Developing tools to evaluate quality of care management for patients living with multiple sclerosis: An original French initiative.

INTRODUCTION: Assessing the quality of care management for patients with a chronic disease such as multiple sclerosis (MS) is a major challenge for healthcare systems around the world. It needs to be carried out using tools that are recognized by professionals and patients alike, and should concern practices, systems, and scientific data. No such tools are currently available in Europe. The purpose of the present study was to develop indicators to contribute to assess the quality of care management for patients with MS in France. METHODS: An expert panel comprising 25 professionals from well known teams across France selected the indicators on the basis of consensus. In accordance with the Rand/UCLA Appropriateness Method, each expert had to agree with the recommendations, and there had to be agreement among the experts. RESULTS: The expert panel selected 48 indicators representing seven domains of care management for patients with MS: physical and rehabilitation medicine, disease progression, access to care, magnetic resonance imaging (MRI) management, relapse management, management of disease-modifying treatments, and management of the symptoms of disability progression. Some of these quality indicators (notably pertaining to MRI management) had not previously been identified in the literature. CONCLUSION: These indicators may allow professionals to comprehensively assess and compare their practices and cooperation, thereby contributing to improve the quality of care management for patients with MS in France.

Evaluation of the quality of the care pathway for patients with multiple sclerosis in France: Results of an original study of a cohort of 700 patients.

INTRODUCTION: Evaluating the quality of the care pathway for patients with chronic diseases, such as multiple sclerosis (MS), is an important issue. Process indicators are a recognized method for evaluating professional practices. However, these tools have been little developed in the field of MS, and few data are available. The aim of this study was to describe, retrospectively, with validated indicators, the quality of the care pathway in a population-based cohort of 700 patients with the first manifestations of the disease occurring between January 1, 2000 and December 31, 2001 and during the first 10 years of disease. METHOD: This assessment was based on 48 indicators specific to MS. The information required for the calculation of each indicator was collected from the source files of the 700 patients of the cohort. RESULTS: Data for the 10 years of follow-up were collected for 80% of the patients. In total, 36 indicators were calculated. These results reveal that there is room for improvement, particularly in terms of the initial assessment, access to ophthalmological evaluation, employment, obtaining an evaluation of the need for rehabilitation and access to such care. CONCLUSION: The results of this survey provide access to unprecedented new data in France, that professionals and patients can appropriate to improve the targeting of actions, to improve the quality of care further for patients with MS in France. We propose to continue this process by submitting, for discussion, a targeted list of updated indicators relating to changes in guidelines, and in issues concerning the quality of patient management.

MSCopilot, a new multiple sclerosis self-assessment digital solution: results of a comparative study versus standard tests.

BACKGROUND AND PURPOSE: Assessing patients' disability in multiple sclerosis (MS) requires time-consuming batteries of hospital tests. MSCopilot is a software medical device for the self-assessment of patients with MS (PwMS), combining four tests: walking, dexterity, cognition and low contrast vision. The objective was to validate MSCopilot versus the Multiple Sclerosis Functional Composite (MSFC). METHODS: This multicentre, open-label, randomized, controlled, crossover study enrolled 141 PwMS and 76 healthy controls (HCs). All participants performed MSCopilot and MSFC tests at day 0. To assess reproducibility, 46 PwMS performed the same tests at day 30 ± 3. The primary end-point was the validation of MSCopilot versus MSFC for the identification of PwMS against HCs, quantified using the area under the curve (AUC). The main secondary end-point was the correlation of MSCopilot z-scores with MSFC z-scores. RESULTS: In all, 116 PwMS and 69 HCs were analysed. The primary end-point was achieved: MSCopilot performance was non-inferior to that of MSFC (AUC 0.92 and 0.89 respectively; P = 0.3). MSCopilot and MSFC discriminated PwMS and HCs with 81% and 76% sensitivity and 82% and 88% specificity respectively. Digital and standard test scores were highly correlated (r = 0.81; P < 0.001). The test-retest study demonstrated the good reproducibility of MSCopilot. CONCLUSION: This study confirms the reliability of MSCopilot and its usability in clinical practice for the monitoring of MS-related disability.

