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Extra-articular features of benign joint hypermobility syndrome.

To define the phenotype of patients with benign joint hypermobility syndrome (BJHS), we studied 58 consecutive patients (mean age 37 yr) presenting to a rheumatology clinic and 30 controls. Patients underwent rheumatological and ophthalmic examination, hypermobility scoring, echocardiography, measurement of bone mineral density (BMD), and skin thickness, elasticity and light transmissibility. The median hypermobility score was 5/9 Beighton and 31/56 Contompasis. Eighteen (31%) patients complained of significant arthralgia. Six (10%) patients and two (7%) controls had mitral valve prolapse (MVP) (chi(2) = 0.27, P = NS). Neither MVP nor aortic diameters showed a correlation with hypermobility score. There was no significant reduction in BMD. There was a significant correlation between hypermobility and light transmissibility of the skin (r = 0.71, P < 0.0001 Contompasis; r = 0.47, P < 0.05 Beighton) and skin stretchiness (r = 0.49, P < 0.05 Contompasis; r = 0.39, P < 0.05 Beighton). On ophthalmic examination, 14 (41%) patients had upper eyelid laxity. Thus, patients with BJHS do not have an increased prevalence of significant cardiac, bone, skin or eye abnormalities, helping differentiate BJHS from other more serious hereditary disorders of connective tissue.

Adolescent↗

The relationship between generalized joint hypermobility and motor development.

PURPOSE: The aim of this study was to investigate the relationship between the presence and localization of joint hypermobility and delay in motor development. METHODS: A retrospective chart review was performed. All children younger than 2.5 years of age and between four and 12 years of age with generalized joint hypermobility were included. Generalized joint hypermobility was assessed using the criteria of Bulbena. In children between one and 2.5 years of age, motor development was measured using the Bayley Scales of Infant Development. In children four to 12 years of age, the Movement Assessment Battery for Children was used. RESULTS: Data from 72 children were available for analysis. In nine of 16 children younger than 2.5 years of age, a delay in motor development was found without a significant association between the delay in motor development (yes/no) and the Bulbena score (odds ratio: 0.4; 95% confidence interval: 0.07-2.1). In children between four and 12 years of age, a severe delay in motor development was present in 14 of 56 children (25%), while 12 of 56 children (21%) were at risk and 30 of 56 (54%) were age appropriate. No significant association between delay in motor development and the Bulbena score was found (odds ratio: 1.3; 95% confidence interval: 0.8-2.1). The age of independent walking was not significantly associated with the number of hypermobile joints (Bulbena score) (linear regression coefficient: 0.3; 95% confidence interval: -1.5 - 2.1). CONCLUSIONS: Although severe delays in motor development may be observed in approximately one third of children with generalized joint hypermobility, there is no association between the amount of generalized joint hypermobility and delay in motor development.

Child↗

Is condylar position a predictor for functional signs of TMJ hypermobility?

Temporomandibular joint (TMJ) hypermobility is noted only when it interferes with smooth mandibular movements. These interferences (viz. clicking sounds and jerky mandibular movements) result from condylar dislocation in front of the eminence at wide mouth opening, or alternatively in front of the articular disc (posterior disc displacement). The aim of this study was to test the hypothesis that condyles of hypermobile persons are positioned more anterosuperiorly to the crest of the eminence during maximum mouth opening than those of persons without TMJ hypermobility. Possible posterior disc displacement was also evaluated. Nine persons with symptomatic hypermobility and nine control persons free of internal derangements were included, their diagnoses being based upon opto-electronic movement recordings. Condylar positions during maximum mouth opening were analysed on magnetic resonance images with two slightly different methods, showing the degree to which the condyles are displaced around the eminence. No posterior disc displacements were found in any of the magnetic resonance images. After excluding an outlier and using both measurement methods, a small difference in condylar position was found between the two groups of subjects. The condyles of all hypermobile persons travelled beyond the eminence; however, so were the condyles of nearly half of the non-hypermobiles. The large overlap between both groups suggests that condylar position alone is not a good predictor for symptomatic TMJ hypermobility. It is probably the combination of condylar location in front of the eminence with a particular line of action of the masticatory muscles, which gives rise to functional signs of hypermobility.

Adult↗

Cervical spine joint hypermobility: a possible predisposing factor for new daily persistent headache.

