MACVIA-LR (Fighting Chronic Diseases for Active and Healthy Ageing in Languedoc-Roussillon): A Success Story of the European Innovation Partnership on Active and Healthy Ageing.

The Région Languedoc Roussillon is the umbrella organisation for an interconnected and integrated project on active and healthy ageing (AHA). It covers the 3 pillars of the European Innovation Partnership on Active and Healthy Ageing (EIP on AHA): (A) Prevention and health promotion, (B) Care and cure, (C) and (D) Active and independent living of elderly people. All sub-activities (poly-pharmacy, falls prevention initiative, prevention of frailty, chronic respiratory diseases, chronic diseases with multimorbidities, chronic infectious diseases, active and independent living and disability) have been included in MACVIA-LR which has a strong political commitment and involves all stakeholders (public, private, patients, policy makers) including CARSAT-LR and the Eurobiomed cluster. It is a Reference Site of the EIP on AHA. The framework of MACVIA-LR has the vision that the prevention and management of chronic diseases is essential for the promotion of AHA and for the reduction of handicap. The main objectives of MACVIA-LR are: (i) to develop innovative solutions for a network of Living labs in order to reduce avoidable hospitalisations and loss of autonomy while improving quality of life, (ii) to disseminate the innovation. The three years of MACVIA-LR activities are reported in this paper.

Therapeutic education in persons with spinal cord injury: a review of the literature.

INTRODUCTION: Therapeutic education is an integrant part of the physical medicine and rehabilitation care of persons with spinal cord injury. It is often conducted in an empirical manner. The objective of this literature review was to evaluate the state of the art regarding the evaluations and therapeutic education programs for persons with spinal cord injury. MATERIAL AND METHOD: Systematic review of the literature with Medline and Cochrane Library databases from 1966 to 2009. RESULTS: The main areas of interest, for the evaluations found in the literature, focused on clinical variables, patients' knowledge, health behaviors, functional independence and quality of life but also psychological dimensions such as health locus of control, representations, abilities to resolve problems and self-perceived efficacy. Ten clinical studies were retained for analysis. These clinical studies were built around various health and educational models. An impact was highlighted on clinical variables (pressure ulcer, urinary tract infection), knowledge, quality of life and psychological criteria (depression, self-perceived efficacy, coping and problem solving strategies). DISCUSSION AND CONCLUSION: The global level of evidence on the effects of therapeutic education in persons with spinal cord injury is low because of the number of studies and their low statistical power and requires additional studies. However, the analysis of the literature allows for discussing the organization of therapeutic education in clinical practice.

Evaluating self-reported pressure ulcer prevention measures in persons with spinal cord injury using the revised Skin Management Needs Assessment Checklist: reliability study.

STUDY DESIGN: Cross-cultural adaptation and reliability study. OBJECTIVE: To translate, evaluate the reliability and cross-culturally adapt the Skin Management Needs Assessment Checklist (SMnac), a questionnaire evaluating the knowledge on pressure ulcer (PU) prevention measures in persons with spinal cord injury (SCI). SUBJECTS: 138 persons with SCI, mean age 45.9 years, mean time since injury 94 months. MATERIAL AND METHOD: The study was carried out in two stages. First, the questionnaire went through a forward-backward translation process and was cross-culturally adapted, according to a validated methodology for self-reported measures. Then, the test-retest reliability was evaluated on a population of persons with SCI. RESULTS: The standardized back-translation and cross-cultural adaptation led to the revised Smack grid, with the addition of seven items representing an update of PU prevention measures. The reliability was excellent (intraclass correlation coefficient: 0.899). CONCLUSION: The revised SMnac is an adaptation of the SMnac, including therapeutic education frameworks and the latest PU prevention practices. It appears to be a reliable tool for assessing the knowledge and benefits of PU prevention in persons with SCI. Further studies are needed to explore its validity and responsiveness to change.

Using an analytical hierarchy process (AHP) for weighting items of a measurement scale: a pilot study.

BACKGROUND: Many clinical scales contain items that are scored separately prior to being compiled into a single score. However, if the items have different degrees of importance, they should be weighted differently before being compiled. The principal aims of this study were to show how the "analytic hierarchy process" (AHP), which has never been used for this purpose, can be applied to weighting the six items of the "London handicap scale", and to compare the AHP to the "conjoint analysis" (CA), which was previously implemented by Harwood et al. (1994) [1]. DESIGN: In order to assess the relative importance of the six items, we submitted AHP and CA to a group of 10 physiatrists. We compared the methods in terms of item ranking according to importance, assessment of fictitious patients based on weights determined by each method, and perceived difficulty by the physiatrist. RESULTS: For both techniques, "Physical independence" (PHY) was the best-weighted item, but other ranks varied depending on the technique. AHP was better than CA in terms of accuracy (global assessment of the clinical status) and perceived difficulty. CONCLUSION: AHP may be used to reveal the importance that experts assign to the items of a multidimensional scale, and to calculate the appropriate weights for specific items. For this purpose, AHP seems to be more accurate than CA.

