Suicidal Behaviors in Women With the Hypermobile Ehlers-Danlos Syndrome.

BACKGROUND: Hypermobile Ehlers-Danlos syndrome (hEDS) is the most frequent heritable disorder of the connective tissue. This is characterized by a generalized fragility of tissues leading to chronic pain, disability and high levels of psychological distress. Suicidal behaviors in those affected are not uncommon but they have not been well studied. We aimed to explore aspects of suicidality and related factors in a group of patients with hEDS. METHOD: Thirty-five women with hEDS were included in this cross-sectional study. They were assessed with the Mini-International Neuropsychiatric Interview for Axis 1 DSM-IV mental disorders and suicidality. They also responded to self-questionnaires assessing health (pain, BMI, and diagnosis delay) and psychosocial variables (social support, physical functioning, coping strategies, personality disturbances, and resilience). RESULTS: Eleven patients (31.4%) had attempted suicide in the past. Fifteen patients (42.9%) had some degree of suicide risk at the time of evaluation, mainly mild risk (60%). Compared with patients without a history of suicide attempt, those who had attempted suicide were younger, scored higher on personality disturbances, especially on depressive, avoidant, antisocial, and borderline trait subscores, and had an increased prevalence of lifetime major depression, mania/hypomanic episodes, and anxiety disorders (p < .05). Binary logistic regression showed that personality disturbances and anxiety disorders increase the probability of belonging to the attempters group. CONCLUSIONS: Consistent with previous reports, these data highlight the high frequency of suicidal behaviors in hEDS patients as well as the importance to explore psychopathology in those affected in order to identify vulnerable individuals and provide specific support.HIGHLIGHTSAttempted suicide in patients with hEDS is not uncommon.Age and the presence of psychopathology are associated with suicide attempt in hEDS patients.Personality disturbances and lifetime anxiety disorders predicted suicide attempted in this sample.

Compressive Garments in Individuals with Autism and Severe Proprioceptive Dysfunction: A Retrospective Exploratory Case Series.

(1) Background: Compression garments (CGs) are an adjuvant treatment for generalized joint hypermobility (GJH), including the Ehlers-Danlos syndrome/hypermobility types. The effects of CGs are likely to be related to better proprioceptive control. We aimed to explore the use of CGs in individuals with autism and severe proprioceptive dysfunction (SPD), including individuals with GJH, to control posture and challenging behaviors. (2) Methods: We retrospectively described 14 patients with autism and SPD, including seven with comorbid GJH, who were hospitalized for major challenging behaviors with remaining behavioral symptomatology after the implementation of multidisciplinary approaches, including medication, treatment of organic comorbidities, and behavioral restructuring. Each patient received a CG to wear for at least 1 h (but most often longer) per day for six weeks. We assessed challenging behaviors in these participants with the Aberrant Behavior Checklist (ABC), sensory integration with the Dunn questionnaire, and postural sway and motor performance using a self-designed motricity path at baseline, two weeks, and six weeks. (3) Results: We observed a significant effect on most ABC rating scores at two weeks, which persisted at six weeks (total score, p = 0.004; irritability, p = 0.007; hyperactivity, p = 0.001; lethargy, p = 0.001). Postural control in dorsal and profile positions was significantly improved between before and after wearing the CGs (p = 0.006 and 0.007, respectively). Motor performance was also significantly improved. However, we did not observe a significant change in Dunn sensory scores. During the six-week duration, the treatment was generally well-tolerated. A comorbid GJH diagnosis was not associated with a better outcome. (4) Conclusions: CGs appear to be a promising adjuvant treatment for both behavioral and postural impairments in individuals with autism and SPD.

Predictors of pain and mobility disability in the hypermobile Ehlers-Danlos syndrome.

