The evidence-based rationale for physical therapy treatment of children, adolescents, and adults diagnosed with joint hypermobility syndrome/hypermobile Ehlers Danlos syndrome.

New insights into the phenotype of Joint Hypermobility Syndrome (JHS) and Ehlers-Danlos Syndrome-hypermobile type (hEDS) have raised many issues in relation to classification, diagnosis, assessment, and treatment. Within the multidisciplinary team, physical therapy plays a central role in management of individuals with hypermobility related disorders. However, many physical therapists are not familiar with the diagnostic criteria, prevalence, common clinical presentation, and management. This guideline aims to provide practitioners with the state of the art regarding the assessment and management of children, adolescents, and adults with JHS/hEDS. Due to the complexity of the symptoms in the profile of JHS/hEDS, the International Classification of Functioning, Disability and Health (ICF) is adopted as a central framework whereby the umbrella term of disability is used to encompass functions, activities and participation, as well as environmental and personal factors. The current evidence-based literature regarding the management of JHS/hEDS is limited in size and quality and there is insufficient research exploring the clinical outcomes of a number of interventions. Multicenter randomized controlled trials are warranted to assess the clinical and cost-effectiveness of interventions for children and adults. Until further multicenter trials are conducted, clinical decision-making should be based on theoretical and the current limited research evidence. For all individuals diagnosed with JHS/hEDS, international consensus and combined efforts to identify risk profiles would create a better understanding of the pathological mechanisms and the potential for optimizing health care for affected individuals. © 2017 Wiley Periodicals, Inc.

Can screening instruments accurately determine poor outcome risk in adults with recent onset low back pain? A systematic review and meta-analysis.

BACKGROUND: Delivering efficient and effective healthcare is crucial for a condition as burdensome as low back pain (LBP). Stratified care strategies may be worthwhile, but rely on early and accurate patient screening using a valid and reliable instrument. The purpose of this study was to evaluate the performance of LBP screening instruments for determining risk of poor outcome in adults with LBP of less than 3 months duration. METHODS: Medline, Embase, CINAHL, PsycINFO, PEDro, Web of Science, SciVerse SCOPUS, and Cochrane Central Register of Controlled Trials were searched from June 2014 to March 2016. Prospective cohort studies involving patients with acute and subacute LBP were included. Studies administered a prognostic screening instrument at inception and reported outcomes at least 12 weeks after screening. Two independent reviewers extracted relevant data using a standardised spreadsheet. We defined poor outcome for pain to be ≥ 3 on an 11-point numeric rating scale and poor outcome for disability to be scores of ≥ 30% disabled (on the study authors' chosen disability outcome measure). RESULTS: We identified 18 eligible studies investigating seven instruments. Five studies investigated the STarT Back Tool: performance for discriminating pain outcomes at follow-up was 'non-informative' (pooled AUC = 0.59 (0.55-0.63), n = 1153) and 'acceptable' for discriminating disability outcomes (pooled AUC = 0.74 (0.66-0.82), n = 821). Seven studies investigated the Orebro Musculoskeletal Pain Screening Questionnaire: performance was 'poor' for discriminating pain outcomes (pooled AUC = 0.69 (0.62-0.76), n = 360), 'acceptable' for disability outcomes (pooled AUC = 0.75 (0.69-0.82), n = 512), and 'excellent' for absenteeism outcomes (pooled AUC = 0.83 (0.75-0.90), n = 243). Two studies investigated the Vermont Disability Prediction Questionnaire and four further instruments were investigated in single studies only. CONCLUSIONS: LBP screening instruments administered in primary care perform poorly at assigning higher risk scores to individuals who develop chronic pain than to those who do not. Risks of a poor disability outcome and prolonged absenteeism are likely to be estimated with greater accuracy. It is important that clinicians who use screening tools to obtain prognostic information consider the potential for misclassification of patient risk and its consequences for care decisions based on screening. However, it needs to be acknowledged that the outcomes on which we evaluated these screening instruments in some cases had a different threshold, outcome, and time period than those they were designed to predict. SYSTEMATIC REVIEW REGISTRATION: PROSPERO international prospective register of systematic reviews registration number CRD42015015778 .

Classical Conditioning Fails to Elicit Allodynia in an Experimental Study with Healthy Humans.