Assessing the impact of a patient education programme on pressure ulcer prevention in patients with spinal cord injuries.

OBJECTIVES: There is currently a low level of evidence for the impact of patient education on the management of patients with chronic neurological disease at risk of developing pressure ulcers. The objective of this study was to assess the impact of a patient education programme on pressure ulcer prevention in patients with chronic spinal cord injuries. MATERIALS AND METHODS: This study included adult patients with any spinal cord injury, regardless of the cause. Participants attended 2 group workshops focusing on pressure ulcer prevention. Various clinical data were gathered during an initial individual interview and at 3, 6 and 12 months, along with rating scale values from the Hospital Anxiety and Depression Scale, Rosenberg self-esteem scale, Schwarzer self-efficacy scale, a quality of life scale (SF-36) and the revised Skin Management Needs Assessment Checklist (Revised SMnac), which was used as the primary endpoint. RESULTS: Twenty patients were included in the study. The mean patient age was 52 years (SD: 9,8). Sixteen patients had traumatic spinal cord injuries, with a median injury duration of 234 months (IQR: 123-407). Seventy-five percent had had a pressure ulcer in the twelve months prior to the study. Patient education was shown to have a significant impact on skin management ability, with a highly significant increase in the overall revised SMnac score at 3 months. These results were stable over time, from 6 to 12 months. Six patients developed a pressure ulcer during the study (30%). CONCLUSION: This study supports the hypothesis that a therapeutic educational program conducted at the chronic phase in spinal cord injured individuals has an impact.

Analysis of the medical causes of death in cerebral palsy.

AIM: To investigate causes of death and age at death in cerebral palsy subjects compared with the general population. METHOD: Analysis of data supplied by the centre of epidemiology on the medical causes of death within the National Institute of Health and Medical Research in France was conducted. Three thousand and thirty-one death certificates indicating a diagnosis of cerebral palsy (ICD-10 code G80) were reported between 2000 and 2008. RESULTS: Median age at death was between 45-54 years and principal cause of death (24%) comprised the category "symptoms, signs, and abnormal results of clinical and laboratory tests, not classified elsewhere". Of these, 66% were related to the circulatory and respiratory systems. "Diseases of the respiratory system" were the second most common cause of death (19% compared with 6% in the French general population). The third most common cause of death was "diseases of the circulatory system" (15% compared with 29% in the French general population). While deaths caused by tumour pathologies in the general population are the most common cause of deaths, these represented only 7% of deaths in subjects with cerebral palsy. INTERPRETATION: These results concur with other published data, i.e. subjects with cerebral palsy die younger than the French general population, and the principal causes of death are respiratory and circulatory problems. This study emphasises the importance of access to epidemiological data about the French cerebral palsy population.

A French observational study of botulinum toxin use in the management of children with cerebral palsy: BOTULOSCOPE.

BACKGROUND: Dystonia and spasticity are common symptoms in children with Cerebral Palsy (CP), whose management is a challenge to overcome in order to enable the harmonized development of motor function during growth. AIM: To describe botulinum toxin A (BTX-A) use and efficacy as a treatment of focal spasticity in CP children in France. METHODS: This prospective observational study included 282 CP children mostly administered according to French standards with BTX-A in lower limbs. Realistic therapeutic objectives were set with parents and children together before treatment initiation and assessed using the Visual Analogue Scale (VAS). Child management was recorded and the efficacy of injections was assessed during a 12-month follow-up period by physicians (Modified Ashworth Scale, joint range of motion, Physician Rating Scale, Gillette Functional Assessment Questionnaire and Gross Motor Function Measure-66) and by patients/parents (Visual Analogue Scale). RESULTS: BTX-A treatment was administered in different muscle localizations at once and at doses higher than those recommended by the French Health Authorities. Children were treated in parallel by physiotherapy, casts and ortheses. Injections reduced spasticity and improved joint range of motion, gait pattern and movement capacity. Pain was reduced after injections. BTX-A administration was safe: no botulism-like case was reported. The log of injected children who were not included in the study suggested that a large population could benefit from BTX-A management. CONCLUSIONS: We showed here the major input of BTX-A injections in the management of spasticity in CP children. The results are in favor of the use of BTX-A as conservative safe and efficient treatment of spasticity in children, which enables functional improvement as well as pain relief.