Aging and sequelae of poliomyelitis.

OBJECTIVE: We estimate that there are about 50,000 persons who survived poliomyelitis in their childhood in France (mean age estimated between 50 and 65 years). After a few decades of stability, 30 to 65% of individuals who had been infected and recovered from polio begin to experience new signs and symptoms. METHOD: Review of the literature on Pubmed with the following keywords "Poliomyelitis" and "Post-Polio Syndrome (PPS)". RESULTS: These new signs and symptoms are characterized by muscular atrophy (decreased muscle mass), muscle weakness and fatigue, muscle and/or joint pain. All these symptoms lead to significant changes in mobility with falls and inability to carry on with daily life activities. There are several intricate causes. The normal aging process and weight gain are regularly blamed. Respiratory disorders and sleep disorders must be looked for: respiratory insufficiency, sleep-related breathing disorders such as sleep apnea, restless legs syndrome. Orthopedics complications are quite common: soft-tissue pathologies of the upper limbs, degenerative pathologies of the large joints or spinal cord, fall-related fractures. Finally, the onset of an authentic PPS is possible. CONCLUSION: The therapeutic care of this late functional deterioration requires regular monitoring check-ups in order to implement preventive measures and appropriate treatment. This therapeutic care must be multidisciplinary as physical rehabilitation; orthotics and technical aids are all essential.

An atypical ballistic traumatic cauda equina syndrome with a positive outcome. Focus on prognostic factors.

INTRODUCTION: Traumatic cauda equina syndromes due to projectile wounds often have a poor prognosis. We report on an unusual ballistic cauda equina traumatism with a good functional outcome. CLINICAL CASE: A 44-year-old man was admitted to emergency room for an incomplete cauda equina syndrome after trying to kill himself by means of a pneumatic nail gun. The nail had gone right through the third lumbar vertebra. Because of the stability of the fracture, orthopaedic surgery was not indicated. Neurological recovery was progressive. At 6 months, there was still a partial L5-S1 motor deficit on the left side but the patient could walk without crutches, and within an unlimited walking distance. DISCUSSION: Initial imaging displayed a projectile trajectory focused on the spinal canal on level L3, which could have been considered as bad prognosis. The positive analytic and functional outcome correlates with the limited neurological tissue damage, probably explained by the ballistic properties of the projectile. CONCLUSION: Apart from the influence of a possible surgical act, the neurological and functional prognosis of a traumatic cauda equina syndrome caused by a projectile also depends on its physical characteristics.

Pressure ulcer risk factors in persons with spinal cord injury part 2: the chronic stage.

INTRODUCTION: Pressure ulcers (PUs) are a common complication following spinal cord injury (SCI). Prevalence for persons in the chronic SCI stage varies between 15 and 30%. The risk assessment scales used nowadays were designed on pathophysiological concepts and are not SCI-specific. Recently, an epidemiological approach to PU risk factors has been proposed for designing an SCI-specific assessment tool. The first results seem quite disappointing, probably because of the level of evidence of the risk factors used. OBJECTIVE: To determine PU risk factors correlated to the chronic stage of SCI. MATERIALS AND METHODS: Systematic review of the literature. RESULTS: There are several PU risk factors for chronic SCI stage: socio-demographics, neurological, medical or behavioral. The level of evidence varies: it is quite high for the socio-demographics and neurological factors and low for behavioral factors. DISCUSSION AND CONCLUSION: Behavioral risk factors (relieving the pressure, careful skin monitoring, smoking) are probably the ones for which a preventive strategy can be established. It is important to develop specific assessment tools for these behavioral risk factors to determine their relevance and evaluate the effect of therapeutic educational programs on persons with SCI.

Rehabilitation of arm function after stroke. Literature review.

