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Hypermobility: prevalence and features in a Swedish population.

In this investigation, parallel to an earlier study [1] of 660 musicians from the USA, 606 individuals from a factory in Ostersund, Sweden, were studied to determine the prevalence and nature of their hypermobility. They were examined using the same protocol and by the same examiner as in the earlier study for the presence of five well recognized features of hypermobility. In this mostly middle-aged population of workers, as in the earlier population of mostly young musicians, joint laxity was found to be far more common among females. In both sexes hypermobility is seen to decline with age, the rate of fall being about the same for men and women.

Adult↗

British consultant rheumatologists' perceptions about the hypermobility syndrome: a national survey.

Three hundred and nineteen UK-based consultant rheumatologists, members of the British Society for Rheumatology (a response rate of 76%), responded to a questionnaire concerning their perceptions of the (benign joint) hypermobility syndrome (HMS). The questions were wide-ranging and covered the nature of the condition, its clinic prevalence, criteria for diagnosis, the efficacy of chosen treatments, the impact of the syndrome on affected individuals and the contribution that it makes to the overall burden of rheumatic disease morbidity. Ninety-two per cent of the respondents believed in the HMS as a distinct clinical entity but only 39% accepted it as a distinct pathological entity. Only 42% were prepared to comment on whether the HMS and Ehlers-Danlos syndrome, hypermobility type (formerly EDS type III) were one and the same entity. There was striking variability in estimated clinic prevalence and no consensus about the diagnostic criteria being used. There was little enthusiasm for treatments currently available. Nearly one-half of the respondents were sceptical about a significant impact of the HMS on people's lives and three-quarters about a significant contribution to the overall burden of rheumatic disease. There was little sign of awareness of findings in recent published studies. It seems unlikely, therefore, that evidence-based medicine is being practised in this area of rheumatology. An unexpected finding was a refreshing enthusiasm for joining regional interest groups on hypermobility (25% of all UK consultants expressed interest).

Diagnosis, Differential↗

Joint hypermobility syndromes.

Inherited connective tissue disorders, among them Ehlers-Danlos syndrome, osteogenesis imperfecta, Marfan syndrome, and Larsen syndrome, are characterized by generalized joint hypermobility. Others, such as Morquio syndrome or achondroplasia, have hypermobility in a more limited distribution. Recent clinical and molecular genetic studies have defined the range of clinical variation and the underlying molecular defects in several disorders. These studies provide hope that more common varieties of joint hypermobility can be understood and that effective therapies are within reach.

Cervical Vertebrae↗

Hypermobility and deconditioning: important links to fibromyalgia/fibrositis.

In this study 210 patients who were identified as having fibromyalgia/fibrositis were evaluated for hypermobility of joints. The patients were then instructed to perform an exercise program, and thereafter their compliance with the program and its effect on their symptoms were evaluated. The data were then analyzed using the Student's t test and Pearson's correlation coefficient. Patients who exercised during the study had improvement. Because the hypermobile patients as a group exercised more than the nonhypermobile group, they showed greater improvement than the nonhypermobile group (58% versus 30%). Patients with fibromyalgia/fibrositis who have articular hypermobility are more likely to exercise, which will improve their symptoms. The ability to show the objective findings of hypermobility to a person with fibromyalgia/fibrositis, a diagnosis based on subjective symptoms and signs, facilitates management of the patient. These tangible signs help the patient accept and adjust to the diagnosis of fibromyalgia/fibrositis.

Adult↗

Occipitoatlantal and occipitoaxial hypermobility in Down syndrome.