Self care programs and multiple sclerosis: physical therapeutics treatment - literature review.

OBJECTIVE: To clarify the therapeutic education program impact with multiple sclerosis patients, literature review. Highlight contents and efficacy. METHOD: A non-systematic review on Medline, PubMed and Cochrane library databases from 1966 to 2010 using the following keywords: "multiple sclerosis", "self-care", "self-management" and specific symptoms keywords. Clinical trials and randomized clinical trials, as well as literature reviews published in English, French and German will be analyzed. RESULTS: Counseling is a part of the non-pharmacological management of chronic illnesses such as multiple sclerosis. Symptoms' diversity and the different clinical forms limit standardized programs of self-care management, applicable to patients. In the literature review, counseling programs have often low metrology. A behavior change with patients and medical staff could exist. To empower the patient, to reduce symptoms' impact and to improve treatment access are the aims of educational therapy. CONCLUSION: Therapeutic education program for multiple sclerosis patients could progress with their standardization and assessment, for each sign. To promote the educational therapy of multiple sclerosis patients, a specific training for medical staff, as specific financing are necessary.

Kienböck's disease and cerebral palsy case report.

INTRODUCTION: Pain is the main problem in patients suffering from cerebral palsy, particularly in adults. The upper limbs are affected in 25% of cases. Here, we report the case of a patient with Kienböck's disease. METHOD: Clinical case and literature review. A 28-year-old man suffering from dystonic quadriplegia consulted for progressively worsening pain in the right wrist. Kienböck's disease was diagnosed and conservative treatment with botulinum toxin in the flexor carpi radialis recommended. A good result was obtained with a decrease in pain. This result was still present two years later. DISCUSSION: Although few references are made to it in literature, Kienböck's disease in cerebral palsy is probably underestimated. Maintenance of the wrist in a permanent flexed position and muscular hypertonia may be risk factors. Knowledge of this particular clinical picture will enable it to be detected promptly and thus enable conservative treatment to be organised with a maximum chance of therapeutic success, preventing the need for surgery.

Vertebrobasilar ischemia after cervical spine manipulation: a case report.

INTRODUCTION: The most serious accidents after cervical spine manipulation are vertebrobasilar ischemia. Their incidence is underestimated. Their risk of apparition is lower if the contraindications are respected and if they are realised according to suitable practice. CASE REPORT: Mrs B, 39 years old, was an active smoker and had migraine for 10 years ago. One day, she presented an unusual headache associated with neck pain that was treated by a cervical spine manipulation. Seven hours after, she developed an alternate syndrome with a right sensory motor defect, a cerebellar syndrome, a pyramidal syndrome and a left defect of cranial nerves. The arteriography showed a thrombosis of the basilar trunk and a dissection of the left vertebral artery. A thrombolysis "in situ" was realized six hours and a half after the onset of the neurological defects. After eight months of rehabilitation, there were still a paralysis of the right upper limb, of the cranial nerves and a cerebellar syndrome but the patient was able to walk with two crutches and can eat by herself. DISCUSSION: Several risk factors were present in this case and there was also a major contraindication to manipulations: unusual acute occipital headache. Given the long period between the onset of neurological symptoms and the confirmation of the diagnosis, intravenous thrombolysis could not be done. Unfortunately, after eight months, important neurological sequels persisted. In order to avoid this type of accident after cervical manipulations, it is necessary to realize a strict medical examination and to implement the recommendations from the French society of manual and orthopaedic osteopathic medicine (Société française de médecine manuelle orthopédique et ostéopathique [SOFMMOO]).

[Intrasphincteric pharmacological treatments in neurourology]. / B. traitements pharmacologiques intra-sphinctériens en neuro-urologie.