The objective of this study was to suggest that joint hypermobility (specifically of the cervical spine) is a predisposing factor for the development of new daily persistent headache (NDPH). Twelve individuals (10 female, 2 male) with primary NDPH were evaluated by one of two physical therapists. Each patient was tested for active cervical range of motion and for the presence of excessive intersegmental vertebral motion in the cervical spine. All patients were screened utilizing the Beighton score, which determines degree of systemic hypermobility. Eleven of the 12 NDPH patients were found to have cervical spine joint hypermobility. Ten of the 12 NDPH patients had evidence of widespread joint hypermobility with the Beighton score. Based on our findings we suggest that joint hypermobility, specifically of the cervical spine, may be a predisposing factor for the development of NDPH.

Adolescent↗

Preterm premature rupture of membranes in a patient with the hypermobility type of the Ehlers-Danlos syndrome. A case report.

OBJECTIVES: This report wants to focus on the risk of severe prematurity in patients with the hypermobility type of the Ehlers-Danlos syndrome (EDS), a heritable disorder of connective tissue. Although various obstetrical complications have been reported in patients with EDS, most reports specifically comment on the severe complications in patients with the vascular type of EDS, including uterine and arterial rupture. Pregnancy outcome in patients presenting the hypermobility type of EDS is poorly documented. CASE: A 33-year-old nullipara was referred for preconceptual genetic counseling with a history of easy bruising, generalized joint hypermobility and chronic arthralgia and myalgia. The diagnosis of the hypermobility type of EDS was confirmed on clinical examination. During her first pregnancy, she underwent a prophylactic McDonald cerclage at 14 weeks' gestation. Premature rupture of membranes occurred at 23 weeks' gestation. A female infant was delivered at 26 weeks and died 3 h after birth. Electron-microscopic examination showed collagen fibre abnormalities in the fetus' skin, which were compatible with the diagnosis of EDS. CONCLUSIONS: Patients with the hypermobility type of EDS can have an increased risk for pregnancy complications, including prematurity due to cervical incompetence and to premature rupture of membranes. We therefore demand the clinician's alertness for possible signs of this underdiagnosed type of EDS and recommend the collaboration between the obstetrician and the medical geneticist in the obstetrical management of these patients.

Adult↗

Relationship between foot angles and hypermobility scores and assessment of foot types in hypermobile individuals.

This study proposed to assess foot types and radiological examination of feet of individuals with hypermobility syndrome in comparison to healthy matched controls for age, sex, and body mass index. The relationship between foot angles and hypermobility scores of subjects in both groups were also~assessed. Twenty individuals having joint hypermobility syndrome previously diagnosed according to Beighton and Bulbena scores were compared to 20 healthy subjects. Standing anteroposterior (AP) and lateral weigthbearing radiographs of feet of the individuals were taken. Foot angles and foot types were evaluated on the AP and lateral views. The mean +/- SD of Beighton and Bulbena scores were significantly higher in the hypermobile individuals (p <.001) than in controls. In both groups, Egyptian and square types of feet were mostly encountered (p >.05). Bilateral calcaneal pitch (CP) angles (p <.001), bilateral talometatarsal (TM) angles (p <.01), and right apex angles (p <.05) were significantly low in hypermobile individuals, whereas first metatarsophalangeal (MTP) angles were significantly high (right foot, p <.01; left foot, p <.05) as compared to the control group. Hypermobility scores correlated negatively with bilateral CP and TM angles and positively with bilateral first MTP angles. No correlation was found for the other~angles. These finding suggest that foot angles (especially CP, TM and first MTP angles) are in relation with severity of hypermobility. There was no difference between foot types in the hypermobile individuals and healthy controls. These angles may be useful for clinically monitoring the foot health in the hypermobile individuals.

Female↗

Generalized Joint Hypermobility and Its Relationship to Injury Patterns Among NCAA Lacrosse Players.

