility Syndrome

NEW YORK (Reuters Health) Jul 24 – Dysautonomia appears to be an extra-articular manifestation of joint hypermobility syndrome, according to results of a study conduced in Israel.

To better understanding the patho-physiology of symptoms such as orthostatic tachycardia and subjective hypo-hidrosis (can’t sweat adequately) in patients with the syndrome, Dr. Giris Jacob, of Rambam Medical Center, Haifa, and colleagues evaluated the frequency of autonomic nerve system complaints and performed autonomic function tests in 48 consecutive patients and 30 sex- and age-matched healthy volunteers.

According to their report in the American Journal of Medicine for July, all 48 patients and 3 volunteers had at least five orthostatic symptoms for at least 6 months. Patients commonly experienced pre-syncopal symptoms, palpitations, chest discomfort, fatigue and heat intolerance.  Even though there were no cardiovagal abnormalities, the patients exhibited signs of dysregulated sympathetic nervous system control. For example, the drop in systolic blood pressure during hyperventilation averaged 11 mm Hg in patients and 5 mm Hg in controls.

Patients also exhibited hyper-responsive reactions to beta-1 and alpha-1 adreno-receptors, as evidenced by lower doses of isoproterenol and phenylephrine required to increase heart rate or systolic blood pressure.  These patients generally don’t require extensive autonomic function testing,

Dr. Jacob told Reuters Health. “The main thing is to get a complete medical history regarding the autonomic nervous system, and hemo-dynamic data, such as blood pressure and heart rate in the supine position and after standing for 5 minutes.”   He believes that pharmacologic treatment is required only when quality of life is compromised significantly by the autonomic symptoms. “A high-salt diet, extra fluid intake, and physical therapy to strengthen the muscles in the lower extremities proved to be very helpful,” he said.  He and his colleagues are now planning to do genetic studies in patients with hypermobile joint syndrome, and to look at the pathophysiology of blood vessels.

Am J Med 2003;115:33-40.
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Association between hyper-flexibility of the thumb and an unexplained bleeding tendency: is it a rule of thumb?

Kaplinsky C, Kenet G, Seligsohn U, Rechavi G.

Department of Paediatric Haematology-Oncology and Institute of Thrombosis and Haemostasis, The Chaim Sheba Medical Centre, Tel-Hashomer, Israel.

A bleeding tendency manifested by petechiae and ecchymoses is one of the most common causes for referral of patients to haematology clinics. Vessel wall pathology is not usually considered to be a cause for deranged haemostasis, although coexistence of increased capillary fragility and joint hypermobility have been reported. We determined the frequency of thumb hyperextensibility and scored the findings in a series of 44 patients referred because of ecchymoses and petechiae, as well as 261 control children and their mothers. All 44 patients had normal coagulation studies. Thumb flexibility score was +4 in 30 patients, +3 in eight patients, +2 in five patients and +1 in one of the index patients. In the control group, only one of 261 had a +4, and three had a +3 score, and two of 260 mothers had a +4 score. Ecchymoses were not observed in any of these subjects, nor in the +1 patients. Based on clinical presentation and normal coagulation studies, we suggest that our patients had an underlying subtype of Ehlers-Danlos syndrome. In view of the dramatically high occurrence of thumb hyperextensibility in patients with unexplained mild bleeding tendency, costly haemostatic and coagulation studies on such patients may not be necessary.

PMID: 9609520 [PubMed – indexed for MEDLINE]
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Joint hypermobility and fibromyalgia in schoolchildren.

Gedalia A, Press J, Klein M, Buskila D.

Department of Pediatrics, Louisiana State University Medical Center, New Orleans 70112-2822.

OBJECTIVES--To test the hypothesis that joint hypermobility may play a part in the pathogenesis of pain in fibromyalgia, schoolchildren were examined for the coexistence of joint hypermobility and fibromyalgia.

METHODS--The study group consisted of 338 children (179 boys, 159 girls; mean age 11.5 years, range 9-15 years) from one public school in Beer-Sheva, Israel. In the assessment of joint hypermobility, the criteria devised by Carter and Bird were used. Any child who met at least three of five criteria was considered to have joint hypermobility. Children were considered to have fibromyalgia if they fulfilled the 1990 American College of Rheumatology criteria for the diagnosis of fibromyalgia, namely, widespread pain in combination with tenderness of 11 or more of the 18 specific tender point sites. The blind assessments of joint hypermobility (by AG) and fibromyalgia (by DB) were carried out independently. RESULTS–Of the 338 children 43 (13%) were found to have joint hypermobility and 21 (6%) fibromyalgia; 17 (81%) of the 21 with fibromyalgia had joint hypermobility and 17 (40%) of the 43 with joint hypermobility had fibromyalgia. Using chi 2 statistical analysis, joint hypermobility and fibromyalgia were found to be highly associated.

CONCLUSIONS–This study suggests that there is a strong association between joint hypermobility and fibromyalgia in schoolchildren. It is possible that joint hypermobility may play a part in the pathogenesis of pain in fibromyalgia. More studies are needed to establish the clinical significance of this observation.

PMID: 8346976 [PubMed – indexed for MEDLINE]

Latest Research

Prof Rodney Grahame & Dr Alan Hakim Department of Rheumatology, University College Hospitals, London

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Do you have HMS? (it is classified as an Ataxia)

We have been working on a 5-part questionnaire to identify hypermobility. If you answer yes to at least 2 of the 5 questions then there is an 80-90% chance you are hypermobile. We hope it will become a simple aide memoire for doctors and allied health practitioners, encouraging them to think about hypermobility as the cause for musculoskeletal problems.

The questions are:

• Can you now (or could you ever) place your hands flat on the floor without bending your knees?
• Can you now (or could you ever) bend your thumb back to touch your forearm?
• As a child did you amuse your friends by contorting your body into strange shapes or could you do the splits?
• As a child or teenager did your shoulder or knee cap dislocate on more than one occassion?
• Do you consider yourself to be double-jointed?

Ref: Hakim AJ, Graham R. A simple questionnaire to detect hypermobility: an adjunct to the assessment of patients with diffuse musculoskeletal pain. International Journal of Clinical Practice 2003; volume 57: p163-166

I’m 5 for 5, I’d say almost 100% of dancers etc. are
do does that predispose us to dysautonomia?
or are dysautomaniacs predisposed to dance?

better days, when I could fly