INTRODUCTION: In the recent literature we can find many articles dealing with upper extremity rehabilitation in stroke patients. New techniques, still under evaluation, are becoming the practical applications for the concept of post-stroke brain plasticity. METHODS: This literature review focuses on controlled randomized studies, reviews and meta-analyses published in the English language from 2004 to 2008. The research was conducted in MEDLINE with the following keywords: "upper limb", "stroke", "rehabilitation". RESULTS: We reviewed 66 studies. The main therapeutic strategies are: activation of the ipsilesional motor cortex, inhibition of the contralesional motor cortex and modulation of the sensory afferents. Keeping a cortical representation of the upper limb distal extremity could prevent the learned non-use phenomenon. The modulation of sensory afferents is then proposed: distal cutaneous electrostimulation, anesthesia of the healthy limb, mirror therapy, virtual reality. Intensifying the rehabilitation care means increasing the total hours of rehabilitation dedicated to the paretic limb (proprioceptive stimulation and repetitive movements). This specific rehabilitation is facilitated by robot-aided therapy in the active-assisted mode, neuromuscular electrostimulation and bilateral task training. Intensifying the rehabilitation training program significantly improves the arm function outcome when performed during subacute stroke rehabilitation (< six months). Ipsilesional neurostimulation as well as mental practice optimize the effect of repetitive gestures for slight motor impairments. Contralesional neurostimulation or anesthesia of the healthy hand both improve the paretic hand's dexterity via a decrease of the transcallosal inhibition. This pathophysiological mechanism could also explain the positive impact of constraint-induced movement therapy (CI therapy) in an environmental setting for chronic stroke patients. CONCLUSION: To ensure a positive functional outcome, stroke rehabilitation programs are based on task-oriented repetitive training. This literature review shows that exercising the hemiparetic hand and wrist is essential in all stages of a stroke rehabilitation program. New data stemming from neurosciences suggest that ipsilesional corticospinal excitability should be a priority.

Pressure ulcer risk factors in persons with SCI: Part I: Acute and rehabilitation stages.

BACKGROUND: Pressure ulcers (PUs) are a common complication following a spinal-cord injury (SCI). Good prevention requires identifying the individuals at risk for developing PUs. Risk assessment scales used nowadays were designed on pathophysiological concepts and are not SCI-specific. Recently, an epidemiological approach to PU risk factors has been proposed to design an SCI-specific assessment tool. The first results seem quite disappointing, probably because of the level of evidence of the risk factors used. OBJECTIVE: To determine PU risk factors correlated to the patients with SCI, medical care management during the acute as well as in the rehabilitation and chronic stages. This first part focuses on identifying the risk factors during the acute and rehabilitation stages. MATERIALS AND METHODS: Systematic review of the literature. RESULTS: Six studies met our inclusion criteria. The risk factors during the acute stage of an SCI are essentially linked to care management and treatment modalities. There is insufficient evidence to make a recommendation on medical risk factors, except for low blood pressure on admission to the Emergency Room, with a moderate level of evidence. Regarding the rehabilitation stage, no study was deemed relevant. DISCUSSION AND CONCLUSIONS: Additional observational studies are needed, for both the acute and rehabilitation stages, to improve this level of evidence. However, this systematic review unveiled the need for a carefully assessed t care management and the related practices, especially during the acute stage of an SCI.

Qualitative evaluation of the expectations of low back pain patients with regard to information gained through semi-directed navigation on the Internet.

OBJECTIVES: To make a qualitative analysis of the expectations of chronic low back pain (LBP) sufferers with regard to information gained using semi-directed Internet navigation on a sample of French LBP-related websites, and to compare the results with those of physical medicine and rehabilitation (PMR) medical doctors (MD). MATERIAL AND METHODS: Twenty-seven hospitalised chronic LBP sufferers assessed in ecological conditions a sample of seven LBP-related websites. The sites were assessed using a simplified version of a rating scale of patients' expectations. Analysis of the relative importance of the different kinds of information delivered was done using a point sharing method. RESULTS: In a comfortable environment, patients gave high scores for medical and extra-medical information, but low scores for website design. Overall quality assessment was similar for patients and MDs. The relative importance of medical and extra-medical information, and design quality, was similar for Chronic LBP patients and MDs. CONCLUSION: PMR MD seemed able to correctly evaluate the overall expectations of chronic LBP patients with regard to information, but their opinions on the different qualities of websites were different. Doctors and patients should collaborate in order to create or validate high quality websites concerned with LBP.

[Bickerstaff's syndrome presenting with coma, tetraplegia and blindness]. / Coma, tétraplégie flasque et cécité révélant un syndrome de Bickerstaff.

INTRODUCTION: Bickerstaff brainstem encephalitis is characterized by the occurrence of ataxia, ophthalmoplegia, motor weakness with areflexia and central nervous system symptoms, with drowsiness, pyramidal syndrome and sensorial symptoms. Diagnosis is based on MR findings and GQ1b antibodies. Treatment is not well known. OBSERVATION: We report a patient aged 39 years native of Laos who presented weakness, loss of reflexes, and drowsiness. Brain MR showed hyperintense signals in the brain stem. GQ1b antibodies were positive. The course was characterized by decrease of the weakness, normalization of MR and negativity of GQ1b antibodies. DISCUSSION: This observation underlines common features of Bickerstaff brainstem encephalitis, Miller Fisher syndrome and Guillain Barre syndrome. A favorable course and GQ1b antibodies are shared by these syndromes.