OBJECTIVE: Associative learning has been proposed as a mechanism behind the persistence of pain after tissue healing. The simultaneous occurrence of nociceptive and non-nociceptive input during acute injury mimics the pairings thought to drive classical conditioning effects. However, empirical evidence for classically conditioned allodynia is lacking. We aimed to manipulate pain thresholds with a classical conditioning procedure that used non-nociceptive somatosensory stimuli as conditioned stimuli (CS) and nociceptive stimuli as unconditioned stimuli. We also explored the influence of gender, depression, anxiety, negative affect, and pain catastrophizing on the main manipulation. DESIGN: Thirty-four healthy humans participated in a differential classical conditioning procedure that used vibrotactile stimulations at two different locations as CS. In an acquisition phase, CS+ was paired with painful thermal stimulation, and CS- with nonpainful thermal stimulation. Heat pain threshold was assessed during paired heat-CS trials before and after acquisition. A 2 (time: 1 and 2) x 2 (condition: CS+ and CS-) repeated-measures analysis of variance compared pain thresholds before and after acquisition. Exploratory analyses explored the influence of gender, depression, anxiety, negative affect, and pain catastrophizing. Postexperiment questions investigated participants' awareness of the contingencies employed. RESULTS: The classical conditioning procedure did not alter pain thresholds. Exploratory analyses did not reveal any influence of individual differences. Thirty of the 34 participants were unaware of the contingencies between stimuli. CONCLUSIONS: The results of this study provide no evidence that allodynia can be induced in healthy humans using a classical conditioning procedure with simultaneous timing.

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

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.

Are You Listening? Facilitation of the Auditory Blink Response in People with Fibromyalgia.

The purpose of the current study was to determine whether auditory prepulse inhibition (PPI) and/or prepulse facilitation (PPF) were altered in people with fibromyalgia (FM) when compared with controls. Eyeblink responses were recorded from 29 females with FM and 27 controls, while they listened to 3 blocks of auditory stimuli that delivered pulses with either PPI or PPF. Using a linear mixed model, our main findings were that there was a GROUP*CONDITION interaction (F4, 1084 = 4.01, P= .0031) indicating that the difference in amplitude between FM group and control group changed depending on the condition (PPI or PPF). Post hoc tests revealed no differences between the groups in response to PPI. The FM group showed a greater reactivity of response to the PPF conditioned stimulus than the control group did (t(39.7) = 2.03, P= .0494). Augmentation of PPF, as demonstrated by the FM group is thought to be linked to alterations in information processing mediated by an autonomically driven general orienting process. Activities that decrease autonomic drive or rebalance autonomic and parasympathetic tone such as vagal stimulation might be pursued as effective interventions for people with FM. PERSPECTIVE: This article presents evidence of preservation of neural circuitry that underpins response suppression and evidence of neural circuit disturbance mediated by autonomic drive-in people with FM. These results are important because intact circuitry underpins the effectiveness of therapies and may be harnessed, and rebalancing autonomic drive may be indicated.

Recognizing and Effectively Managing Hypermobility-Related Conditions.

Hypermobility spectrum disorder (HSD) and hypermobile Ehlers-Danlos syndrome (hEDS) can cause widespread or chronic pain, fatigue, and proprioceptive and coordination deficits resulting in functional restrictions. These conditions are common and often unrecognized, and patients are likely to present in physical therapy for musculoskeletal injuries, pain, or coordination deficits. Although physical therapy is considered central to managing these conditions, many patients report pain and iatrogenic injuries due to inappropriate interventions. The diagnostic classification for these conditions was revised in 2017 to supersede previous diagnostic categories of Joint Hypermobility Syndrome and Ehlers-Danlos Syndrome-hypermobility type/type III. It is now known that these conditions affect multiple body systems and not just joints and that patients require a holistic approach. This Perspective article will describe the 2017 diagnostic classification system, clinical presentation, examination, evaluation, and management of patients with HSD/hEDS. Both adult and pediatric cases are presented to illustrate the patient management concepts discussed. This knowledge can lead to more effective management of this patient population.

The disappearing hand: vestibular stimulation does not improve hand localisation.

BACKGROUND: Bodily self-consciousness depends on the coherent integration of sensory information. In addition to visual and somatosensory information processing, vestibular contributions have been proposed and investigated. Vestibular information seems especially important for self-location, but remains difficult to study. METHODS: This randomised controlled experiment used the MIRAGE multisensory illusion box to induce a conflict between the visually- and proprioceptively-encoded position of one hand. Over time, the perceived location of the hand slowly shifts, due to the fact that proprioceptive input is progressively weighted more heavily than the visual input. We hypothesised that left cold caloric vestibular stimulation (CVS) augments this shift in hand localisation. RESULTS: The results from 24 healthy participants do not support our hypothesis: CVS had no effect on the estimations with which the perceived position of the hand shifted from the visually- to the proprioceptively-encoded position. Participants were more likely to report that their hand was 'no longer there' after CVS. Taken together, neither the physical nor the subjective data provide evidence for vestibular enhanced self-location.

Was That Painful or Nonpainful? The Sensation and Pain Rating Scale Performs Well in the Experimental Context.

