| Hypermobility
clinical features
In the syndromic forms of hypermobility
there are other features of the relevant syndrome. In the idiopathic form
clinical features include:
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joint pains, particularly in the knee following
exertion
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three or more signs of ligamentous laxity:
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elbow hyperextension of greater than or
equal to 10 degrees.
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ability to push back thumb to touch the
anterior surface of the forearm.
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flexion of 5th metacarpophalangeal joint
to 90 degrees
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knee hyperextension of greater than or
equal to 10 degrees.
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ability to put palms on floor with knees
extended
joint laxity
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Hypermobility is common, and is more frequent
in girls than boys. There are some specific causes, but mild hypermobility
can be idiopathic.
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Management is by avoidance of activity
that causes pain.
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Prognosis is good, with reduction of hypermobility
with age. However, in severe instances secondary arthritis may develop.
Dysautonomia Common in
Joint Hypermobility Syndrome
NEW YORK (Reuters Health) Jul 24 - Dysautonomia
appears to be an extraarticular manifestation of joint hypermobility syndrome,
according to results of a study conduced in Israel.
To better understanding the pathophysiology
of symptoms such as orthostatic tachycardia and subjective hypohidrosis
(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
presyncopal 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 hyperresponsive
reactions to beta-1 and alpha-1 adrenoreceptors, 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 hemodynamic 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.
Association between hyperflexibility
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]
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
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:
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Can you now (or could you ever) place your
hands flat on the floor without bending your knees?
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Can you now (or could you ever) bend your
thumb back to touch your forearm?
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As a child did you amuse your friends by
contorting your body into strange shapes or could you do the splits?
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As a child or teenager did your shoulder
or knee cap dislocate on more than one occassion?
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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 |