[Complex regional pain syndrome of the knee: early and beneficial action of diphosphonates on pain and function]. / Algodystrophie de genou: effet précoce et spectaculaire des biphosphonates sur la douleur et la fonction.

We report the case of a 24-years-old man with parcellar Complex Regional Pain Syndrome I (CRPS I) of the patella, responsible for major functional limitation. The diagnosis was based on physical exam and X-ray, in the absence of other articular or peri-articular diseases. The patient received two pamidronate perfusions over a week, with a spectacular decrease of pain, which allowed him to follow the rehabilitation program in good conditions. He was therefore able to go back to work. The place of this treatment in CRPS I is discussed.

[Influence of acute aggravations on the development of long-term handicap in relapsing remitting multiple sclerosis: a clinical study in 99 patients]. / Influence des poussées sur la survenue d'un handicap au long cours dans la sclérose en plaques de forme rémittente: étude observationnelle de 99 patients.

INTRODUCTION: Rate of relapse occurring during the first 5 years of MS-RR is a prognosis factor of occurrence of disability or secondary progressive (SP) phase. Progressive phase, related to chronic axonal loss, is mainly considered as the principal factor of disability progression. Influence of acute relapses during the relapsing-remitting phase on disability development is not known as a prognosis factor. OBJECTIVES: To determine the influence of the exacerbations among patients with RR-MS after the second clinical event on the disability occurrence. METHODS: Diagnosis of multiple sclerosis was established according to Poser's classification. Disability measurement was made with the use of the Expanded Disability Status Scale (EDSS). The patients included in the study were classified as clinically definite RR-MS, with an EDSS score500 m. The study began at the time of the second clinical event and ended when an EDSS score of 4.0 was reached or when a SP phase was beginning or at the last follow-up visit date if these two stages were not reached. The primary outcome measure was the comparison of the risk and the average time to reach an EDSS>or=4.0 or a SP form according to the annual exacerbation rate (AER) using Kaplan-Meier survival curve. RESULTS: Among the 238 ms patients of the database, 136 patients were classified as having a definite RR-MS. Among these 136 patients, 99 patients could be included in the study according to the inclusion criteria. The median follow up of the patients since the first clinical event was 9.8 years (range 4 to 44). The average EDSS score was 0.7 at the beginning of the study and 2.3 at the end. 20.2p.cent of patients (n=20) reached an EDSS score of 4.0 or a SP-MS. The median AER was 0.4 and the average 0.62 (range 0 to 6.1). The time to reach the primary end point for 25p.cent of the population was 17.8 years in group with an AER<0.4 (group A) and 6.9 years in group with an AER>0.4 (group B) (logrank; p<0.0001). The relative risk for patients of the group B compared to group A to reach an EDSS of 4.0 or a SP form was 8.01 (IC-95p.cent: 2.74-23.46; p=0.0001). CONCLUSIONS: In spite of a limited number of patients, this study gives evidence that a high rate of acute exacerbations in RR-MS patients after the second clinical event may be an independent predictive factor of long-term residual disability progression. High relapse rate leads to a more frequent and faster SP or EDSS>4.0 occurrence.

[Family study allows more optimistic prognosis and genetic counselling in a child with a deletion of exons 50-51 of the dystrophin gene]. / Impact de l'étude familiale sur le pronostic et le conseil génétique chez un enfant porteur d'une délétion des exons 50-51 du gène de la dystrophine.

INTRODUCTION: In frame deletions of exons encoding the central rod domain of dystrophin have been associated with a highly variable phenotype, including asymptomatic individuals. The lack of family history impairs accurate genetic counselling. OBSERVATION: We report on a 4-year-old child suffering from transient episodes of limping at the age of 2 and several episodes of fall since the age of 3. Clinical examination did not show muscle weakness. CPK levels were increased (1300 UI). EMG was normal. Muscle histology showed a rhabdomyolysis without features of muscular dystrophy. Immunolabelling for dystrophin, merosin and dysferlin were normal. Western blot analysis of muscular proteins showed reduced-size dystrophin bands and a slightly reduced intensity for dystrophin, alpha and gamma-sarcoglycan. Multiplex PCR of the dystrophin gene showed an in-frame deletion of exons 50-51, predicted to be associated to a Becker type of dystrophinopathy. Intragenic markers and quantitative PCR suggested maternal inheritance. This was confirmed by testing the maternal grand-parents, revealing that the asymptomatic 69-year-old grand father was a carrier. Three additional healthy males, whose ages ranged from 28 to 55 years and who were asymptomatic, also carried the mutation. The proband became spontaneously asymptomatic and cardiac echography was normal. In light of these data, genetic counselling was more reassuring and the mutation carrier maternal aunt, who was pregnant, decided to continue the pregnancy. CONCLUSION: This case report emphasizes the importance of family molecular analysis, especially in males from the maternal lineage, for genetic counselling of dystrophinopathies associated to atypical features or to an isolate increase of muscular enzymes level in a young boy with no positive family history.