In experiments on pain, participants are frequently exposed to nonpainful and painful stimuli; however, the conventional pain-rating scales lack a nonpainful range and a clear point of transition from nonpainful to painful events. The Sensation and Pain Rating Scale (SPARS) assesses the full stimulus intensity range, extending from no sensation (rating: -50) to worst pain imaginable (rating: +50), and it explicitly identifies pain threshold (rating: 0). Here, we tested the SPARS in 2 experiments by using laser heat stimuli to establish its stimulus-response characteristics (Experiment 1, N = 19, 13 stimulus intensities applied 26 times each across a 1-4 J range), and compared it to 0 to 100 scales that assess nonpainful (0: no sensation, 100: pain) and painful (0: no pain, 100: worst pain imaginable) events (Experiment 2, N = 7, 9 stimulus intensities applied 36 times each across a 1.5-4.5 J range). Despite high inter- and intraindividual variations, we found a reasonably consistent curvilinear stimulus-response relationship (the curve flattens around pain threshold), with stable response characteristics across the range of the scale. The SPARS ratings transformed to a 0 to 100 range tended to be lower than the 0 to 100 pain rating scale in the noxious stimulus intensity range and greater than the 0 to 100 nonpainful sensation scale in the non-noxious stimulus range, likely reflecting differences in scale dimensionality. The SPARS overcomes limitations in scale range inherent to conventional pain rating scales. As such, it is well suited to experimental studies that must quantify a wider range of perceptual intensity or distinguish between painful and nonpainful events. PERSPECTIVE: This article presents the stimulus-response characteristics of a new scale designed to allow participants to rate a range of nonpainful and painful stimuli. The scale could be useful for research that involves exposing participants to a range of stimulation intensities or requires a clear distinction between nonpainful and painful events.

Humor Use Moderates the Relation of Stressful Life Events With Psychological Distress.

Three studies examined humor and adjustment to stressful events. In Study 1, patients with fibromyalgia syndrome ( N = 22) reported on mental and physical adjustment, social interaction, and reappraisal of their illness. Dispositional humor was associated with reduced distress and fewer physical symptoms. Study 2 ( N = 109) examined undergraduates' reports of stressful events. Dispositional, self-enhancing, affiliative, and self-defeating humor showed direct effects on distress, which were mediated by social interaction and reappraisal. Moreover, dispositional and aggressive humor showed stress-buffering effects. Study 3 ( N = 105) examined undergraduates' adjustment to the September 11, 2001, attacks at 1 and 3 months postattack. At T1, affiliative humor showed a stress-buffering effect on distress. Social interaction mediated the relation of self-enhancing humor with reduced T1 distress, and mediated relations of aggressive and self-defeating humor with greater distress. Relations of T1 dispositional and self-defeating humor to changes in T2 distress were mediated by reappraisal.

Prevalence, injury rate and, symptom frequency in generalized joint laxity and joint hypermobility syndrome in a "healthy" college population.

Generalized joint hypermobility (GJH) and joint hypermobility syndrome (JHS) are gaining increased attention as potential sources of pain and injury. The aims of this study were to evaluate prevalence of GJH and JHS and to determine whether musculoskeletal injuries and symptoms commonly attributed to GJH and JHS were more common within a "healthy" college student population. The study involved a convenience sample of 267 college and graduate students, aged 17-26. GJH was assessed using the Beighton score with a cutoff of 5/9, while JHS was assessed using the Brighton criteria. Injury history and symptoms were assessed by recall. Prevalence of GJH was 26.2 % overall (females 36.7 %, males 13.7 %). Prevalence of JHS was 19.5 % overall (females 24.5 %, males 13.7 %). Injury rates were not significantly different for individuals who had GJH vs. those who did not have GJH. Individuals with JHS were significantly more likely to have had sprains, back pain, and stress fractures. Symptoms were no different between those with GJH and those who did not have GJH. However, individuals with JHS were significantly more likely to report clumsiness, easy bruising, and balance problems than those who did not have JHS. GJH and JHS were relatively common in this healthy college student population; GJH was not associated with increased incidence of injury or symptoms commonly attributed to JHS, but JHS was associated with increased incidence of some injuries and symptoms.

Pain by Association? Experimental Modulation of Human Pain Thresholds Using Classical Conditioning.