Neurogenic detrusor-sphincter dyssynergia can be treated medically by intrasphincteric injection of lidocaine or botulinum toxin. The indications, practical modalities and results of these two molecules are described in the light of a review of the literature, which indicates that lidocaine injections alone are useless. The results of the various types of transperineal injection of botulinum toxin A with electromyographic or direct endoscopic guidance are described.

[Pain in adults with cerebral palsy]. / La douleur chez le sujet adulte infirme moteur cérébral.

INTRODUCTION: Pain is the main problem in patients with cerebral palsy. To improve the quality of life of people with cerebral palsy, a good knowledge of the clinical syndrome is necessary. METHOD: We reviewed the published data of the APIB study. We analysed data about pain, especially its correlation with age, motor handicap, and depressive syndrome. We also reviewed the literature to survey the pain syndrome in such a situation. RESULTS: A total of 562 patients responded to the APIB survey (mean age 36+/-14 years). Of these, 75% complained about physical pain. Pain was significantly associated with age, motor handicap, depressive syndrome, and sleep disorders. DISCUSSION: A study of the literature confirms our results on pain in cerebral palsy from the APIB survey. The main localisations of pain are the spine, and scapular and pelvic girdles. Pain syndromes of the upper and lower limbs are also frequent, with much due to overuse. Some rare causes include heterotopic ossifications. CONCLUSION: A good knowledge of the characteristics of the pain syndromes in people with cerebral palsy is important because of the great prevalence of this problem in this population. Better knowledge will help prevent the pain and improve quality of life.

Physical training and multiple sclerosis.

For many years, patients with multiple sclerosis (MS) were advised to avoid exercise because of the risk of increased neurological impairment. This article reviews the literature related to MS and physical exercise. Physical exercise depends on patients' physiological tolerance and response to exercise. MS patients can exhibit dysfunction of cardiovascular adjustment accompanied by respiratory involvement, which can alter aerobic capacity. These abnormalities tend to increase with the neurological impairment. Muscle weakness is the consequence of not only altered central motor drive but also disuse. Several studies have shown the benefits of physical training, with improvements in aerobic capacity, gait parameters and fatigue, and an influence on quality of life. Regular aerobic physical activity is necessary to maintain the benefit of physical training.

[Disabilities, access to medical care, and way of life of adults with cerebral palsy. APIB study: initial results]. / Handicaps, recours aux soins et conditions de vie des Adultes atteints de paralysie cérébrale infantile en Bretagne (APIB): premiers résultats.

UNLABELLED: Cerebral palsy is defined as a nonprogressive brain lesion that occurs during prenatal or perinatal development. The Breizh IMC health network objective is to improve the knowledge about the way of life and the health status of adults with cerebral palsy. OBJECTIVE: To describe disabilities, way of life and access to medical care for older teens and adults with cerebral palsy. METHOD: We conducted a descriptive study using health insurance data to contact patients. A questionnaire was addressed to people with cerebral palsy who were older than 16 years. Data were then compared to data of a general population sample. RESULTS: In total, 562 persons responded: 56% male and 44% female, mean age 36. Motor disability was more important with age (50% at age 20 and 70% after 60). A total of 66% of the sample? had cognitive impairment or mental retardation, 60% needed help when going outside, and 40% required assistance with eating, bathing and dressing. The level of education was variable. Health problems were more frequent in patients with cerebral palsy than in the general population and were progressive. Of the sample, 75% experienced pain and half felt depressed or lonely. Medication use was higher in the sample than in the general population. CONCLUSION: More study is needed to improve the knowledge of the health status of patients with cerebral palsy. This study highlights health problems that may be considered a basis for the development of care programs.

[Two cases of rehabilitation in Ehler-Danlos syndrome]. / Prise en en charge rééducative de la maladie d'Elhers-Danlos: à propos de deux cas.

