Exploring joint hypermobility syndrome, developmental coordination disorder and pain

INTRODUCTION Floppy, clumsy, hypermobile children are increasingly referred to occupational and physical therapy under the label of dyspraxia. Motor impairments associated with the umbrella diagnosis of developmental coordination disorder (DCD) have been reported as persisting into adolescence and adulthood and subsequently affecting functional abilities (Cousins and Smyth 2003). Within this heterogeneous condition the underlying mechanisms causing the motor difficulties remains unclear. Ayers (1985) hypothesised that some individuals might have somatosensory processing issues contributing to their poor motor planning and coordination difficulties. Similarities in functional difficulties have been noted in children with a diagnosis of DCD and joint hypermobility syndrome (JHS) (Kirby and Davies 2006). There is limited understanding of the relationship between the two conditions. JHS is a multisystemic inherited connective tissue disorder, in which hypermobile joints, pain, clumsiness, poor proprioception and dislocations are familiar features (Grahame and Hakim 2006; Adib et al 2005). It has been suggested that adults with JHS show poor movement patterns which contribute to biomechanical dysfunction and continuing pain (Clark et al 2009). Pain and disability reported in adults with JHS often leads to anxiety, depression, work incapacity and social isolation (Grahame and Hakim 2006). The purpose of this study was to explore the association between adults with JHS and DCD and long term pain. METHODOLOGY/ METHODS A mixed methods design influenced by a pragmatic paradigm was utilised. Subjects: 90 patients with JHS (18-65 years) recruited from a hypermobility clinic were compared, using a questionnaire, with 113 healthy volunteers (18-65 years) with no pain recruited from a university. Analysis: Quantitative data were described and examined by regression, odds ratios were calculated. Qualitative data was analysed thematically FINDINGS The percentage of subjects who reported DCD in patients with JHS and healthy volunteers were 56% and 19% respectively. A significant association between patients with JHS and DCD was noted, chi square = 30.11, p < .001. Patients with JHS were 6 times [95% CI 2.9 – 10.3] more likely to report DCD than healthy volunteers. Pain was a significant feature with an average of 9.8 pain sites reported (out of a total of 17). Open ended questions revealed many patients recalling pain starting in early childhood and adolescence. DISCUSSION These results suggest a significant association between patients with JHS and DCD and the reporting of long term pain. Early recognition and understanding of the needs of children with DCD who present with somatosensory impairment, pain modulation and JHS is therefore essential. Sensory integration therapy as part of a comprehensive early intervention program has the potential to mitigate long term problems. A multidisciplinary approach which involves health professionals and teachers is also recommended. CONCLUSION This research may be considered an early step in the identification of an association of DCD and JHS. Further studies are required to explore somatosensory processing issues experienced by those with DCD and JHS as this might be an important underlying mechanism

Self-similar spherically symmetric cosmological models with a perfect fluid and a scalar field

Self-similar, spherically symmetric cosmological models with a perfect fluid and a scalar field with an exponential potential are investigated. New variables are defined which lead to a compact state space, and dynamical systems methods are utilised to analyse the models. Due to the existence of monotone functions global dynamical results can be deduced. In particular, all of the future and past attractors for these models are obtained and the global results are discussed. The essential physical results are that initially expanding models always evolve away from a massless scalar field model with an initial singularity and, depending on the parameters of the models, either recollapse to a second singularity or expand forever towards a flat power-law inflationary model. The special cases in which there is no barotropic fluid and in which the scalar field is massless are considered in more detail in order to illustrate the asymptotic results. Some phase portraits are presented and the intermediate dynamics and hence the physical properties of the models are discussed.Comment: 31 pages, 4 figure

An Exploration of Neurophysiological Symptoms in Patients with Joint Hypermobility Syndrome and their Impact on Quality of Life.