OBJECTIVE: To prospectively observe and compare injury patterns between hypermobile and nonhypermobile NCAA athletes. DESIGN AND SETTING: Athletes were screened for generalized joint hypermobility before the 1995 lacrosse season. Injuries were recorded through the end of the postseason and compared in hypermobile and nonhypermobile athletes. SUBJECTS: A total of 310 male and female volunteers from 17 lacrosse teams participated in the study. MEASUREMENTS: Hypermobility was evaluated with the technique of Carter and Wilkinson (as modified by Beighton and colleagues), which uses 9 joint measurements to assess global joint mobility. For an athlete to be considered hypermobile, 5/9 of these measurements must have been positive. Next, certified athletic trainers prospectively recorded injuries and hours of practice and game participation on a standard form. After the season, all data forms were returned to us for analysis. Significance was set at P = .05, and x(2) and independent t tests were used to compare injuries between groups. RESULTS: Twenty of 147 men (13.6%) and 54 of 163 women (33.1%) were hypermobile, yielding an overall hypermobility prevalence of 23.8%. One hundred athletes sustained 134 injuries. There were no significant differences in overall injury rate among hypermobile (2.29/1000 hours) compared with nonhypermobile (3.54/1000 hours) athletes. Nonhypermobile athletes suffered contact injuries at a higher rate (1.38/1000 hours) than hypermobile athletes (0.52/1000 hours). Hypermobile athletes showed an increased rate of ankle injuries, and nonhypermobile athletes showed a trend toward an increased rate of strains. Multiple approaches to analysis of the data revealed no other significant findings. CONCLUSIONS: There was no difference in overall injury rates between hypermobile and nonhypermobile athletes in this sample. This finding is somewhat surprising in light of significant evidence that hypermobility appears to be a factor in joint complaints among nonathletes. Additional research is needed to clearly determine whether a relationship exists between hypermobility and injury rates among athletes.

Journal Article↗

Joint hypermobility in pediatric practice--a review.

It has been well established that joint hypermobility may predispose children to the development of joint pain. Joint hypermobility represents an extreme variation of normal joint mobility with no underlying connective tissue diseases. Its prevalence among children varies from series to series. Evidence that there are differences between races and sexes exist. Joint hypermobility diminishes markedly throughout childhood and then more slowly during adult life. Although quantitative measurements in the assessment of joint hypermobility have been described, the criteria defined by Carter and Bird are preferred. Any subject who is able to perform 3 or more of the 5 maneuvers devised by these criteria was considered to have joint hypermobility. The mechanism in which joint symptoms develop is not well understood. Excessive motion or inappropriate physical activities that hyperextend the joint capsule, the ligaments and soft tissue constituent causing injury. These injuries are manifested by pain. The clinical features, prognosis and relevance to pediatric practice are discussed.

Child↗

[Hypermobility syndrome. When too much activity causes pain].

Hypermobility of joints and spine is a consequence of abnormal laxity of ligaments, joint capsules and intervertebral discs. Hypermobility per se is a state, not a disease. However, it may lead to generalized arthralgia or localized symptoms (frequent ankle sprains, knee effusions, dislocations of the shoulders of recurrent episodes of back pain). These events may occur even after minor strains. Young women are preferentially affected. The term "hypermobility syndrome" is defined as a combination of hypermobility with arthralgia, low back pain, frequent dislocation or sprains. It is often the result of a hereditary disorder of connective tissue. Individuals with hypermobility are at increased risk for premature osteoarthrosis or polytopic disc degeneration of the spine. However, the vast majority of hyperlax individuals are free of symptoms. Those with symptoms who do not develop osteoarthrosis or disc degeneration usually experience spontaneous improvement with increasing age, thus losing their juvenile hyperlaxity. This usually occurs between the ages of thirty and forty. The present review article also provides guidelines for management and patient information.

Adolescent↗

Generalized joint hypermobility and black admixture in school children of Bahia, Brazil.

Joint hypermobility was investigated in a sample of 3,000 school children, ages 6--7 years, in Bahia, Brazil. A sampling method was designed to assure an equal number of 50 children in the smallest size classes, when age, race, and sex were held constant. Race was classified in five subgroups to assess the proportion of black admixture. The overall frequency of generalized joint hypermobility was 2.3%. The darker the children the lower the frequency of affected children. However, this racial effect was also associated with age: The highest frequency of generalized joint hypermobility was among the youngest and less black mixed children, whereas among the oldest and darker children there was no case of joint hypermobility.

Adolescent↗

Hypermobility: features and differential incidence between the sexes.