Aim: Among the Ehlers-Danlos syndromes, the hypermobile subtype (hEDS) is the most common. The variety, accumulation and duration of the painful symptoms make hEDS a chronic and highly disabling condition. Identifying drivers of pain and mobility disability in hEDS is necessary to provide adapted prevention and intervention programs.Methods: A cross-sectional sample of 75 adults with hEDS according to Villefranche criteria (67 women; 36 years, SD = 11.2) responded to self-assessment questionnaires regarding psychological (negative thoughts and emotions), health (pain, fatigue, diagnostic delay, body mass index, and mobility disability) and socio-demographic variables.Results: Forty-three percent of the participants suffer from severe pain, and two thirds suffer from a high level of mobility disability. Multivariate analyses show that delay of diagnosis, being professionally active and thoughts of helplessness are variables that increase the likelihood of severe pain, whereas age, fatigue, and body mass index predict mobility disability.Conclusion: These results confirm previous research concerning the high disease burden observed in hEDS patients, and highlight the importance of multidisciplinary management that includes nutritional and psychological advice to address this pathology. Increasing awareness of the clinical aspects of hEDS among physicians is necessary to reduce diagnostic delay and minimize negatives outcomes.Implications for RehabilitationA high proportion of patients with hypermobile Ehlers-Danlos syndrome suffer from severe pain (>42%) and a high level of mobility disability (>65%).Different psychosocial and health variables predict pain and mobility disability in patients with hypermobile Ehlers-Danlos syndrome.Awareness concerning the clinical picture of hypermobile Ehlers-Danlos syndrome among health care professionals is necessary to reduce diagnosis delay and the burden of the disease.

Autism, Joint Hypermobility-Related Disorders and Pain.

Autism Spectrum Disorder (ASD) and Joint Hypermobility-Related Disorders are blanket terms for two etiologically and clinically heterogeneous groups of pathologies that usually appears in childhood. These conditions are seen by different medical fields, such as psychiatry in the case of ASD, and musculoskeletal disciplines and genetics in the case of hypermobility-related disorders. Thus, a link between them is rarely established in clinical setting, despite a scarce but growing body of research suggesting that both conditions co-occur more often than expected by chance. Hypermobility is a frequent sign of hereditary disorders of connective tissue (e.g., Ehlers-Danlos syndromes, Marfan syndrome), in which the main characteristic is the multisystem fragility that prone to proprioceptive and motor coordination dysfunction and hence to trauma and chronic pain. Considering the high probability that pain remains disregarded and untreated in people with ASD due to communication and methodological difficulties, increasing awareness about the interconnection between ASD and hypermobility-related disorders is relevant, since it may help identify those ASD patients susceptible to chronic pain.

Low- and high-anxious hypermobile Ehlers-Danlos syndrome patients: comparison of psychosocial and health variables.

Despite the frequent co-ocurrence of hypermobile Ehler-Danlos syndrome (hEDS) and pathological anxiety, little is known about the psychosocial and health implications of such comorbidity. Our aim was to explore the association between high levels of anxiety and psychosocial (catastrophizing, kinesiophobia, somatosensory amplification, social support and functioning), health (pain, fatigue, BMI, tobacco/alcohol use, depression, diagnosis delay, general health), and sociodemographic factors in people with hEDS. In this cross-sectional study, 80 hEDS patients were divided into two groups according to self-reported anxiety levels: low and high. Psychosocial, sociodemographic and health variables were compared between the groups. Forty-one participants reported a high level of anxiety (51.2%). No differences were found in the sociodemographic variables between high-anxious and low-anxious patients. The percentage of participants with severe fatigue and high depressive symptomatology was significantly higher in the high-anxious group (80.5 vs 56.4; 26.8 vs 12.8%, respectively). High-anxious hEDS patients also showed significantly higher levels of pain catastrophizing, somatosensory amplification as well as a poorer social functioning and general health. Multivariate analyses showed that somatosensory amplification, pain catastrophizing and poor social functioning are variables that increase the probability of belonging to the high-anxious group. Despite limitations, this first study comparing high-anxious versus low-anxious hEDS patients with respect to health aspects, highlight the importance of considering the psychosocial factors (many susceptible to modification), to improve the adjustment to this chronic condition and provide support to those affected through a biopsychosocial approach.

Diagnosis of Ehlers-Danlos syndrome after a first shoulder dislocation.

