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ObjectiveTo obtain insight into experiences of patients with a neuromuscular disease and chronic fatigue and their healthcare professionals regarding content and delivery of a multidisciplinary outpatient self-management group programme to improve social participation. This will inform future implementation.DesignA mixed method study alongside a randomised controlled trial.SettingUniversity hospital, rehabilitation centre and community health centre.Participants29 patients with a neuromuscular disease and chronic fatigue and 13 healthcare professionals participated in this mixed methods study.InterventionMultidisciplinary group programme, called Energetic, consisted of a 4 months intervention with weekly meetings and covered four modules: (1) individually tailored aerobic exercise training; (2) education about aerobic exercise; (3) self-management training in applying energy conservation strategies and (4) implementation and relapse prevention in daily life.Main measuresQuantitative data were collected by a questionnaire measuring patients’ (n=25, all completed the programme) satisfaction with the perceived results, content and delivery of the programme. Qualitative data were collected by individual and focus group interviews to gain insight in the experiences of patients (n=18), next of kin (n=2) and healthcare professionals (n=13) with facilitators and barriers to programme implementation.ResultsPatients were satisfied with the number and length of the sessions, the different modules and the therapists. Analysis of the interviews led to five themes: (1) the combination of modules makes a complete picture, (2) the programme is physically and mentally intensive, (3) the group setting is valuable, (4) small variations in delivery occur in different settings, (5) therapists are coaches. Suggestions for programme improvement include a combination of face to face and e-health, enhancement of therapists’ skills in guiding group interventions and inclusion of more booster sessions to evaluate and maintain self-management competencies.ConclusionsThe Energetic programme could be implemented in different healthcare settings and group settings, and a combination of modules proved to be a facilitator for improving self-management.Trial registration numberNCT02208687.

Item does not contain fulltext The aim of this research was to study impairments, activity limitations and participation restrictions due to upper limb involvement in people with four different types of neuromuscular disorders (NMD) - FacioScapuloHumeral Dystrophy (FSHD), Limb-Girdle Muscular Dystrophy (LGMD), Spinal Muscular Atrophy (SMA) and Duchenne Muscular Dystrophy (DMD) - and to investigate whether common or different profiles could be identified. Total of 267 respondents with NMD from the Netherlands answered a set of questionnaires covering upper limb impairments (pain and stiffness), activity limitations and participation restrictions. Pain was most often reported by persons with FSHD. Problems in the FSHD group were mainly characterized by relatively high scores for pain and stiffness and low scores for activity limitations. People with LGMD reported also low scores for activity limitations. Conversely, people with SMA and DMD experienced in general relatively low scores for pain and stiffness and high scores for activity limitations. Although people with FSHD and LGMD had relatively few activity limitations, all NMD groups experienced restrictions when participating in social activities. Our results indicate specific profiles of upper limb function in different types of NMD. While the profile observed in persons with FSHD seems to reflect overuse, the profile seen in persons with DMD and SMA is suspicious of disuse, each requiring a specific rehabilitation strategy.

Background: To retard shortening of finger flexors in patients with Duchenne muscular dystrophy (DMD), hand orthoses are prescribed. However, many patients do not wear the orthoses regularly. To optimize orthotic interventions, we need insight into the factors influencing compliance. Objective: To evaluate the compliance regarding hand orthoses in an adult DMD population and to explore experiences and perceptions of DMD patients wearing orthoses, and of their caregivers. Methods: Mixed methods observational study, combining quantitative and qualitative data from medical charts combined with qualitative semi-structured interviews using a constant comparative method and a short validated questionnaire (D-QUEST). Results: 65 medical charts were analyzed. 48 patients were assessed as needing hand orthoses, of whom 37.5 % were compliant. Qualitative data analyses revealed (1) motivation: preservation of hand function; (2) barriers: discomfort and impediments; (3) facilitators: good fit and personalized wearing schedule; (4) fitting process: satisfactory, but patients do not readily seek help when barriers appear. Conclusions: Patients are motivated to wear hand orthoses, but often discontinue use because of orthosis-and disease-specific barriers. The identification of these barriers leads to practical and feasible recommendations concerning the orthoses and the fitting process, such as less rigid material, preservation of some function while wearing the orthoses, and fixed evaluation points. The findings were confirmed by the D-QUEST.