Six hundred sixty individuals from a music school were studied to determine the frequency of incidence and the nature of their hypermobility. They were interviewed and examined for the 5 recognized features of hypermobility (laxity of the thumbs, fingers, elbows, spine, and knees). The incidence of hypermobility, according to the number of joints involved, followed an empiric geometric law. Approximately 27% of the individuals had 1 lax joint, whereas only 3% possessed all 5 features. Specific features were present at different frequencies in the 2 sexes. The ratio of the occurrence of 2 features in women compared with the occurrence in men was 2:1. Ratios for the occurrence of 3, 4, and 5 features were 4:1, 8:1, and 3:1, respectively. Thus, hypermobility was a predominantly female characteristic. Joint laxity declined with age, although not to a statistically significant degree. In men, the decline started when they were in their mid-twenties; however, in women, joint laxity continued through the mid-forties.

Adolescent↗

Joint hypermobility in primary mitral valve prolapse patients.

Twenty-seven patients with echocardiographic evidence of primary mitral valve prolapse (MVP) were tested for the presence of joint hypermobility using the criteria of Beighton and Horan. In the examined group, joint hypermobility was found in 14 patients (52%). This occurrence was statistically significant. In patients with simultaneous occurrence of MVP and articular hypermobility we have found a number of pathologic arthrologic manifestations, such as arthralgias, synovitic reactions, distortions, low back pain, and others. In patients with articular hypermobility, there were increased functions of antigen B 35.

Adolescent↗

No neurological involvement for more than 40 years in Klippel-Feil syndrome with severe hypermobility of the upper cervical spine.

We report the case of a 42-year-old woman with Klippel-Feil syndrome, who showed severe hypermobility of the upper cervical spine without neurological involvement for more than 40 years. Radiographs revealed the presence of the odontoid bone and fusion of the atlas, odontoid bone, and occiput. Congenital fusion was present from the axis to C5 as a block vertebra. Lateral flexion-extension radiographs revealed severe hypermobility at the junction between the odontoid bone and the axis. Prophylactic surgical stabilization has been recommended in patients with severe hypermobility, but adjacent disc problems may possibly occur at the unfused levels in the future. We believe that early prophylactic stabilization should not be indicated for Klippel-Feil syndrome without neurological involvement only because of hypermobility.

Adult↗

Relationship of Q angle and joint hypermobility and Q angle values in different positions.

Patellar malalignment is the most important reason for anterior knee pain. Patellar alignment is commonly determined by the measurement of the quadriceps (Q) angle. In this study, our primary aim was to investigate the Q angle difference between healthy individuals with and without joint hypermobility. The other objectives were to compare the Q angle values, which were measured in supine and upright positions, to determine hypermobility frequency among healthy males in a certain population, and to investigate the correlation between the existent skeletal deformities and Beighton score levels. Two hundred fifty-three healthy male college students were examined for hypermobility and skeletal deformities. According to the Beighton scoring system, three groups (n=20) were formed, and Q angle measurements were performed on the 60 individuals in both supine and upright positions. In the comparison of the groups, the mean Q angle values in healthy hypermobile individuals were found to be significantly higher than that of the nonhypermobile ones (p<0.05). No statistical difference was found between Q angle values in supine and upright positions (p>0.05). The frequency of joint hypermobility (Beighton score 4 or more) was found to be 29.25% in this population. No correlation was determined between existent skeletal deformities and Beighton score values (p>0.05). In conclusion, the Q angle evaluation among healthy hypermobile individuals may have a prognostic value for probable knee pathologies that may appear in the future.

Adult↗

[Segmental hypermobility of the spine before and following discectomy].

In 145 patients with open dissectomies (virgin back) preoperative functional x-rays (flexion/extension) were available. 80 patients out of this group could be used for additional postoperative functional x-rays. The mean follow-up was 28 +/- 18 months. The segmental mobility was measured using the translation in the z- and the angular rotation about the x-axis (methods of Morgan/ King and Dupuis et al.). The patients were divided in 3 groups in respect to the level of operation. For each patient 3 segments (L3/4 to L5/S1) were analyzed. Segmental hypermobility was defined with a translation of more than 4 mm respectively a rotation of more than 18 degrees (ROM flexion/extension). In patients operated at the level of L4/5 (n = 62) segmental hypermobility was found in 12.9% at L3/4, in 8.1% at L4/5 and in 17.7% at L5/S1. Patients with a discectomy at the level of L5/S1 (n = 79) showed preoperative hypermobility in 10.1% at L3/4, in 15.1% at L4/5 and in 12.6% at L5/S1. In postoperative x-rays patients with discectomy at L4/5 (n = 34) showed no significant differences after operation, neither in translation nor in rotation. In the L5/S1-discectomy group (n = 45) the mobility for rotation at the level L4/5 increased significantly from 6.7 degrees to 10.2 degrees and in the level L5/S1 from 5.2 degrees to 8.4 degrees. There was no significant difference for the translation in all 3 segments. In both discectomy groups the number of segments with hypermobility didn't change significantly after operation.