PURPOSE: Ehlers-Danlos syndrome is rare and little known. It comprises a heterogeneous group of heritable connective tissue disorders characterized by articular hypermobility, skin extensibility and tissue fragility. Diagnosis may be difficult and often delayed. CASE REPORT: Here we describe 2 cases of women with Ehlers-Danlos syndrome. The first had scoliosis with back pain and joint hypermobility. The second presented with hypermobility and joint dislocation, which increased during pregnancy. In these 2 cases, rehabilitation management produced clinical improvement. DISCUSSION: Orthopaedic complications such joint pain, joint swelling, joint dislocation, back pain, with walking and hand function disability are the main problems in Ehlers-Danos syndrome. Surgery may be necessary to correct dislocated joints but is often not sufficient to resolve the handicap, and physical therapy has an important place in management. CONCLUSION: Ehlers-Danlos syndrome is an evolving disease that can lead to great impairment. Thus, physicians must be aware of this syndrome to offer the best management, with the appropriate use of orthotic devices, specific strengthening routines, education in proper body mechanics and assistive devices, to prevent joint dislocation and subluxations responsible for pain and handicap.

[Multiple sclerosis and physical activities]. / Activité sportive et sclérose en plaques.

INTRODUCTION: Multiple sclerosis (MS) is a neurological disease of the central nervous system that most often affects young people. It is characterized by various debilitating symptoms. The aim of this study was to assess physical activities in patients with MS and how these affect their quality of life. METHODS: Forty-five patients were questioned about their physical activities. The following were taken into account: the characteristics of MS within each patient, the level of disease severity (EDSS score) and the quality of life (on the SEP-59 questionnaire). RESULTS: Fifteen patients had to give up a physical activity because of their illness but more so due to ataxia, fatigue and muscular weakness. Fifteen patients take part in a physical activity, the principal motivation being for their personal well-being. The mean EDSS was 4.2. Answers on the SEP-59 revealed found that patients with MS who took part in a physical activity had significantly better well-being in general. DISCUSSION AND CONCLUSION: Physical activities seem to improve the quality of life of patients with MS who, in general, already have a poor quality of life in relation to the general population. Knowing that muscular function is improved through exercise in patients with an EDSS less than 6, it would seem advantageous for them to take part in a regular physical activity.

Effects of pregnancy and childbirth on the incidence of urinary disorders in multiple sclerosis.

OBJECTIVE: Women with multiple sclerosis frequently pose questions to physicians regarding the potential negative impact of pregnancy, especially on urinary disorders. About 50 to 80% of patients suffer from urinary disorders during the course of the disease. Trauma to the pelvic floor and the urethral sphincter during vaginal delivery may lead to the development of stress urinary incontinence. The purpose of this work was to study the consequences of pregnancy and childbirth on urinary problems. STUDY DESIGN: An inception cohort of 368 consecutive women suffering from multiple sclerosis (MS), according to the Poser criteria, were included in the study between June 1999 and June 2000. For each patient a full urological and obstetrical record was obtained. RESULTS: Two hundred and seventy-three women (74%) had had at least one pregnancy. The parous women were older at the time of the study (mean age: 45.5 years vs 35.5 years), and were older at MS onset (mean age: 32.8 years vs 25.7 years). The nulliparous women were more rapidly disabled, with a shorter time to reach an EDSS score of 3 from MS onset (mean time of 5.9 years versus 8.2 years in parous women). In parous women, 259 (95%) had had at least one vaginal delivery and 14 (5%) had had a caesarean only. Delivery modalities had no influence on urinary disorder frequency or the type of problems. CONCLUSION: On the basis of these data, there is actually no clear argument for systematically performing caesarean section in MS women. Urinary disorders in these women were mostly linked to the duration and the severity of the disease but not to pregnancy or delivery modalities. From our point of view, caesarean section must be decided only on classic obstetrical criteria as for healthy women independently of multiple sclerosis. However our data were open and retrospective.

Placebo controlled, randomised, double blind study of the effects of botulinum A toxin on detrusor sphincter dyssynergia in multiple sclerosis patients.