Contains fulltext : 173234.pdf (Publisher’s version ) (Closed access) Purpose To investigate the upper extremity (UE) at the level of impairments and related activity limitations and participation restrictions in people with facioscapulohumeral muscular dystrophy (FSHD). Methods The study was conducted using web-based questionnaires that were distributed amongst people with FSHD in the Netherlands. Eighty-eight respondents started the survey, and 71 completed it. The questionnaires covered the following dimensions: Function, Activity and Participation of the International Classification of Functioning Disability and Health. Results More than 40% of the respondents experienced pain in one arm or both the arms. Increased pain and stiffness scores and longer disease duration were associated with increased limitation scores. For basic activities, lifting the arm above shoulder-level was most frequently reported as most limited, coherent with the clinical picture of FSHD. Among the respondents, 50% indicated restrictions at school, 78% indicated restrictions at work and more than 80% indicated restrictions whilst participating in sports, hobbies, household activities and romantic relationships. Conclusions This study has shown that alongside the well-known problem of lifting the arms above shoulder-level, UE activities below shoulder height during vocational and occupational activities are also problematic in patients with FSHD. Alongside disease duration, pain and stiffness are associated with UE activity limitations. Implications for Rehabilitation Attention is needed for pain and experienced stiffness in the upper extremity as it is frequently present in patients with FSHD. Rehabilitation professionals need to be aware that patients with FSHD not only experience problems with activities above shoulder height, but also with activities below shoulder height. At least 50% of the patients with FSHD experience restrictions in participation as a result of limitations in their UE.

ObjectiveTo investigate the effectiveness of Energetic, a self-management group program combining aerobic training, energy conservation management, and relapse prevention to improve social participation in patients with neuromuscular disease (NMD) and chronic fatigue.MethodsIn this multicenter, assessor-blinded, 2-armed randomized controlled trial with repeated measurements, 53 patients with various types of NMD and chronic fatigue were randomly allocated to Energetic, a 4-month group intervention, or to usual care. The primary endpoint was social participation assessed with the Canadian Occupational Performance Measure (COPM) performance scale immediately postintervention. Secondary outcomes included COPM satisfaction scale, 6-Minute Walk Test (6MWT), and Checklist Individual Strength–subscale fatigue. Participants were followed for 11 months postintervention. Data were analyzed with linear models that account for repeated measurements.ResultsDirectly after intervention, the mean group difference for COPM-performance was 1.7 (95% confidence interval [CI] 1.0–2.4; p < 0.0001) in favor of the intervention group (n = 29), adjusted for baseline, sex, diagnosis, and work status. This effect was retained at 11 months follow-up (0.9; 95% CI 0.0–1.7; p = 0.049). The COPM satisfaction scale and 6MWT improved more in the intervention group compared to usual care. After 3 and 11 months follow-up, most beneficial effects on social participation and functional endurance were retained.ConclusionEnergetic led to sustainable improvements in social participation and functional endurance compared to usual care in patients with NMD and chronic fatigue.Clinicaltrials.gov identifierNCT02208687.Classification of evidenceThis study provides Class III evidence that a combination of aerobic training, energy conservation management, and relapse prevention improves social participation in patients with NMD and chronic fatigue.

There is lack of knowledge, evidence, and guidelines for rehabilitation interventions for persons with neuralgic amyotrophy (NA) or brachial plexus pathology. A first pilot study, evaluating the effect of an integrated rehabilitation programme, showed improvements in activity and participation levels.To gain insight, from the perspective of patients and therapists, into the critical ingredients of the programme, that contributed to improvements in activity and participation.A qualitative study using semi-structured interviews with eight patients and five therapists (three occupational therapists and two physical therapists). Participants were asked to identify and describe factors regarding the rehabilitation that they perceived as positive and aspects of the programme that could be improved. Data were analysed using a constant comparative approach.Patients reported (1) Time to diagnose: "Finally I'm in the right place;" (2) Awareness: "They gave me a mirror;" (3) Partnership: "There was real contact with the therapists; we made decisions together;" (4) Close collaboration: "Overlapping scopes of practice; doing the same from a different perspective;" and finally (5) Self-management: "Now I can do it myself." Therapists reported (1) "Patients knowledge and understanding is critical to success;" (2) "Activate problem solving and decision making;" (3) "Personalize your therapy; it's more than just giving exercises and information;" (4) "Constant consultation within the team; consistency in messages and approach;" and (5)" Ultimately the patient is in charge."The critical ingredients, correspond well with each other and include a person-centred approach, education, support in problem solving and decision making and an integrated team approach. These ingredients provided the patients with confidence to take responsibility to manage their everyday lives, the ultimate goal of the programme. Implications for rehabilitation Both patients and therapists believe that the ability to self-manage and take control should be the outcome of high quality integrated rehabilitation programmes for patients with neuralgic amyotrophy and/or other brachial plexus injuries. A person-centred, collaborative, and integrated team approach, among all members of the team, are critical components of care delivery in personalised interventions. Critical programme ingredients are knowledge and education of both the patient and therapists; partnership between patient-therapist and within the team; patient activation and self-reflection; and personalised care. Patients recommend more options for personalisation of the intensity and duration of rehabilitation, the possibility to consult a psychologist and peer support within a group setting.