[Does the quality of Web sites related to low back pain meet patients expectations? A systematic review]. / La qualité de l'information disponible sur Internet concernant la lombalgie est-elle adaptée aux attentes des patients? Revue systématique.

INTRODUCTION: An assessment of 36 back-pain-related French-language Web sites, miming a patient search strategy, was previously done by use of evidence-based items. Medical information quality was poor, as already noted about English-language Web sites. Thus, patients' expectations may exceed that provided by Web sites with simple medical information. OBJECTIVES: To study whether French-language Web sites related to low back pain meet patients expectations and to valid a rating scale including patients' expectations. MATERIALS AND METHOD: First we reviewed French-language Web sites with new keywords and medical gateways. Second, we systematically double assessed back pain-related Web sites with a health care professional and patient-centered scale. RESULTS: We found 30 additional Web sites not found with the previous search, 7 focusing on patient information. The rating scale is valid, and its use on a Web site sample leads to results different from those generated by an evidence-based medicine rating scale but close to a more global assessment. DISCUSSION: French-language Web sites related to low back pain do not meet patients' expectations. Patients participation in Web site assessment or construction could help to close the gap between the expectations of people with low back pain and information delivered by doctors.

[Camptocormia disclosing Parkinson's disease]. / Camptocormie révélatrice d'une maladie de Parkinson.

Camptocormia is characterised as an extreme bent-forward posture of the trunk that disappears in the recumbent position. On X-ray, trunk flexion appears without vertebral rotation as in scoliosis. The condition is a well-known complication of Parkinson's disease (PD) at the late stage. The authors present the case of a 77-year-old woman affected by severe camptocormia, which appeared and worsened in less than 6 months and hindered gait. Despite no signs of PD, neuro-imaging (DAT-Scan) showed an L-Dopa transducer decrease in putamens. A few weeks later, bradykinesia appeared and the clinical diagnosis of PD became more obvious. L-Dopa improved bradykinesia but did not change the bent-spine posture. A 1-year follow-up showed no other signs of PD other than bradykinesia, but the camptocormia was unchanged.

[Mitochondrial cardiomyopathy in an adult: a case history]. / Cardiomyopathie mitochondriale de l'adulte: à propos d'un cas.

We report an original case of mitochondrial cardiomyopathy discovered in a young woman during an episode of cardiac decompensation. The diagnosis was suspected from the echocardiographic appearances of granite-like heterogeneous hypertrophic cardiomyopathy. It was confirmed by endomyocardial biopsies. The clinical evolution was favourable with classical treatment. Mitochondrial cardiomyopathy is a rare cause of cardiomyopathy, generally observed in children, with multisystemic localisation. The pathophysiology and genetics are complex. Cardiac involvement is observed in 25% of cases, with the principal manifestation being hypertrophic cardiomyopathy. In the absence of any specific clinical or paraclinical signs, echocardiography and MRI are the techniques of choice for morphological evaluation. Diagnosis relies upon myocardial biopsy, which should be readily advocated in every unexplained case of cardiomyopathy in a young subject. The prognosis is poor and no specific treatment is available.

[Giant vascular tumour in an adult: tufted angioma or kaposiform hemangioendothelioma]. / Tumeur vasculaire géante de l'adulte: angiome en touffe ou hémangioendothéliome kaposiforme.

INTRODUCTION: Tufted angioma and kaposiform hemangioendothelioma are two rare benign but aggressive vascular tumours that occur mainly in children. OBSERVATION: A 72 year-old man consulted for a 50 cm wide vascular tumour of the right shoulder which was increasing for 10 years. On histological examination there were features of tufted angioma and kaposiform hemangioendothelioma. DISCUSSION: The tumour of this patient was atypical because of its big size never described before. The histological association of aspects which could correspond to tufted angioma and kaposiform hemangioendothelioma seems to confirm recent publications which support the hypothesis that these two tumours are two evolutive stages of one and only entity.