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Presentation and physical therapy management of upper cervical instability in patients with symptomatic generalized joint hypermobility: International expert consensus recommendations.

ABSTRACT: Experts in symptomatic generalized joint hypermobility (S-GJH) agree that upper cervical instability (UCI) needs to be better recognized in S-GJH, which commonly presents in the clinic as generalized hypermobility spectrum disorder and hypermobile Ehlers-Danlos syndrome. While mild UCI may be common, it can still be impactful; though considerably less common, severe UCI can potentially be debilitating. UCI includes both atlanto-occipital and atlantoaxial instability. In the absence of research or published literature describing validated tests or prediction rules, it is not clear what signs and symptoms are most important for diagnosis of UCI. Similarly, healthcare providers lack agreed-upon ways to screen and classify different types or severity of UCI and how to manage UCI in this population. Consequently, recognition and management of UCI in this population has likely been inconsistent and not based on the knowledge and skills of the most experienced clinicians. The current work represents efforts of an international team of physical/physiotherapy clinicians and a S-GJH expert rheumatologist to develop expert consensus recommendations for screening, assessing, and managing patients with UCI associated with S-GJH. Hopefully these recommendations can improve overall recognition and care for this population by combining expertise from physical/physiotherapy clinicians and researchers spanning three continents. These recommendations may also stimulate more research into recognition and conservative care for this complex condition.

SUBMITTER: Russek LN

PROVIDER: S-EPMC9893781 | biostudies-literature | 2022

REPOSITORIES: biostudies-literature

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Presentation and physical therapy management of upper cervical instability in patients with symptomatic generalized joint hypermobility: International expert consensus recommendations.

Russek Leslie N LN Block Nancy P NP Byrne Elaine E Chalela Susan S Chan Cliffton C Comerford Mark M Frost Nicole N Hennessey Sharon S McCarthy Ann A Nicholson Leslie L LL Parry Jason J Simmonds Jane J Stott Patricia J PJ Thomas Lucy L Treleaven Julia J Wagner Wendy W Hakim Alan A

Frontiers in medicine 20230118

Experts in symptomatic generalized joint hypermobility (S-GJH) agree that upper cervical instability (UCI) needs to be better recognized in S-GJH, which commonly presents in the clinic as generalized hypermobility spectrum disorder and hypermobile Ehlers-Danlos syndrome. While mild UCI may be common, it can still be impactful; though considerably less common, severe UCI can potentially be debilitating. UCI includes both atlanto-occipital and atlantoaxial instability. In the absence of research o ...[more]

PMID: 36743665

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Project description:Background Balance training is the first choice of treatment for chronic ankle instability (CAI). However, there is a lack of research on the effects of balance training in CAI with generalized joint hypermobility (GJH). This study is to compare the outcomes of balance training in CAI patients with and without GJH. Methods Forty CAI patients were assigned into the GJH group (Beighton ≥ 4, 20) and non-GJH group (Beighton < 4, 20) and they received same 3-month supervised balance training. Repeated measure ANOVA and independent t test were used to analyze self-reported questionnaires (Foot and ankle ability measure, FAAM), the number of patients experiencing ankle sprain, isokinetic muscle strength and postural control tests (Star excursion balance test, SEBT and Balance errors system, BES) before training, post-training immediately, and post-training 3 months, respectively. Results At baseline, no differences were found between groups with except for GJH group having poorer SEBT in the posteromedial direction (83.6 ± 10.1 vs 92.8 ± 12.3, %) and in the posterolateral direction (84.7 ± 11.7 vs 95.7 ± 8.7, %). Following the balance training, GJH group demonstrated lower re-sprain ratio (immediately after training, 11.1% vs 23.5%, 3 month after training, 16.7% vs 29.4%) than non-GJH group, as well as greater FAAM-S score, plantarflexion strength and dorsiflexion strength at post-training immediately and 3 months, and both groups improved similarly in the FAAM-A score, muscle strength and balance control (SEBT in the posterior-lateral and posterior-medial directions, and BES scores) compared with baseline. Conclusions CAI patients with GJH gained equally even better postural stability and muscle strength after the balance training than the non-GJH patients. Balance training could still be an effective treatment for CAI patients with GJH before considering surgery. Trial registration ChiCTR1900023999, June 21st, 2019. Supplementary Information The online version contains supplementary material available at 10.1186/s12891-023-06179-2.