BACKGROUND: Shoulder dislocation is often the first symptom of Ehlers-Danlos syndrome (EDS). Whether it occurs in early-onset EDS is unknown. In most cases, surgical failure leads to the diagnosis. We aimed to determine whether clinical symptoms can signal the presence of EDS at a first dislocation. MATERIALS AND METHODS: In this retrospective study, we analyzed clinical and radiologic data for 27 patients with EDS and shoulder instability and a control population of 40 consecutive non-EDS patients undergoing surgery for an unstable shoulder. Data were collected on gender, age, single or bilateral disease, general hyperlaxity, shoulder hyperlaxity, number of dislocations or subluxations, nontraumatic onset, and pain specificity. Nerve and vascular injuries, joint disorders, and family history were recorded, and radiologic data were reported. RESULTS: Age <14 years, female sex, bilateral disorder, and general hyperlaxity were significantly more frequent in patients with EDS and a first dislocation than in those without EDS. Painless dislocation with pain after dislocation and concomitant nerve injury were more frequent in affected patients, as were hemostasis disorders and a family history of joint hyperlaxity. Bone lesions were not seen on radiographs. Only the hyperlaxity sign (external rotation >85°) did not differ between the groups. CONCLUSION: After a first dislocation in a young girl with global hyperlaxity but not necessarily shoulder hyperlaxity, painless atraumatic dislocation with pain after reduction can suggest EDS.

Pain management in the Ehlers-Danlos syndromes.

Chronic pain in the Ehlers-Danlos syndromes (EDS) is common and may be severe. According to one study, nearly 90% of patients report some form of chronic pain. Pain, which is often one of the first symptoms to occur, may be widespread or localized to one region such as an arm or a leg. Studies on treatment modalities are few and insufficient to guide management. The following is a discussion of the evidence regarding the underlying mechanisms of pain in EDS. The causes of pain in this condition are multifactorial and include joint subluxations and dislocations, previous surgery, muscle weakness, proprioceptive disorders, and vertebral instability. Affected persons may also present with generalized body pain, fatigue, headaches, gastrointestinal pain, temporomandibular joint pain, dysmenorrhea, and vulvodynia. Pain management strategies may be focused around treating the cause of the pain (e.g., dislocation of a joint, proprioceptive disorder) and minimizing the sensation of pain. Management strategies for chronic pain in EDS includes physical therapy, medications, as well as durable medical equipment such as cushions, compressive garments, and braces. The different modalities are discussed in this paper. © 2017 Wiley Periodicals, Inc.

Gynecologic symptoms and the influence on reproductive life in 386 women with hypermobility type ehlers-danlos syndrome: a cohort study.

BACKGROUND: Hypermobile Ehlers-Danlos syndrome (hEDS), is probably the most common disease among heritable connective tissue disorders. It affects women more than men and causes symptoms in multiple organs. It is associated with chronic pain, skin fragility and abnormal bleeding. These characteristics may hamper reproductive life. We conducted a study to evaluate the gynecologic and obstetric outcomes in women with hEDS. We also explored a possible hormonal modulation of the hEDS symptoms. The gynecologic and obstetric history of 386 consecutive women diagnosed with hEDS was collected by a standardized questionnaire and a medical consultation performed by a senior gynecologist in an expert centre for hEDS between May 2012 and December 2014. RESULTS: We observed a high frequency of gynecologic complaints, specifically: menorrhagia (76 %), dysmenorrhea (72 %) and dyspareunia (43 %). Endometriosis was not highly prevalent in this population. The obstetric outcomes were similar to those of the general French population for deliveries by cesarean section (14.6 %) and premature births (6.2 %) but the incidence of multiple spontaneous abortion (13 %) and spontaneous abortion (28 %) were significantly higher. A subset of women were sensitive to hormonal fluctuations with more severe symptoms occurring during puberty, prior to menstruation, during the postpartum period as well as on oral contraception. CONCLUSIONS: Increased awareness of the gynecological symptomatology in women with hEDS can help discriminate between endometriosis and thus prevent useless, and potentially dangerous, surgery. This study also suggests that hormonal modulation may be an appropriate treatment for a subset of women with hEDS.

[Not Available]. / Ehlers-Danlos-Tschernogobow : Histoire contrarlée de la maladie.