Study Design Longitudinal clinical measurement. Introduction Sensory alterations in the hand can present as both decreased sensation or numbness, and hyperesthesia, including mechanical allodynia and cold intolerance. However, few patient-reported outcomes have been developed and validated for evaluation, particularly for increased sensitivity. The Radboud Evaluation of Sensitivity was developed in the Netherlands for patient-reported evaluation of hand sensitivity in complex regional pain syndrome. Purpose of the Study The purpose of this study was to translate into English and culturally validate the Radboud Evaluation of Sensitivity for the North American context. Methods Forward and backward translation, followed by a psychometric evaluation of the synthesized version of the translated tool, was undertaken in a heterogeneous group of persons after hand injury, including nerve injuries, hand trauma, and complex regional pain syndrome. Results Thirty-six persons completed test-retest reliability testing, yielding an intraclass correlation coefficient of 0.92 (95% CI 0.85 to 0.96) for single measures. Internal consistency was also high at α = 0.96 in a larger sample (n = 56). Although some support for construct validity was generated, several validity hypotheses were not confirmed. Of interest, there appeared to be significant differences in the scores between persons with hypoesthesia as compared with those with hyperesthesia. Conclusions The Radboud Evaluation of Sensitivity, English version appears to be a reliable tool for the self-reported evaluation of sensory alterations in the hand, including both hypoesthesia and hyperesthesia. More research is needed to add to the extent of and confidence in the validity and responsiveness of this assessment. Level of Evidence Level II.

A systematic review was conducted to assess the evidence base of occupational therapy for adults with neuromuscular diseases. A comprehensive search for controlled and uncontrolled studies was performed in the Cochrane, MEDLINE, CINHAL, and EMBASE databases. After screening 3,534 titles and abstracts, six articles were retrieved for more detailed evaluation. Only two studies met all of the predefined criteria and were subjected to methodological assessment. These studies provide indications for the efficacy of hand training in muscle diseases. There is a lack of scientific evidence regarding the question of whether patients with neuromuscular diseases are better capable of participating in meaningful activities and roles through occupational therapy. Recommendations for future research are given.

Background and Purpose— Occupational therapy (OT) is an important aspect of stroke rehabilitation. The objective of this study was to determine from the available literature whether OT interventions improve outcome for stroke patients. Methods— An extensive search in MEDLINE, CINAHL, EMBASE, AMED, and SCISEARCH was performed. Studies with controlled and uncontrolled designs were included. Seven intervention categories were distinguished and separately analyzed. If a quantitative approach (meta-analysis) of data analysis was not appropriate, a qualitative approach (best-evidence synthesis), based on the type of design, methodological quality, and significant findings of outcome and/or process measures, was performed. Results— Thirty-two studies were included in this review, of which 18 were randomized controlled trials. Ten randomized controlled trials had a high methodological quality. For the comprehensive OT intervention, the pooled standardized mean difference for primary activities of daily living (ADL) (0.46; CI, 0.04 to 0.88), extended ADL (0.32; CI, 0.00 to 0.64), and social participation (0.33; CI, 0.03 to 0.62) favored treatment. For the training of skills intervention, some evidence for improvement in primary ADL was found. Insufficient evidence was found to indicate that the provision of splints is effective in decreasing muscle tone. Conclusions— This review identified small but significant effect sizes for the efficacy of comprehensive OT on primary ADL, extended ADL, and social participation. These results correspond to the outcome of a systematic review of intensified rehabilitation for stroke patients. The amount of evidence with respect to specific interventions, however, is limited. More research is needed to enable evidence-based OT for stroke patients.