STUDY DESIGN: In this study, the authors evaluated upper cervical spine in 75 children and adolescents with Down syndrome on the basis of lateral flexion-extension radiographs. OBJECTIVE: To assess occipitoatlantal motion and occipitoaxial motion in children and adolescents with Down syndrome compared with age-matched control subjects. SUMMARY OF BACKGROUND DATA: Although previous studies have described a high prevalence of occipitoatlantal hypermobility in Down syndrome, there have been no comparisons with age-matched control subjects. Only a few reports have mentioned the physiologic relation between the occiput and axis other than basilar impression. Moreover, there have been no reports examining anteroposterior mobility in abnormal conditions. METHODS: Seventy-five children and adolescents with Down syndrome and 30 age-matched control subjects were examined. Lateral radiographs of the upper cervical spine in flexion and extension were taken, and anteroposterior translation of the occiput in relation to the atlas and axis was measured. RESULTS: Anteroposterior occipitoatlantal hypermobility was found to be present in children and adolescents with Down syndrome even when compared with age-matched control subjects. Occipitoaxial hypermobility was observed only when atlantoaxial instability was present. CONCLUSION: In evaluating the upper cervical spine in Down syndrome, it is necessary to pay attention to the relation between the occiput, atlas, and axis.

Adolescent↗

Posterior occipitoatlantal hypermobility in Down syndrome: an analysis of 199 patients.

We endeavored to determine the prevalence of occipitoatlantal hypermobility in individuals with Down syndrome, to establish objective radiographic criteria for this entity, and to correlate this with neurologic abnormality. In a retrospective analysis, upper cervical spine radiographs of 210 patients with Down syndrome were compared with those of 102 normal individuals. Radiographs were evaluated using the Powers ratio. Patients identified with radiographic evidence of posterior occipitoatlantal hypermobility were then examined clinically and compared with a matched group of patients with Down syndrome and normal Powers ratios. Of the patients with Down syndrome, 8.5% had a Powers ratio of < 0.55, which was indicative of posterior occipitoatlantal hypermobility (POAH). Furthermore, 66% of those with an abnormal Powers ratio had positive neurologic findings upon physical exam, a finding that was statistically significant when compared to a matched group of patients with Down syndrome and normal Powers ratio.

Adolescent↗

The relationship between joint hypermobility and neurodevelopmental attributes in elementary school children.

Joint hypermobility is associated with motor developmental delay in infancy. To assess this finding in school-aged children, 320 first- and second-grade elementary school children and 110 children attending a special education program were assessed. Joint hypermobility was found in 40 (12.4%) and seven (6.4%) of the children attending the regular and special education classes, respectively. No difference in the neurologic status or verbal and eye-hand coordination task performance was found between the children of the study group and their age- and sex-matched controls. It appears that joint hypermobility and neurodevelopmental dysfunctions are not causally related and have a different maturational course.

Child↗

Associated deformities and hypermobility in hallux valgus: an investigation with weightbearing radiographs.

BACKGROUND: Hallux valgus has been reported to recur after surgical correction in patients subsequently diagnosed with hypermobility of the first ray, pronation of the foot, and pes planovalgus. An objective means of assessing the foot for these deformities preoperatively may avert a poor outcome. This investigation evaluated the efficacy of full-length weightbearing radiographs to recognize associated deformities in patients with hallux valgus before surgery. METHODS: This study compared five parameters from anteroposterior and four parameters from lateral weightbearing pedal radiographs of patients with moderate to severe hallux valgus to a control group to identify differences in the alignment of the midfoot and the first metatarsal-medial cuneiform joint. An examination for clinical evidence of hypermobility was also performed on both groups. RESULTS: The hallux valgus group demonstrated increased abduction and dorsiflexion of the midfoot. The mean talonavicular coverage angle and lateral talo-first metatarsal angle of this group was greater than the mean values for the controls. Radiographic evaluation also revealed differences in the alignment of the first metatarsal-medial cuneiform joint in the sagittal plane. The hallux valgus group possessed a mean of 2 mm of dorsal translation and 2 degrees of dorsiflexion at this joint compared to the controls. CONCLUSIONS: Weightbearing radiographs permit the recognition of associated malalignments of the foot in patients with hallux valgus. The comparisons performed in this study identified deformities consistent with pes planovalgus and hypermobility of the first ray in patients with moderate to severe hallux valgus. The results of our study support a recommendation for a thorough evaluation of full-length, biplanar weightbearing radiographs via the measurement of midfoot and first ray alignment for concomitant deformities of the foot in patients with hallux valgus.