Purpose: The purpose of this study was to explore the prevalence of neurophysiological symptoms in patients with Joint Hypermobility Syndrome (JHS) and their impact on quality of life. Relevance: Clinical experience suggests patients with JHS suffer from neurophysiological symptoms contributing to skill and health impairments affecting quality of life. MethodsA sample of 90 JHS-patients (mean age 34.7 ± 9.9 years), diagnosed according to the Brighton Criteria were compared with 113 healthy volunteers (mean age 35.7 ± 12.9) with no musculoskeletal pain. Neurophysiological symptoms were collected in a self report questionnaire. The Functional Difficulties Questionnaire was used for the assessment of developmental coordination disorder (DCD). A pain chart was employed to collect data relating to musculoskeletal pain. The SF-12 medical outcomes questionnaire was used for assessing quality of life. Analysis: Chi-square was employed to compare group proportions. Continuous numerical data comparisons were analysed using independent sample t-tests. Regression analysis was employed to analyse multiple variables. Results: Patients with JHS were significantly more likely to report the following than healthy volunteers; autonomic symptoms (70%, 12%); gastrointestinal symptoms (71%, 9%); DCD (56%, 19%) and chronic fatigue syndrome (31%, 1%). The mean number of pain sites reported for patients with JHS were 9.83 ± 4.18. Patients with JHS reported significantly lower physical component summary scores (PCS) of the SF-12 than healthy volunteers (p < 0.001). Pain was a significant predictor of reduced PCS of the SF-12 (p < 0.001) in a model that explained 23% of the variance. Conclusions Neurophysiological symptoms were common. Pain was a significant contributor to the health burden of patients with JHS. Research is required to explore the connectivity and implications of these symptoms in relation to the central nervous system. Implications: There is a requirement to acknowledge and understand the multidimensional nature of JHS

What is the best way to keep walking and moving around for individuals with Machado-Joseph disease? A scoping review through the lens of Aboriginal families with Machado-Joseph disease in the Top End of Australia

Objectives: Machado-Joseph disease (MJD) is the most common spinocerebellar ataxia worldwide. Prevalence is highest in affected remote Aboriginal communities of the Top End of Australia. Aboriginal families with MJD from Groote Eylandt believe 'staying strong on the inside and outside' works best to keep them walking and moving around, in accordance with six key domains that form the 'Staying Strong' Framework. The aim of this current study was to review the literature to: (1) map the range of interventions/strategies that have been explored to promote walking and moving around (functional mobility) for individuals with MJD and; (2) align these interventions to the 'Staying Strong' Framework described by Aboriginal families with MJD. Design: Scoping review. Data sources: Searches were conducted in July 2018 in MEDLINE, EMBASE, CINAHL, PsychINFO and Cochrane Databases. Eligibility criteria for selecting studies: Peer-reviewed studies that (1) included adolescents/adults with MJD, (2) explored the effects of any intervention on mobility and (3) included a measure of mobility, function and/or ataxia were included in the review. Results: Thirty studies were included. Few studies involved participants with MJD alone (12/30). Most studies explored interventions that aligned with two 'Staying Strong' Framework domains, 'exercising your body' (n=13) and 'searching for good medicine' (n=17). Few studies aligned with the domains having 'something important to do' (n=2) or 'keeping yourself happy' (n=2). No studies aligned with the domains 'going country' or 'families helping each other'. Conclusions: Evidence for interventions to promote mobility that align with the 'Staying Strong' Framework were focused on staying strong on the outside (physically) with little reflection on staying strong on the inside (emotionally, mentally and spiritually). Findings suggest future research is required to investigate the benefits of lifestyle activity programmes that address both physical and psychosocial well-being for families with MJD

Staying Strong Toolbox: co-design of a physical activity and lifestyle program for Aboriginal families with Machado-Joseph disease in the Top End of Australia