Item does not contain fulltext PURPOSE: The aims of this review were (1) to provide insight into the natural course of upper-extremity (UE) impairments and UE activity limitations associated with facioscapulohumeral dystrophy (FSHD) and limb-girdle muscular dystrophies (LGMD), and (2) to provide an overview of outcome measures used to evaluate UE function and activity in patients with FSHD and LGMD. METHODS: Scientific literature databases (PubMed, MEDLINE, EMBASE, CINAHL and Cochrane) were searched for relevant publications. INCLUSION CRITERIA: (1) studies that included persons with a diagnosis of FSHD or LGMD; and (2) studies that reported the natural course of the UE functions and/or activity with outcome measures at these levels. RESULTS: 247 publications were screened, of which 16 fulfilled the selection criteria. Most studies used manual muscle testing (MMT) to evaluate UE function and the Brooke Scale to evaluate UE mobility activities. The clinical picture of UE impairments and limitations of UE activities in FSHD and LGMD patients was highly variable. In general, FSHD and LGMD patients experience difficulty elevating their upper extremities and the execution of tasks takes considerably longer time. CONCLUSIONS: The clinical course of UE impairments and activity limitations associated with FSHD and LGMD is difficult to predict due to its high variability. Although measures like MMT and the Brooke Scale are often used, there is a lack of more specific outcome measures to assess UE function and UE capacity and performance in daily life. Measures such as 3D motion analysis and electromyography (EMG) recordings are recommended to provide additional insight in UE function. Questionnaires like the Abilhand are recommended to assess UE capacity and accelerometry to assess UE performance in daily life. IMPLICATIONS FOR REHABILITATION: There is a need for specific outcome measures on the level of UE activity. Both the level of capacity and performance should be assessed. Possible outcome measures include 3D motion analysis to assess UE function, questionnaires like the Abilhand to assess UE capacity and accelerometry to assess performance of UE activities in daily life.

Contains fulltext : 155131.pdf (Publisher’s version ) (Open Access) BACKGROUND: Chronic fatigue is present in more than 60% of the patients with a neuromuscular disease and can be their most disabling symptom. In combination with other impairments, fatigue often results in low levels of physical activity and decreased social participation, leading to high societal costs. 'Energetic' is a self-management group program aimed at improving social participation, physical endurance and alleviating fatigue in these patients. The primary aim of this study is to evaluate the effectiveness and cost-effectiveness of the Energetic program. METHODS/DESIGN: A multicentered, assessor-blinded, two-armed randomized controlled trial is conducted with evaluations at inclusion and four, seven and fifteen months later. The study includes patients with a neuromuscular disease and chronic fatigue and, when present, their caregivers. The participants are randomized (ratio 1:1) to either an intervention group, receiving the Energetic program, or a control group, receiving usual care (i.e., no specific intervention). The Energetic program covers four months and includes four modules: 1) individually tailored aerobic exercise training; 2) education about aerobic exercise; 3) self-management training in applying energy conservation strategies; and 4) implementation and relapse prevention in daily life. Two months after cessation of the program a booster session is provided. The primary outcome is the perceived performance score of the Canadian Occupational Performance Measure (COPM). Secondary outcomes include the COPM-satisfaction score, and measures of fatigue, physical endurance, activity engagement, mood, and self-efficacy. Caregiver burden is also evaluated as a secondary outcome. Health-related quality of life and medical and societal costs are assessed to estimate cost-effectiveness of the program. DISCUSSION: The Energetic study is the first randomized controlled trial to evaluate the effectiveness and cost-effectiveness of a combined physical and self-management group training program for improving social participation, physical endurance and alleviating fatigue in patients with neuromuscular diseases. It will generate new insights in (cost-)effective rehabilitation strategies for these incurable conditions. TRIAL REGISTRATION: Clinicaltrials.gov NCT02208687 .

Objective: To compare the kinematics and muscle activity of subjects with facioscapulohumeral dystrophy (FSHD) and healthy control subjects during the performance of standardized upper extremity tasks.Design: Exploratory case-control study.Setting: A movement laboratory.Participants: Subjects (N=19) with FSHD (n=11) and healthy control subjects (n=8) were measured.Interventions: Not applicable.Main Outcome Measures: Kinematic data were recorded using a 3-dimensional motion capturing system. Muscle activities, recorded using electromyography, were obtained from 6 superficial muscles around the glenohumeral joint. Shoulder elevation and elbow flexion angles, and maximum electromyographic activity during the movements as a percentage of maximum voluntary contraction (MVC) were calculated.Results: Kinematic differences between the FSHD group and the healthy control group were found in the shoulder elevation angle during single shoulder movements and both reaching tasks. In general, subjects with FSHD had higher percentages of muscle activation. The median activity of the trapezius was close to the MVC activity during the single shoulder movements. Moreover, deltoid and pectoralis muscles were also highly active.Conclusions: Higher activation of the trapezius in subjects with FSHD indicates a mechanism that could help relieve impaired shoulder muscles during arm elevation around shoulder height. Compared with healthy subjects, persons with FSHD activated their shoulder muscles to a greater extent during movements that required arm elevation. (c) 2014 by the American Congress of Rehabilitation Medicine

