Prolonged standing behaviour in people with joint hypermobility syndrome

Background Joint Hypermobility Syndrome (JHS) is a rare Heritable Disorder of Connective tissue characterised by generalised joint laxity and chronic widespread pain. Joint Hypermobility Syndrome has a large impact on patients’ day to day activities, and many complain of symptoms when standing for prolonged periods. This study investigates whether people with JHS exhibit the same behaviours to deal with the effects of prolonged standing as people with equal hypermobility and no pain, and people with normal flexibility and no pain. Methods Twenty three people with JHS, 22 people with Generalised Joint Hypermobility (GJH), and 22 people with normal flexibility (NF) were asked to stand for a maximum of 15 min across two force-plates. Fidgets were counted and quantified using a cumulative sum algorithm and sway parameters of the quiet standing periods between fidgets were calculated. Results Average standing time for participants with JHS was 7.35 min and none stood for the full 15 min. All participants with GJH and NF completed 15 min of standing. There were no differences in fidgeting behaviour between any groups. There was a difference in anteroposterior sway (p = .029) during the quiet standing periods. Conclusion There is no evidence to suggest people with JHS exhibit different fidgeting behaviour. Increased anteroposterior-sway may suggest a muscle weakness and strengthening muscles around the ankle may reduce postural sway and potentially improve the ability to stand for prolonged periods.

Achenbach’s hand and digital paroxysmal haematomas: a possible association with joint hypermobility syndrome in two sibling cases

A pair of middle-aged sisters is presented who experienced episodes of spontaneous, relatively atraumatic, acute onset, painful sub-cutaneous bleeding into the digits of their hands over a number of preceding years. A literature search revealed they had Achenbach’s syndrome, which is a benign and self-limiting condition that resolves completely within 2–4 days. However, a hitherto unreported association between Achenbach’s syndrome and joint hypermobility with its abnormal collagen is made, along with a tentative causality hypothesis that perhaps in the presence of atypical connective tissue, the architecture and integrity of the peripheral capillary bed of the hand could be adversely affected and therefore predispose towards paroxysmal palm and/or finger bleeds.

Association between Postural Orthostatic Tachycardia Syndrome and Joint Hypermobility

Joint hypermobility syndrome refers to increased joint flexibility beyond the normal range of motion. This syndrome has a benign form known as Ehlers-Danlos syndrome type 3. This is a disorder in which hypermobility is accompanied by clinical manifestations in the absence of any systemic disease. A clinical finding associated with this condition is postural orthostatic tachycardia syndrome. The following is a rare case of joint hypermobility syndrome and postural orthostatic tachycardia syndrome. The relevance of this case report lies in the impact that this disease had on the patient’s quality of life and the limitation in the performance of activities of daily living.

Optimization of Therapy in Patients with Lower Back Pain Depending on the Presence of Benign Joint Hypermobility Syndrome