Adolescent↗

Pubovaginal sling in the treatment of stress urinary incontinence for urethral hypermobility and intrinsic sphincteric deficiency.

PURPOSE: This study was undertaken to evaluate the use of pubovaginal sling for the treatment of female stress urinary incontinence in patients with intrinsic sphincteric deficiency and patients with urethral hypermobility. MATERIALS AND METHODS: Sixty-two patients aging 22 to 73 years-old (mean = 49.6) with a median parity of 4.1 (range 0 - 14) who underwent pubovaginal autologous fascial sling procedures for stress urinary incontinence from August/1999 to August/2002 were prospectively analyzed. Objective pre and postoperative urodynamic evaluation was performed in all cases. The patients were divided into 2 groups: thirty-nine patients (62.9%) with urethral hypermobility (Valsalva leak point pressure equal or superior to 60 cm of H(2)O) and twenty-three patients (37.1%) with intrinsic sphincteric insufficiency (Valsalva leak point pressure below 60 cm of H(2)O). RESULTS: The average follow-up period was 24.8 months, ranging from 3 to 38 months. Three patients (4.8%) had detrusor overactivity before the operation, and 36 patients (58.1%) had voiding dysfunction before surgery. The postoperative objective cure rate was 88.7% for stress urinary incontinence. The study also showed that 32.2% of the patients had voiding dysfunction and 11.3% had detrusor overactivity. The mean hospital stay was 3.1 days (range 2 - 4). No difference in the above parameters was noticed between patients with intrinsic sphincteric deficiency and those with urethral hypermobility. CONCLUSION: Construction of a pubovaginal sling is an effective technique for the relief of severe stress urinary incontinence, for both patients with urethral hipermobility and with intrinsic sphincteric deficiency, having a cure rate of 88.7%. The high frequency of postoperative voiding urgency was not related to the detrusor overactivity as evaluated by urodynamic studies.

Journal Article↗

Collagen implant for treating stress urinary incontinence in women with urethral hypermobility.

PURPOSE: We evaluated the use of collagen in women with stress urinary incontinence and urethral hypermobility. MATERIALS AND METHODS: We treated 90 patients with stress urinary incontinence and urethral hypermobility with 1 to 3 injections of Contigen implant (C. R. Bard, Inc., Covington, Georgia) between June 1996 and October 1998. Observations at 3, 6 and 12 months included continence grade, 7-day voiding diaries and quality of life questionnaires. Abdominal leak point pressure was determined at baseline and 12 months. RESULTS: At the 6-month followup 68 patients remained in the study, of whom 30 (44%) were dry and 24 (35%) were improved. Of the 58 patients who reached the 12-month followup 19 (33%) were dry and 19 (33%) were improved. Considering all patients entered into the study at 6 months 30 (33%) were dry and 24 (27%) were improved. Of the original 90 patients 19 (21%) were dry and 19 (21%) were improved at 12 months. The probability of maintaining initial improvement for 12 months was 44%. The success of bulking agent therapy was not predicted by the initial incontinence grade. Improved continence grade correlated with improved leak point pressure. CONCLUSIONS: This therapy is appropriate in women with urethral hypermobility who wish to avoid surgical risks and in those in whom surgery is ill advised.

Adult↗

Generalized joint hypermobility and temporomandibular disorders: inherited connective tissue disease as a model with maximum expression.