OBJECTIVE: The purpose of the study was to evaluate the efficacy and safety of botulinum A toxin in the treatment of detrusor sphincter dyssynergia in multiple sclerosis patients. METHODS: This was a multicentre, placebo controlled, randomised, double blind study. Patients with chronic urinary retention were included if they had post-voiding residual urine volume between 100 and 500 ml. They received a single transperineal injection of either botulinum A toxin (100 U Allergan) or placebo in the sphincter and also 5 mg slow release alfuzosin bid over 4 months. Main endpoint was post-voiding residual urine volume assessed 1 month after injection. Follow up duration was 4 months. Statistical analysis was performed using a sequential method, the triangular test. RESULTS: The study was stopped after the fourth analysis (86 patients had been included: placebo: 41, botulinum A toxin: 45). At inclusion, there was no significant difference between groups whichever variable was considered. Mean (standard deviation) post-voiding residual urine volume was 217 (96) and 220 (99) ml in placebo and botulinum A toxin groups, respectively. One month later, post-voiding residual urine volume was 206 (145) and 186 (158) ml (p = 0.45) in placebo and botulinum A toxin groups, respectively. However, compared to placebo, botulinum A toxin significantly increased voiding volume (+54%, p = 0.02) and reduced pre-micturition (-29%, p = 0.02) and maximal (-21%, p = 0.02) detrusor pressures. Other secondary urodynamic endpoints and tolerance were similar in the two groups. CONCLUSIONS: In multiple sclerosis patients with detrusor sphincter dyssynergia, a single injection of botulinum A toxin (100 U Allergan) does not decrease post-voiding residual urine volume.

[Home care and quality of life three years after stroke]. / Maintien à domicile et qualité de vie à distance d'un accident vasculaire cérébral.

INTRODUCTION: Stroke is the most disabling chronic disease among adults. The assessment of quality of life is of great importance to improve the management of patients with stroke sequelae returning to the community. METHOD: A total of 93 patients and their referring clinicians were contacted. Quality of life of patients three years after stroke was assessed by use of the Short-Form Health Survey (SF-36). RESULTS: Thirteen patients died during this period. Fifty participated in the study. The average age was 66.8 years. The patients who died were older and had an initial greater impairment after stroke than survivors. In most of the cases, a life plan, built during the inpatient rehabilitation period, could be achieved. Just six patients had to change their way of life during the three years after stroke. A total of 44% of the patients needed human assistance for basic daily living activities. Patients with stroke had lower scores on the SF-36 than their healthy peers. All subscale scores differed for patients with stroke, as compared with health peers. Initial disability, the occurrence of falls, age and urinary incontinence at the time of the study indicated poor prognosis. DISCUSSION: Our results are close to those previously published: a great number of people who survive stroke live with sequelae that requires assistance in the home and have decreased quality of life. These results stress the importance of follow-up by a rehabilitation team to prevent altered quality of life, with a special care to urinary incontinence and falls, especially in old people with abundant sequelae of stroke.

[Charcot-Marie-Tooth disease associated with syringomyelia revealed by urinary disorders]. / Association de maladie de Charcot-Marie-Tooth et de syringomyélie révélée par la survenue de troubles urinaires.

PURPOSE: Charcot-Marie-Tooth (CMT) disease is characterized by genetic and clinical heterogeneity. The occurrence of urinary disorders is unusual and requires further investigation. CMT disease and syringomyelia are not usually associated. Moreover, primary urinary signs in syringomyelia are not frequent. CASE REPORT: An 11-year-old girl with CMT disease 1A duplication presented with motor deficit of the leg associated with urinary disorders. Urodynamic study revealed neurogenic detrusor overactivity, and magnetic resonance imaging confirmed syringomyelia extending from T11 to an L1 located conus. DISCUSSION: Review of the literature confirmed the possibility of primary urinary signs in syringomyelia, with only one case of CMT and urinary signs described. In this case, the diagnosis was radicullar and medullar compression by a hypertrophic nerve formation called an "onion bulb" reflecting the demyelinisation and remyelinisation cycle. CONCLUSION: Urinary signs in CMT disease are exceptional and require investigation into the possibility of a central lesion.