Item does not contain fulltext OBJECTIVE: To develop recommendations regarding outcome measures and topics to be addressed in rehabilitation for persons with neuralgic amyotrophy (NA), this study explored which functions and activities are related to persisting pain in NA and which questionnaires best capture these factors. DESIGN: A questionnaire-based survey from 2 cross-sectional cohorts, one of patients visiting the neurology outpatient clinic and a cohort seen at a multidisciplinary plexus clinic. SETTING: Two tertiary referral clinics based in the Department of Neurology and Rehabilitation from a university medical center provided the data. PARTICIPANTS: A referred sample of patients (N=248) with either idiopathic or hereditary NA who fulfilled the criteria for this disorder, in whom the last episode of NA had been at least 6 months ago and included brachial plexus involvement. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: Two custom clinical screening questionnaires were used as well as the Shoulder Rating Questionnaire-Dutch Language Version, the Shoulder Pain and Disability Index (SPADI), the Shoulder Disability Questionnaire (SDQ), and Overall Disability Sum Score. RESULTS: The survey confirms the high prevalence of persisting pain and impairments. More than half of the patients were restricted by pain, while in those without pain 60% experienced residual paresis. Correlations show an intimate relation between pain, scapular instability, problems with overhead activities, and increased fatigability. A standard physical therapy approach was ineffective or aggravated symptoms in more than 50%. CONCLUSIONS: Pain and fatigue are strongly correlated to persisting scapular instability and increased fatigability of the affected muscles in NA. Our results suggest that an integrated rehabilitation approach is needed in which all of these factors are addressed. We further recommend using the SPADI and SDQ in future studies to evaluate the natural course and treatment effects in NA.

Item does not contain fulltext BACKGROUND: Neuralgic Amyotrophy (NA) is characterized by neuropathic pain, subsequent patchy paresis and possible sensory loss in the upper extremity. Many patients experience difficulties in performing activities of daily life and are unable to resume work. We developed a combined physical- and occupational therapy program for patients recovering from NA. OBJECTIVE: Evaluation of the effectiveness of a multidisciplinary intervention program for patients with subacute NA. METHODS: We performed a within subject proof-of-principle pilot study in eight patients with subacute NA. Patients followed 8 hours of physical and 8 hours of occupational therapy spread over a 16-week period. Primary outcome measures: The Canadian Occupational Performance Measure (COPM) and Shoulder Rating Questionnaire (SRQ). Secondary outcome measure: Disability of Arm Shoulder and Hand (DASH). RESULTS: Improvements (mean (95% CI)) were found in the performance and satisfaction scores of the COPM +2.3 (0.9-3.7) and +1.4 (0.4-2.4) points, respectively and the SRQ +14.8 (7.4-22.0) points. The majority of patients (6 out of 8) also demonstrated improvements in the DASH. CONCLUSION: The proposed physical and occupational therapy program, may be effective for patients with subacute NA, as demonstrated by improvements in activity, performance and participation.

Minis MA, Cup EH, Heerkens YF, Engels JA, van Engelen BG, Oostendorp RA. Exploring employment in consultation reports of patients with neuromuscular diseases. Arch Phys Med Rehabil 2012;93:2276-80. Objectives: To explore consultation reports for patient and employment characteristics and recommendations on employ- ment regarding patients with neuromuscular diseases (NMDs). Design: Retrospective study of multidisciplinary reports. Setting: An outpatient neuromuscular clinic at a university hospital. Participants: Reports (N102) of patients with NMDs. Interventions: Based on one-off consultations by occupational therapists, physical therapists, and speech therapists and a multidisci- plinary meeting, recommendations were developed regarding therapy content and volume in primary care or rehabilitation settings. Main Outcome Measures: A checklist has been developed to examine employment characteristics. A general questionnaire has been used including demographic variables and data on employment. Results: Of the 102 reports available, 86 were included for analysis. Sixty-nine reports contained information on employ- ment. Thirty-seven patients (43%) with NMD were employed, most in white-collar or moderately strenuous jobs. Of the 37 employed patients, 28 (76%) worked using adaptations. Thirty- two (87%) had employment problems; of these, 15 (40%) needed improvement in 1 or more environmental factors. Twenty patients (54%) needed advice regarding participation in employment, of whom 19 were referred to primary care or rehabilitation settings for treatment to enhance employment participation. Conclusions: Eighty percent of the included consultation re- ports contained information on employment. Less than half the patients with NMD were employed, most in office-related jobs, using some kind of adaptations. Nineteen of 20 patients who agreed to recommendations regarding therapy were adequately referred by occupational therapists and physical therapists for treatment of employment problems.