AIMS: To study the relationship between generalized joint hypermobility (GJH) and temporomandibular disorders (TMD) by assessing prevalence and patient characteristics of TMD in a population of patients with maximum expression of GJH as a symptom of inherited connective tissue disease. In addition, diagnostic reliability of a series of clinical signs indicative of temporomandibular joint (TMJ) hypermobility was tested. METHODS: The study sample consisted of 42 subjects with GJH, 24 with Marfan syndrome and 18 with Ehlers-Danlos syndrome. A subgroup of 27 individuals was selected by age (> or = 18 yrs) and was compared to 40 controls with TMD and normal peripheral joint mobility. TMD diagnoses were assigned to each subject according to the Research Diagnostic Criteria for Temporomandibular Disorders (RDC/TMD). RESULTS: In the GJH sample (n = 42), 71.4% of the subjects were symptomatic for TMD. Of those, 13.3% had sought treatment. A myofascial pain diagnosis was made in 69%, disc dislocation with reduction was diagnosed in 85.7%, and TMJ arthralgia in 61.9%. Multiple TMD diagnoses were assigned in 69% of the subjects; of these, 57% had 3 or more subgroup diagnoses. Joint noises (P < .01) and recurrent TMJ dislocations (P < .01) were a frequent finding in adult GJH subjects (n = 27) compared to controls, with symptomatic GJH subjects presenting more and more prolonged dislocation events than asymptomatic subjects (P < .001). TMJ hypermobility signs were expressed significantly more often in GJH compared to controls with TMD and normal joint mobility. CONCLUSION: This study indicates a positive relationship between GJH and TMD.

Adolescent↗

Stress incontinence and low urethral closure pressure. Correlation of preoperative urethral hypermobility with successful suburethral sling procedures.

Forty-eight women with genuine stress incontinence and low urethral closure pressure were treated with a suburethral sling procedure using polytetrafluoroethylene. All patients underwent a preoperative clinical evaluation and multichannel urodynamic testing. The clinical examination included a "Q-tip" test to determine the presence or absence of urethral hypermobility. Urethral hypermobility was defined as a maximal angle change of greater than or equal to 30 degrees from the horizontal, measured during straining or coughing in the lithotomy position. Thirty-four patients underwent repeat multichannel urodynamic testing three months postoperatively to determine the objective surgical success. Ninety-three percent of patients (27/29) with a positive preoperative Q-tip test were cured. Of patients with a negative preoperative Q-tip test, only 20% (1/5) were cured. Preoperative urethral hypermobility was a good prognostic indicator of operative success when a suburethral sling procedure was used to treat genuine stress incontinence and low urethral closure pressure.

Adult↗

Arthritis/arthralgia and hypermobility of the joints in schoolchildren.

Studies of pediatric clinic populations have shown that a high proportion of children with rheumatic complaints demonstrate hypermobility of the joints. In order to compare the frequency and nature of articular complaints in children with hypermobility to that seen in nonhypermobile controls, we examined 192 normal students aged 5-19 years. Overall, 34% (41/109 girls and 25/83 boys) were found to be hypermobile. Consenting parents of hypermobile children were given a questionnaire and interview designed to detect a history of arthritis/arthralgia, as were parents of age and sex matched nonhypermobile controls. Fifty percent of the hypermobile group had a history of arthralgia, compared to 20% of controls. Ten percent in each group had had arthritis. Data from our comparative study supports the possible association between joint hypermobility and the development of articular complaints in children.

Adolescent↗

A hypermobility study in ballet dancers.

Hypermobility, using Beighton's mobility score, was present in 9.5% of 377 ballet dancers. Females were more mobile than males. A direct relationship was found between forward flexion and both the duration of dance training and age. This suggests that forward flexion is acquired, and unlike generalized hypermobility, which was found among family members of the hypermobile group. None of the hypermobile dancers studied had evidence of mitral valve prolapse but injuries were significantly more common compared to controls (p less than 0.05). We regard hypermobility as a liability to the ballet dancer.

Adolescent↗

The hypermobility syndrome.

The hypermobility syndrome has been recognized as a definitive diagnostic entity among children referred to a Pediatric Arthritis Clinic with musculoskeletal complaints. The diagnosis of hypermobility was made by the ability of the patients to perform at least three of the following maneuvers: (1) extension of the wrists and metacarpal phalanges so that the fingers are parallel to the dorsum of the forearm; (2) passive apposition of thumbs to the flexor aspect of the forearm; (3) hyperextension of elbows (greater than or equal to 10 degrees); (4) hyperextension of knees (greater than or equal to 10 degrees); (5) flexion of trunk with knees extended so palms rest on the floor. Of 262 patients, 15 (5.7%) referred to an arthritis clinic between January 1979 and July 1981 were subsequently determined to have hypermobility as the basis for their rheumatic complaints. Three of these 15 had concomitant juvenile arthritis. The presenting complaint of pain was most often localized to the knees, hands, and fingers. Arthritis and elevated ESRs were not seen except in the three patients who had concomitant juvenile arthritis. All patients responded to physical therapy and nonsteroidal analgesic anti-inflammatory drugs. This is an entity not sufficiently well recognized as a source of musculoskeletal complaints in the United States. It will usually respond well to reassurance and symptomatic therapy.