Physical activity has positive health implications for individuals living with neurodegenerative diseases. The success of physical activity programs, particularly in culturally and linguistically diverse populations, is typically dependent on their alignment with the culture, lifestyle and environmental context of those involved. Aboriginal families living in remote communities in the Top End of Australia invited researchers to collaborate with them to co-design a physical activity and lifestyle program to keep individuals with Machado-Joseph disease (MJD) walking and moving around. The knowledge of Aboriginal families living with MJD, combined with findings from worldwide MJD research, formed the foundation for the co-design. An experience-based co-design (EBCD) approach, drawing from Indigenous and Participatory methodologies, was used. An expert panel of individuals with lived experience of MJD participated in a series of co-design phases. Prearranged and spontaneous co-design meetings were led by local community researchers within each phase. Data was collected using a culturally responsive ethnographic approach and analysed thematically. Sixteen panel members worked to develop the 'Staying Strong Toolbox' to cater for individuals with MJD who are 'walking strong'; or 'wobbly'; or 'in a wheelchair'. Based on the 'Staying Strong Framework', the Toolbox was developed as a spiral bound A3 book designed to guide the user to select from a range of activities to keep them walking and moving around and to identify those activities most important to them to work on. The 'Staying Strong Toolbox' is a community driven, evidence based resource for a physical activity and lifestyle program for Aboriginal families with MJD. The Toolbox provides a guide for health professionals and support workers to deliver person-centred support to Aboriginal families with MJD, and that can be modified for use by other families with MJD or people with other forms of ataxia around the world

Ballistic protective properties of material representative of English civil war buff-coats and clothing

One type of clothing system used in the English Civil War, more common amongst cavalrymen than infantrymen, was the linen shirt, wool waistcoat and buff-coat. Ballistic testing was conducted to estimate the velocity at which 50% of 12-bore lead spherical projectiles (V50) would be expected to perforate this clothing system when mounted on gelatine (a tissue simulant used in wound ballistic studies). An estimated six-shot V50 for the clothing system was calculated as 102 m/s. The distance at which the projectile would have decelerated from the muzzle of the weapon to this velocity in free flight was triple the recognised effective range of weapons of the era suggesting that the clothing system would provide limited protection for the wearer. The estimated V50 was also compared with recorded bounce-and-roll data; this suggested that the clothing system could provide some protection to the wearer from ricochets. Finally, potential wounding behind the clothing system was investigated; the results compared favourably with seventeenth century medical writings

Childhood overweight and obesity and back pain risk : a cohort study of 466 997 children

To assess the association between age, sex, socioeconomic group, weight status and back pain risk in a large general population cohort of children. A dynamic cohort of children aged 4 years in the Information System for Research in Primary Care (SIDIAP) electronic primary care records data in Catalonia. Multivariable Cox models were fitted to explore the association between back pain and weight status categories according to the WHO 2007 growth reference groups (body mass index for age z-score). Models were adjusted for age, sex, socioeconomic status and nationality. Children seen at age 4 years at paediatric primary care clinics between 1 January 2006 and 31 December 2013 and followed up until 31 December 2016 or age 15 years. Incident back pain registered by paediatricians at primary care using the International Statistical Classification of Diseases and Health Related Problems, 10th Edition code M54. 466 997 children were followed for a median 5.0 years (IQR 5.1). In multivariable models, overweight and obesity increased back pain risk, with adjusted HRs of 1.18 (95% CI 1.09 to 1.27) and 1.34 (95%CI 1.19 to 1.51) for overweight and obesity, respectively. Females were at greater risk of back pain than males with adjusted HR 1.40 (95%CI 1.35 to 1.46). Adjusted HR was 1.43 (95%CI 1.33 to 1.55) for back pain in children from the most deprived socioeconomic groups compared with the least deprived socioeconomic groups. Maintaining a healthy weight from an early age may reduce the prevalence of back pain in both children and adults. Overweight female children from deprived socioeconomic groups are at greatest risk of back pain and represent a target population for intervention

Age-related differences in memory after attending to distinctiveness or similarity during learning

Episodic memory is vulnerable to age-related change, with older adults demonstrating both impairments in retrieving contextual details and susceptibility to interference among similar events. Such impairments may be due in part to an age-related decline in the ability to encode distinct memory representations. Recent research has examined how manipulating stimulus properties to emphasize distinctiveness can reduce age-related deficits in memory. However, few studies have addressed whether learning strategies that differentially encourage distinctiveness processing attenuate age-related differences in episodic memory. In the present study, participants engaged in two incidental encoding tasks emphasizing either distinctiveness or similarity processing. Results demonstrated higher rates of recollection for stimuli studied under the distinctiveness task than the similarity task in younger but not older adults. These findings suggest a declining capacity for distinctiveness processing to benefit memory in older adults, and raise the possibility that strategies that enhance gist-based encoding may attenuate age-related memory deficits