Purpose: A systematic review was conducted to summarize and evaluate the literature on the effectiveness of speech pathology interventions in adults with neuromuscular diseases. Method: Databases searched included the Cochrane Database of Systematic Reviews, the Cochrane Central Register of Controlled Trials, MEDLINE, CINAHL, EMBASE, PsycINFO and PubMed. A total of 1,772 articles were independently screened on title and abstract by 2 reviewers. Results: No randomized controlled trials or clinical controlled trials were found. Four other designs were included. Only one study on oculopharyngeal muscle dystrophy (OPMD) appeared to have sufficient methodological quality. There is evidence indicating that correction of head position in patients with OPMD improves swallowing efficiency (level III evidence). Conclusion: Despite 1,772 studies, there is only evidence of level III regarding the effectiveness of speech pathology interventions in patients with OPMD. Recommendations for future research are given.

Contains fulltext : 81577.pdf (Publisher’s version ) (Closed access) BACKGROUND AND PURPOSE: To report the predictive validity of the perceived limitations in activities and need questionnaire (PLAN-Q), a screening instrument to support neurologists to select patients with neuromuscular disorders (NMD) for referral for a one-off consultation by occupational therapist (OT), physical therapist (PT) and speech therapist (ST). METHODS: In a cross-sectional validation study, 102 patients with various NMD participated. Patients received a one-off consultation by an expert OT, PT and ST and filled out the PLAN-Q. Therapists rated the appropriateness of the one-off consultations based on need, available treatment and patient's motivation. Receiver Operation Characteristic analysis and multivariate logistic regression analysis were used to obtain a PLAN-Q based prediction model for the appropriateness of the one-off consultations. RESULTS: Probability for a one-off OT consultation increased from 64% to 78% (95% CI: 69-85%). Prior test probability for a one-off ST consultation increased from 44% to 61% (95% CI: 48-73%). Prior test probability for one-off PT consultation could not be increased. CONCLUSION: Screening patients with NMD using the PLAN-Q may assist neurologists in selecting the appropriate patients for a one-off consultation by OT and ST. Unlike our expectations the screening did not guide referral for a one-off consultation by PT.

Contains fulltext : 53667.pdf (Publisher’s version ) (Closed access) PURPOSE To compare the volume of occupational therapy (OT), physical therapy (PT) and speech therapy (ST) as currently received by patients with neuromuscular diseases with the volume of OT, PT and ST recommended by a multidisciplinary team. METHOD: The use of OT, PT and ST was studied retrospectively and prospectively in a reference group (n = 106) receiving usual care and in an intervention group (n = 102) receiving advice based on multidisciplinary assessments. A cost analysis was made and the implementation of the advice was evaluated at 6 months. INTERVENTION: Multidisciplinary assessments consisted of a single consultation by OT, PT and ST each, followed by a multidisciplinary meeting and integrated advice. OUTCOME VARIABLES: Volume (frequency times duration) of therapy, relative over- and underuse of therapy and costs of therapy and intervention. RESULTS: Compared to the multidisciplinary advice, there was 40% underuse of OT among patients with neuromuscular disease. For PT, there was 32% overuse and 22% underuse; for ST, there was neither over- nor underuse. Some 40% of patients received once-only advice regarding ST compared to 27% regarding OT and 19% regarding PT. The costs of the multidisciplinary advice were estimated at euro245 per patient. If fully implemented, our multidisciplinary approach would result in a mean cost savings of euro85.20 per patient. The recommended therapy had, however, been implemented only partially at 6 months follow-up. CONCLUSIONS: Some patients with a neuromuscular disease do not receive any form of allied healthcare, whereas they should. Among patients with neuromuscular disease who do receive some form of allied healthcare, quite a few receive these treatments for too long periods of time. Ways need to be developed to improve implementation of the multidisciplinary advice and to obtain a more favourable balance between its costs and benefits.