Adolescent↗

Treatment of stress urinary incontinence in women with urethral hypermobility and intrinsic sphincter deficiency.

PURPOSE: We compared 2 treatment modalities (sling cystourethropexy and periurethral collagen injection) in patients with intrinsic sphincter deficiency alone or with urethral hypermobility (combined stress urinary incontinence). MATERIALS AND METHODS: We retrospectively reviewed a series of 50 consecutive patients treated surgically for intrinsic sphincter deficiency during a 2-year period. All patients were evaluated by history and physical examination to assess urethral hypermobility and urodynamic testing. Intrinsic sphincter deficiency was assessed by abdominal leak point pressure and video urodynamics. Of the 50 patients 28 underwent a pubovaginal sling operation and 22 received a periurethral injection of collagen. RESULTS: Of the patients studied 40% had combined stress urinary incontinence. A pubovaginal sling procedure resulted in a cure rate of 81% in this group, compared to 25% for periurethral injection of collagen. CONCLUSIONS: A subgroup of women exists with combined stress urinary incontinence due to urethral hypermobility and intrinsic sphincter deficiency. When treated with sling cystourethropexy women with combined stress urinary incontinence do as well or better than those with intrinsic sphincter deficiency alone and those treated with periurethral collagen injection do worse.

Adult↗

Cardiovascular abnormalities in the Marfanoid hypermobility syndrome.

Two patients are described who had the body habitus of the Marfan syndrome, cutaneous hyperextensibility, and atrophic "cigarette paper" scars. One had mild joint hypermobility and the other had generalized ligamentous laxity. Both individuals are considered to be examples of the Marfanoid hypermobility syndrome, a generalized heritable connective tissue disease with features of, but genetically distinct from, the Marfan and Ehlers-Danlos syndromes. One patient had a "floppy" mitral valve and the other had an aortic aneurysm due to cystic medionecrosis of the aorta; both findings provide evidence that cardiovascular disease may be a significant feature of this syndrome.

Adult↗

Amelioration of symptoms by enhancement of proprioception in patients with joint hypermobility syndrome.

OBJECTIVE: Joint hypermobility syndrome (JHS) is characterized by an association between joint hypermobility and musculoskeletal pains, the latter occurring in the absence of any objective indicator of rheumatic disease. The lack of a recognizable disease marker makes this condition difficult to identify and manage. We previously observed that patients with JHS have impaired proprioception compared with that of a matched control group. The purpose of this study was to investigate whether a home-based exercise program could produce objective enhancement of proprioception as well as alleviate symptoms in JHS. METHODS: A threshold detection paradigm was used to assess knee joint proprioception, balance was assessed using a balance board, and quadriceps and hamstring strength were measured by an isokinetic dynamometer. A visual analog scale was used to assess musculoskeletal pain, and quality of life was evaluated by a Short Form 36 questionnaire. Assessments were performed before and after an 8-week program of progressive closed kinetic chain exercises. RESULTS: Following the exercise program, proprioceptive acuity increased in 16 of 18 subjects and was very significantly improved overall (P < 0.001). There was a comparable improvement in performance on the balance board (P < 0.001), and quadriceps and hamstring strength also increased significantly. Symptomatic improvement also occurred, in terms of both pain (P = 0.003) and quality-of-life (P = 0.029 for physical functioning; P = 0.008 for mental health) scores. CONCLUSION: Appropriate exercises lead not only to symptomatic improvement, but also to demonstrable enhancement of objective parameters such as proprioception.

Adolescent↗