Adult↗

The association of soft-tissue rheumatism and hypermobility.

Soft-tissue rheumatism (STR--tendinitis, bursitis, fasciitis and fibromyalgia) accounts for up to 25% of referrals to rheumatologists. The estimated prevalence of generalized hypermobility in the adult population is 5-15%. There have previously been suggestions that hypermobile individuals may be predisposed to soft-tissue trauma and subsequent musculoskeletal pain. This study was designed to examine the mobility status and physical activity level in consecutive rheumatology clinic attendees with a primary diagnosis of STR. Of 82 patients up to age 70 yr with STR, 29 (35%) met criteria for generalized hypermobility. Hypermobile compared to non-hypermobile individuals reported significantly more previous episodes of STR (90% vs 51%, P < 0.01), and more recurrent episodes of STR at a single site (69% vs 38%, P < 0.001). Although we were unable to show any difference in the time spent carrying out physical activity between the two groups, the hypermobile patients were performing significantly more repetitive activities. When specific anatomical sites of STR were analysed, small joints (elbows, hands and feet) currently affected with STR were more likely to show localized hypermobility than if those joints were asymptomatic. These findings suggest that hypermobility may be a factor in the development of STR. Repetitive activity may be a contributing factor towards STR in some hypermobile individuals.

Adolescent↗

Standardized radiologic protocol for the study of common coccygodynia and characteristics of the lesions observed in the sitting position. Clinical elements differentiating luxation, hypermobility, and normal mobility.

STUDY DESIGN: Ninety-one patients with common coccygodynia and 47 control subjects prospectively underwent dynamic radiographic imagery. OBJECTIVES: To standardize the radiologic protocol to better define normal and abnormal mobility of the coccyx, and to study clinical parameters useful in classifying and differentiating the lesions. SUMMARY OF BACKGROUND DATA: In a previous study, comparison of films taken in the sitting and standing positions allowed to individualize two distinct coccygeal lesions: luxation and hypermobility. Measurement technique was precise and reproducible, but the control group was not pain-free. No specific clinical features were described. METHODS: Standing films were made first. Control subjects were healthy volunteers. The following items were recorded: presence of an initial traumatic event, elapsed time before investigation, body mass index, presence of an acute pain when passing from sitting to standing, effect of intradiscal steroid injection, and angle of the coccyx with respect to the seat. RESULTS: Hypermobility was defined as a flexion of more than 25 degrees, luxation by displacement of more than 25% of the coccyx. The base angle is a good predictor of the direction in which the coccyx moves when sitting. In the "luxation" group, a history of initial trauma, a shorter clinical course, pain when standing up, increased body mass index, and satisfactory results with intradiscal injection were found more frequently than in the "normal" group. The "hypermobility" group had characteristics between these two groups. CONCLUSION: Common coccygodynia is associated in 48.4% of patients with a luxation or hypermobility of the coccyx. A distinct clinical presentation was found in individuals with luxation of the coccyx.

Adult↗

Joint hypermobility and genetic collagen disorders: are they related?

The HDCTs constitute a heterogeneous group of rare genetically determined diseases, the best known of which are Ehlers-Danlos and Marfan syndromes and osteogenesis imperfecta. Hypermobility is a feature common to them all, but it is also a feature that is highly prevalent in the population at large. Symptomatic hypermobile subjects (whose symptoms are attributable to their hypermobility) are said to be suffering from the benign joint hypermobility syndrome, which has many features that overlap with the HDCTs. It is not yet known whether there is a variety of hypermobility (symptomatic or otherwise) that is not part of a connective tissue disorder.

Collagen↗