Cup EH, Pieterse AJ, ten Broek-Pastoor JM, Munneke M, van Engelen BG, Hendricks HT, van der Wilt GJ, Oostendorp RA. Exercise therapy and other types of physical therapy for patients with neuromuscular diseases: a systematic review. Arch Phys Med Rehabil 2007;88:1452-64. Objective: To summarize and critically appraise the avail- able evidence on exercise therapy and other types of physical therapies for patients with neuromuscular diseases (NMD). Data Sources: Cochrane Central Register of Controlled Trials and Cochrane Database of Systematic Reviews, Med- line, CINAHL, EMBASE (Rehabilitation and Physical Medi- cine), and reference lists of reviews and articles. Study Selection: Randomized clinical trials (RCTs), con- trolled clinical trials (CCTs), and other designs were included. Study participants had to have any of the following types of NMD: motoneuron diseases, disorders of the motor nerve roots or peripheral nerves, neuromuscular transmission disorders, or muscle diseases. All types of exercise therapy and other phys- ical therapy modalities were included. Outcome measures had to be at the level of body functions, activities, or participation according to the definitions of the International Classification of Functioning, Disability and Health (ICF). Data Extraction: Two reviewers independently decided on inclusion or exclusion of articles and rated the methodologic quality of the studies included. All RCTs, CCTs, and other designs only if of sufficient methodologic quality were in- cluded in a best evidence synthesis. A level of evidence was attributed for each subgroup of NMD and each type of intervention. Data Synthesis: Initially 58 studies were included: 12 RCTs, 5 CCTs, and 41 other designs. After methodologic assessment, 19 other designs were excluded from further anal- ysis. There is level II evidence ("likely to be effective") for strengthening exercises in combination with aerobic exercises for patients with muscle disorders. Level III evidence ("indi- cations of effectiveness") was found for aerobic exercises in patients with muscle disorders and for the combination of muscle strengthening and aerobic exercises in a heterogeneous group of muscle disorders. Finally, there is level III evidence for breathing exercises for patients with myasthenia gravis and for patients with myotonic muscular dystrophy. Adverse ef- fects of exercise therapy were negligible. Conclusions: The available evidence is limited, but relevant for clinicians. Future studies should be preferably multicen- tered, and use an international classification of the variables of exercise therapy and an ICF core set for NMD in order to improve comparability of results.

Limb-Girdle Muscular Dystrophies (LGMD) mainly involve the pelvic and/or shoulder girdle muscles. One of the major problems is limitations in performing daily activities. The natural course of the disease has been described before, and research is done to investigate functional ability of walking. However, little is known about functional abilities of the arms, which is even more important in performing many daily activities. Clear understanding is essential to improve rehabilitation therapies and for development of new arm supports. The aim of this study is to investigate arm function of people with LGMD on activity level, by using questionnaires. A web-based questionnaire to assess arm function was developed. We used questions from the Brooke scale (grading scale, 1–6), the Capabilities of Upper Extremity questionnaire (CUE) (basic arm functions, 30 items, max score 210, higher score means better function) and Abilhand questionnaire (22 ADL items, max score 44, higher score means lower function). Also questions about disease duration, age, gender were asked. The Dutch patient organization (VSN) distributed the questionnaire. Fifty-one people (age 6–72 y) participated in the period August 2011 till February 2012. Preliminary results show that in this group, 33% has Brooke scale 1 and 51% Brooke 2–4. The CUE total median score was 159 (range 60–210), corresponding with 75.7% of the maximum score. The total Abilhand median score was 39 (range 8–44) corresponding with a 88.6% of the maximum score. Especially activities above shoulder level are problematic. Patterns of limitations in arm function are further analyzed as are correlations with disease course, age, and gender. The project McArm is a national Dutch ‘Pieken in de Delta’ project. It is partially funded by: Agentschap.nl, an agency of the Dutch Ministry of Economic Affairs, Agriculture and Innovation, the Province of Noord Brabant and the Province of Limburg.