Ehlers-Danlos disease had a paradoxical fate. Clinically identified 123 years ago, it is unknown to nearly all present doctors. Often described for its dramatic aspects, diagnostic confusion with fibromyalgia in particular, ignorance of effective treatments such as oxygen therapy and orthotics are new concepts that should shake the prejudices derived from the history of this disease.

Brain injury unmasking Ehlers-Danlos syndromes after trauma: the fiber print.

BACKGROUND: The role of physical trauma in the onset of symptoms in Ehlers-Danlos syndrome (EDS) has never been characterized. We sought to search and describe brain lesions EDS patients also having personal history of physical trauma. We systematically performed brain magnetic resonance imaging in a first cohort of patients with a hypermobility type of EDS which described the onset of their disease or its worsening after a physical trauma. Unexpected yet consistent findings that were thought to be related to the reported traumas led to perform brain imaging in all subsequent patients with similar symptoms regardless of a history of trauma and to search for a prior trauma by active questioning. RESULTS: Fifty-nine patients were recruited and analyzed, among which 53 (89.8%) were women. Overall, 26 (44.1%) reported a personal history of physical trauma. Six signs pertaining to subcortical lesions and affecting white matter tracts were identified. Those included lesions of the reticular formation, the two lenticular nuclei, the corpus callosum and the arcuate fasciculus. Thirty-six patients (61.0%) had at least 5 of the 6 imaging signs. In case of a trauma before 18, patients had significantly more lesions of the reticular formation (100% vs. 50%; p = 0.0035). CONCLUSIONS: Patients with EDS, hypermobility type, were found to have consistent and specific brain lesions involving white matter tracts. Moreover, the record of a physical trauma in a substantial proportion of cases suggests that these lesions could be post-trauma consequences. Therefore, physical trauma could be a triggering factor in EDS.

Difficulty eating and significant weight loss in joint hypermobility syndrome/Ehlers-Danlos syndrome, hypermobility type.

Joint Hypermobility Syndrome, also known as Ehlers-Danlos Syndrome Hypermobility Type (JHS/EDS-HT), is a heritable disorder of connective tissue, common but poorly known by the medical community. Although generalized joint hypermobility and fragility of tissues have been described as core features, recent research highlights the multisystemic nature of JHS/EDS-HT, which presents with a wide range of articular and extra-articular symptoms. Among these, gastrointestinal problems, temporomandibular disorders, and smell and taste abnormalities are common among those affected, having significant implications for eating. The present work reviews the literature linking JHS/EDS-HT and eating problems. Two illustrative case reports, in which JHS/EDS-HT manifestations contribute to developing and maintaining disturbed eating behaviors and significant weight loss, are presented.

Functional digestive symptoms and quality of life in patients with Ehlers-Danlos syndromes: results of a national cohort study on 134 patients.

BACKGROUND AND OBJECTIVES: Ehlers-Danlos syndromes (EDS) are a heterogeneous group of heritable connective tissue disorders. Gastrointestinal manifestations in EDS have been described but their frequency, nature and impact are poorly known. We aimed to assess digestive features in a national cohort of EDS patients. METHODS: A questionnaire has been sent to 212 EDS patients through the French patient support group, all of which had been formally diagnosed according to the Villefranche criteria. The questionnaire included questions about digestive functional symptoms, the GIQLI (Gastrointestinal Quality of Life Index), KESS scoring system and the Rome III criteria. RESULTS: Overall, 135 patients (64% response rate) completed the questionnaire and 134 were analyzable (123 women; 91%). Mean age and Body Mass Index were respectively 35±14.7 years and 24.3±6.1 kg/m(2). The most common EDS subtype was hypermobility form (n=108; 80.6%). GIQLI and KESS median values were respectively 63.5 (27-117) and 19 [13.5-22]. Eighty four percent of patients had functional bowel disorders (FBD) according to the Rome III criteria. An irritable bowel syndrome according to the same criteria was observed in 64 patients (48%) and 48 patients (36%) reported functional constipation. A gastro-esophageal reflux disease (GERD) was reported in 90 patients (68.7%), significantly associated with a poorer GIQLI (60.5±16.8 versus 75.9±20.3; p<0.0001). GIQLI was also negatively impacted by the presence of an irritable bowel syndrome or functional constipation (p=0.007). There was a significant correlation between FBD and GERD. CONCLUSIONS: Natural frequency of gastrointestinal manifestations in EDS seems higher than previously assessed. FBD and GERD are very common in our study population, the largest ever published until now. Their impact is herein shown to be important. A systematic clinical assessment of digestive features should be recommended in EDS.