UNLABELLED: A classical conditioning framework is often used for clinical reasoning about pain that persists after tissue healing. However, experimental studies demonstrating classically conditioned pain in humans are lacking. The current study tested whether non-nociceptive somatosensory stimuli can come to modulate pain thresholds after being paired with painful nociceptive stimuli in healthy humans. We used a differential simultaneous conditioning paradigm in which one nonpainful vibrotactile conditioned stimulus (CS(+)) was simultaneously paired with an unconditioned painful laser stimulus, and another vibrotactile stimulus (CS(-)) was paired with a nonpainful laser stimulus. After acquisition, at-pain-threshold laser stimuli were delivered simultaneously with a CS(+) or CS(-) vibrotactile stimulus. The primary outcome was the percentage of at-threshold laser stimuli that were reported as painful. The results were as expected: after conditioning, at-threshold laser trials paired with the CS(+) were reported as painful more often, as more intense, and as more unpleasant than those paired with the CS(-). This study provides new evidence that pain thresholds can be modulated via classical conditioning, even when the stimulus used to test the threshold cannot be anticipated. As such, it lays a critical foundation for further investigations of classical conditioning as a possible driver of persistent pain. PERSPECTIVE: This study provides new evidence that human pain thresholds can be influenced by non-nociceptive somatosensory stimuli, via a classical conditioning effect. As such, it lays a critical foundation for further investigations of classical conditioning as a possible driver of persistent pain.

United States Physical Therapists' Knowledge About Joint Hypermobility Syndrome Compared with Fibromyalgia and Rheumatoid Arthritis.

BACKGROUND: Joint hypermobility syndrome (JHS) is one of the most common inherited connective tissue disorders. It causes significant pain and disability for all age groups, ranging from developmental delay among children to widespread chronic pain in adults. Experts in JHS assert that the condition is under-recognized and poorly managed. PURPOSE: The aim of this study was to assess US physical therapists' knowledge about JHS compared with other causes of widespread pain and activity limitations: fibromyalgia, juvenile rheumatoid arthritis and adult rheumatoid arthritis. METHODS: Cross-sectional, Internet-based survey of randomly selected members of the American Physical Therapy Association and descriptive statistics were used to explore physical therapists' knowledge about JHS, fibromyalgia, juvenile rheumatoid arthritis and adult rheumatoid arthritis, and chi square was used to compare knowledge about the different conditions. RESULTS: The response rate was 15.5% (496). Although 36% recognized the Beighton Scale for assessing joint hypermobility, only 26.8% of respondents were familiar with the Brighton Criteria for diagnosing JHS. Few respondents (11-19%) realized that JHS has extra-articular features such as anxiety disorder, fatigue, headache, delayed motor development, easy bruising and sleep disturbance. Physical therapists working in environments most likely to see patients with JHS underestimated the likely prevalence in their patient population. CONCLUSIONS: The results suggest that many physical therapists in the United States are not familiar with the diagnostic criteria, prevalence or common clinical presentation of JHS.

A cross-sectional survey assessing sources of movement-related fear among people with fibromyalgia syndrome.

Fear of movement may contribute to functional limitations and loss of well-being among individuals with fibromyalgia (FM). The objectives of this study were to assess factors contributing to movement-related fear and to explore relationships among these factors, function and wellness, in a widespread population of people with FM. This was an internet survey of individuals with FM. Respondents completed a battery of surveys including the Fibromyalgia Impact Questionnaire--Revised (FIQR), Tampa Scale of Kinesiophobia (TSK), Activities-Specific Balance Confidence Scale (ABC), Primary Care Posttraumatic Stress Disorder screen (PC-PTSD), Vertigo Symptom Scale (VSS-SF), a joint hypermobility syndrome screen (JHS), and screening questions related to obsessive-compulsive personality disorder (OCPD), physical activity, work status, and demographics. Analysis included descriptive statistics, Pearson product-moment correlations, and linear regression. Over a 2-year period, 1,125 people (97.6 % female) completed the survey battery. Kinesiophobia was present in 72.9 % of the respondents, balance confidence was compromised in 74.8 %, PTSD likely in 60.4 %, joint hypermobility syndrome likely in 46.6 %, and OCPD tendencies in 26.8 %. The total FIQR and FIQR perceived function subscores were highly correlated (p < 0.0005, r > 0.4) with pain, kinesiophobia, balance confidence, and vertigo. Reported activity level had poor correlation (r < 0.25) with all measured variables. Pain, ABC, VSS, and TSK predicted FIQR and FIQR-pf, explaining 65 and 48 % of the variance, respectively. Kinesiophobia, balance complaints, vertigo, PTSD, and joint hypermobility were common in this population of people with FM. Sources of movement-related fear correlated to overall wellness and perceived function as measured by the FIQR and FIQR-pf.

Pilot study assessing balance in women with fibromyalgia syndrome.

The purpose of current study was to assess postural control and balance self-efficacy in people with fibro-myalgia syndrome [FMS]. Thirty-two females with FMS completed the Activity-specific Balance Confidence Scale, Berg Balance test, NeuroCom Balance Master sensory-organization test, and limits of stability. There was a high prevalence of reported falls and a low mean score on the Activity-specific Balance Confidence Scale. A significant number of subjects scored below the population norm fifth percentile score on the sensory-organization test composite, visual, and vestibular sections. Preliminary evidence suggests that women with FMS may present with deficits in postural control, sensory organization, and balance self-efficacy.