Lower back pain is pain that is localized in the lower back, buttocks, and sacroiliac area. Lower back pain is the most common reason for limiting physical activity in patients under 45 years of age. If we take into account both acute and chronic pain. Lower back pain is the most common complaint after colds and minor injuries. At the same time, even a thorough examination may not always reveal the cause of lower back pain. There is evidence of more frequent development of pain in the lower back on the background of benign joint hypermobility syndrome. Lower back pain in patients with benign joint hypermobility syndrome is usually assessed on the basis of visual analog scale data and is quite subjective. To assess the severity of changes in the lumbar spine, an ultrasound method can be used, one of the options of which is elastometry, which allows to determine the stiffness of the studied tissues, in particular to assess muscle tone. Muscle relaxants are commonly used for therapy, but it is usually not possible to assess their effect on actual muscle relaxation. The purpose of our study was to determine the effectiveness of using muscle relaxants in patients with lower back pain and benign joint hypermobility syndrome by performing elastometry of the iliopsoas muscles in these patients. Material and methods. To achieve this goal, we examined 65 patients with lower back pain aged 45.62±2.24 years, among them there were 32 women and 33 men. Patients with impaired limb sensitivity, muscle weakness, pelvic dysfunction, severe pain at night, fever and unmotivated weight loss, history of cancer, etc. were excluded from the study. To participate in the study, all patients underwent X-ray and ultrasound examination of the hip joints, in the presence of signs of osteoarthritis, patients were excluded from the study. Among the patients examined by the Bayton criteria, 26 patients were diagnosed with benign joint hypermobility syndrome, among them were 19 women and 7 men. We used shear wave elastometry to determine the stiffness of the distal lumbosacral muscle. A visual analog scale of pain with a gradation from 1 to 10 was used to determine the severity of the pain syndrome. After the diagnosis of lower back pain, patients were prescribed anti-inflammatory therapy in the form of 100 mg of diclofenac per day and tizanidine 4 mg 3 times a day for two weeks. After two weeks, the examination of patients was repeated. Results and discussion. In patients with lower back pain, the initial values of iliopsoas muscle stiffness were 11.85±1.37 kPa on the right and 12.1±1.45 kPa on the left, in patients with signs of benign joint hypermobility syndrome and lower back pain, the stiffness was iliopsoas muscle were 12.64±1.42 kPa on the right and 12.49±1.67 kPa on the left. No statistically significant difference was found between the studied groups. The severity of pain in the group of patients with lower back pain without benign joint hypermobility syndrome was 7.98±1.45, in patients with lower back pain and signs of benign joint hypermobility syndrome was 7.67±1.33 After 2 weeks of therapy in patients with lower back pain without signs of benign joint hypermobility syndrome, the lumbar-iliac muscle stiffness was 7.69±1.23 kPa on the right and 7.98±1.38 kPa on the left, the difference with the initial values was significant (p <0.05). In patients with signs of benign joint hypermobility syndrome and lower back pain after treatment, the lumbar-lumbar muscle stiffness was 10.29±1.97 kPa on the right and 10.89±1.75 kPa on the left. The difference was significant (p <0.05) both with the initial indicators and with the indicators of the group of patients with lower back pain without signs of benign joint hypermobility syndrome. The severity of pain on the visual analog scale in the group of patients with lower back pain without benign joint hypermobility syndrome after therapy was 3.49±1.98, in patients with lower back pain and signs of benign joint hypermobility syndrome was 5.21±1.43. The difference was significant (p <0.05) both with the initial indicators and with the indicators of the group of patients with lower back pain without signs of benign joint hypermobility syndrome and had a clear correlation with the indicators of lumbar-iliac muscle stiffness according to shear wave elastometry (r = 0.63, p = 0.032) Conclusion. Thus, the use of tizanidine in therapy can reduce the stiffness of the lumbosacral muscles of the back and reduce the intensity of pain in patients with lower back pain. At the same time, the patient's presence of benign joint hypermobility syndrome significantly reduced the degree of muscle relaxation and provoked greater resistance to pain therapy. The use of shear wave elastometry allows controling the quality of treatment in the dynamics. At the same time, there is a need to study the possibility of using this method of research in other spastic changes in the muscular system, which should be a prospect for further research

Adaptation of balance reactions following forward perturbations in people with joint hypermobility syndrome

Background Joint Hypermobility Syndrome (JHS) is a Heritable Disorder of Connective tissue characterised by joint laxity and chronic widespread arthralgia. People with JHS exhibit a range of other symptoms including balance problems. To explore balance further, the objective of this study is to compare responses to forward perturbations between three groups; people who are hypermobile with (JHS) and without symptoms and people with normal flexibility. Methods Twenty-one participants with JHS, 23 participants with Generalised Joint Hypermobility (GJH) and 22 participants who have normal flexibility (NF) stood on a platform that performed 6 sequential, sudden forward perturbations (the platform moved to the anterior to the participant). Electromyographic outcomes (EMG) and kinematics for the lower limbs were recorded using a Vicon motion capture system. Within and between group comparisons were made using Kruskal Wallis tests. Results There were no significant differences between groups in muscle onset latency. At the 1st perturbation the group with JHS had significantly longer time-to-peak amplitude than the NF group in tibialis anterior, vastus medialis, rectus femoris, vastus lateralis, and than the GJH group in the gluteus medius. The JHS group showed significantly higher cumulative joint angle (CA) than the NF group in the hip and knee at the 1st and 2nd and 6th perturbation, and in the ankle at the 2nd perturbation. Participants with JHS had significantly higher CA than the GJH group at the in the hip and knee in the 1st and 2nd perturbation. There were no significant differences in TTR. Conclusions The JHS group were able to normalise the timing of their muscular response in relation to control groups. They were less able to normalise joint CA, which may be indicative of impaired balance control and strength, resulting in reduced stability.