Effectiveness of a lumbar belt in subacute low back pain: an open, multicentric, and randomized clinical study.

STUDY DESIGN: Multicentric, randomized, and controlled study of clinical evaluation of medical device in subacute low back pain. OBJECTIVE: To evaluate the effects of an elastic lumbar belt on functional capacity, pain intensity in low back pain treatment, and the benefice on medical cost. SUMMARY OF BACKGROUND DATA: There is limited evidence of efficiency of lumbar supports for treatment of low back pain. There is also a lack of the methodology in the studies reported on the efficiency of this device. METHODS: This study is randomized, multicentric, and controlled with 2 groups: a patient group treated with a lumbar belt (BWG) and a control group (CG). The main criteria of clinical evaluation were the physical restoration assessed with the EIFEL scale, the pain assessed by a visual analogic scale, the main economical criteria was the overall cost of associated medical treatments. RESULTS: One hundred ninety-seven patients have participated. The results show a higher decrease in EIFEL score in BWG than CG between days 0 and 90 (7.6 +/- 4.4 vs. de 6.1 +/- 4.7;P = 0.023). Respectively significant reduction in visual analogic scale was also noticed (41.5 +/- 21.4 vs. 32.0 +/- 20; P = 0.002). Pharmacologic consumption decreased at D90 (the proportion of patients who did not take any medication in BWG is 60.8% vs. 40% in CG;P = 0.029). CONCLUSION: Lumbar belt wearing is consequent in subacute low back pain to improve significantly the functional status, the pain level, and the pharmacologic consumption. This study may be useful to underline the interest of lumbar support as a complementary and nonpharmacologic treatment beside the classic medication use in low back pain treatment.

[The appreciation of the handicap in the brothers of the coast (1664-1675), according to Alexandre-Olivier Exmelin, surgeon of the privateers]. / L'appréciation du handicap chez les frères de la côte (1664-1675), selon Alexandre-Olivier Exmelin, chirurgien de la flibuste.

The reparation of corporeal damages, consequences of intentional or no intentional violence is a part of measurement of stability and progress in the human societies interested by a dignity life for the victims. Initiated by Hammourabi Code and continued by the Jews in the Bible, the reference was (now and still its) the amputed or impaired part of body (hand, arm, leg, eye...). For every part a fare in money was indicated or a rate in percentage. The Coast brothers translate in ecus or in slaves. This code indicates the originality of a society founded on violence, the robbery and murder with introduction of cooperative if not democratic modalities of functioning. The role of Bertrand d'Ogeron, governor of the Turtle Island was very beneficent.

Andrew Taylor Still and the birth of osteopathy (Baldwin, Kansas, USA, 1855).

Osteopathy has gained ground in recent years and has been seeking recognition in France. Physicians often lack the information needed to answer patients who have derived from the media, advertisements, and other patients what they believe is a clear idea of osteopathy and its twin sister chiropractic. My academic activities led me to the heart of the United States, to Kansas, where settlers and Indians once stood face to face and where wagon trains left daily for the Western territories. There, in Baldwin, Andrew Taylor Still "discovered" osteopathy. I conducted an in-depth study of the birth of osteopathy and of the ideological and cultural influences that shaped this doctrine. The circumstances that surrounded the development of osteopathy deserve to be widely known because they explain how contemporary osteopaths work. Indeed, although the terms are different, the ideology that underlies osteopathy seems unchanged. The history of osteopathy emphasizes the importance of logical thinking in medicine, of the principle of pathophysiological foundation, of diagnostic hypotheses, and of careful treatment selection complying with the rules of deontology and ethics. Osteopathy is without doubt a product of society and perhaps also of vogue. It cannot leave physicians indifferent.