Project description:PurposeTo explore the relationship between generalized joint hypermobility and hip labrum width.MethodsA retrospective review was performed of a single-surgeon database containing patients who underwent hip arthroscopy between 2014 and 2017. Patients were assessed for generalized laxity via Beighton Test Scoring (BTS), which tests for hyperextension of the fifth metacarpophalangeal joint, thumb apposition, elbow hyperextension, knee hyperextension, and trunk flexion on a 9-point scale. Patients were stratified into a "high BTS cohort" with a BTS ≥4, and a control cohort with BTS <4. Magnetic resonance imaging measurements of labral width for each patient were conducted by 2 blinded, musculoskeletal fellowship-trained radiologists at standardized "clockface" locations using a previously validated technique. Statistical analyses used to determine associations between BTS and labral width included Mann-Whitney U and Fisher exact testing as well as linear regression.ResultsThirty-four patients met inclusion criteria (17 cases, 17 controls). Both groups were composed exclusively of female patients. There was no significant difference between cases or controls in terms of age (33.3 ± 10.4 years vs 35.2 ± 8.3 years, P = .57) or body mass index (26.1 ± 9.3 vs 23.6 ± 3.4, P = .36). The high Beighton score cohort had significantly thinner labrae at the indirect rectus (5.35 ± 1.2 mm vs 7.1 ± 1.1 mm, P < .001) and anterosuperior position (5.53 ± 1.4 mm vs 7.27 ± 1.6 mm, P = .003). There was no statistical difference between the high Beighton score cohort and controls at the psoas U position (6.47 ± 1.6 mm vs 7.43 ± 1.7 mm, P = .112). Linear regression analysis demonstrated Beighton score was significantly negatively associated with labrum width at the indirect rectus position (R2 = 0.33, P < .001) and the anterosuperior position (R2 = 0.25, P = .004).ConclusionsPatients with a BTS ≥4 were found to have significantly thinner labra than those with a BTS of <4.Level of evidenceIII, retrospective comparative trial.

Project description:BackgroundFamilial dysautonomia (Riley-Day syndrome, hereditary sensory autonomic neuropathy type-III) is a rare genetic disease caused by impaired development of sensory and afferent autonomic nerves. As a consequence, patients develop neurogenic dysphagia with frequent aspiration, chronic lung disease, and chemoreflex failure leading to severe sleep disordered breathing. The purpose of these guidelines is to provide recommendations for the diagnosis and treatment of respiratory disorders in familial dysautonomia.MethodsWe performed a systematic review to summarize the evidence related to our questions. When evidence was not sufficient, we used data from the New York University Familial Dysautonomia Patient Registry, a database containing ongoing prospective comprehensive clinical data from 670 cases. The evidence was summarized and discussed by a multidisciplinary panel of experts. Evidence-based and expert recommendations were then formulated, written, and graded using the Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) system.ResultsRecommendations were formulated for or against specific diagnostic tests and clinical interventions. Diagnostic tests reviewed included radiological evaluation, dysphagia evaluation, gastroesophageal evaluation, bronchoscopy and bronchoalveolar lavage, pulmonary function tests, laryngoscopy and polysomnography. Clinical interventions and therapies reviewed included prevention and management of aspiration, airway mucus clearance and chest physical therapy, viral respiratory infections, precautions during high altitude or air-flight travel, non-invasive ventilation during sleep, antibiotic therapy, steroid therapy, oxygen therapy, gastrostomy tube placement, Nissen fundoplication surgery, scoliosis surgery, tracheostomy and lung lobectomy.ConclusionsExpert recommendations for the diagnosis and management of respiratory disease in patients with familial dysautonomia are provided. Frequent reassessment and updating will be needed.

Dataset Information

Presentation and physical therapy management of upper cervical instability in patients with symptomatic generalized joint hypermobility: International expert consensus recommendations.

Publications

Presentation and physical therapy management of upper cervical instability in patients with symptomatic generalized joint hypermobility: International expert consensus recommendations.

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