Persons with neurological disorders may experience fatigue and secondary physical de-conditioning. A multidisciplinary team developed a client-centred self-management group program including several components: aerobic training; education about training; education on managing fatigue and implementation of training and energy conservation strategies in daily life. The aim was to evaluate the effectiveness of this individually tailored group program. An uncontrolled pilot study with pre-post and follow up (after 3months) measurements was carried out. Participants had a neurological condition, suffered from lack of energy and were motivated to participate in the 15weeks program. Measurement instruments included Canadian Occupational Performance Measure (COPM), Six Minutes Walking Test (6MWT)), Checklist Individual Strength (CIS), and SF-36. Two groups were evaluated, including 13 participants with different neurological disorders (different neuromuscular disorders n =10, multiple sclerosis n =2, stroke n =1). There was a significant improvement following the program on the COPM performance and COPM satisfaction scores, on the CIS-fatigue, and on the domain health change in the SF-36. The improvement on the 6MWT minute walking test was obvious but not significant. At follow up, there was a slight deterioration in most scores except for health change on the SF-36. However, follow-up scores were still better than before the program. These preliminary positive results regarding the effectiveness of this program lead to improvements of the program. These include more psychological support and more support regarding problems at work. The next step will be setting up a randomized clinical trial and implementation of the program in other settings.

Purpose: To construct a questionnaire to measure skills in which notably both hands are active, which is suitable for patients with a disease of one or both hands and to research its response stability and possible improvements. Method: Using the Dutch elaboration of the ICIDH and then experts in a Delphi round, the questionnaire was constructed. Thereafter, test-retest and inter-observer reliability was examined. Fifty-four patients with RSD in one upper extremity and a normal contralateral extremity participated in the study, the first twenty to evaluate the construction of the questionnaire and the others to test reliability. Results: The constructed Radboud skills questionnaire was reliable in terms of response stability (median coefficients of variation 2.2% to 6.6%). Correlation's between categories of items were fair to good. Conclusions: A useful questionnaire was constructed to map alterations in the level of disability in patients with a disease of one hand or both hands.

Limb-Girdle Muscular Dystrophies (LGMD) mainly involve the pelvic and shoulder girdle muscles. One of the major problems is limitations in daily activities. The natural course of the diseases is described before, and research is done to investigate functional ability of walking. However, little is known about functional abilities of the arms. Arm functionality is complex, and although all kind of therapy including surgery are performed, little is known of the kinematics. The aim of this study is to investigate arm kinematics. A protocol containing a set of various movements was developed for 3D arm kinematics in LGMD, by using a VICON motion capturing system. The set of movements consists of four singular movements (shoulder abduction/flexion/rotation, horizontal shoulder adduction) and three more complex movements (hand to mouth, reaching at shoulder level left/right, pulling/pushing). EMG was also recorded during execution of all movements, and the EMG signal of maximal voluntary contraction (MVC) was measured before and after the trial. The results will be compared with an age matched healthy control group. The Dutch patient organization (VSN) has supported with recruiting participants for the research. Approval was received from the Ethical Committee. Six persons with LGMD and six controls will participate in the research. Kinematic patterns will be described, and the percentage of EMG signal of the EMG–MVC will be calculated The results of study will be presented. We expect to find that compensatory movements are used, as are muscle co-contractions, and persons with LGMD will have a higher percentage of EMG–MVC while performing movements than healthy controls. The project McArm is a national Dutch ‘Pieken in de Delta’ project. It is partially funded by: Agentschap.nl, an agency of the Dutch Ministry of Economic Affairs, Agriculture and Innovation, the Province of Noord Brabant and the Province of Limburg.

Facioscapulohumeral muscular dystrophy (FSHD) is a progressive muscular dystrophy. One of the major problems is limitations in performing daily activities. The natural course of the disease has been described before, and research is done to investigate functional ability of walking. However, little is known about functional abilities of the arms, which are even more important in performing many daily activities. Clear understanding is essential to improve rehabilitation therapies and for development of new arm supports. The aim of this study is to investigate arm function of people with FSHD on activity level, by using questionnaires. A web-based questionnaire to assess arm function was developed. We used questions from the Brooke scale (grading scale, 1–6), the Capabilities of Upper Extremity questionnaire (CUE) (basic arm functions, 30 items, max score 210, higher score means better function) and Abilhand questionnaire (22 ADL items, max score 44, higher score meaning lower function). Also questions about disease duration, age, gender were asked. The Dutch patient organization (VSN) supported with distribution of the questionnaire. Seventy-six people participated (age 13–74 y) in the period August 2011 till February 2012. Preliminary results show that from this group, 88% have Brooke 2–4. The CUE total median score was 155 (range 53–210), corresponding with 73.8% of the maximum score. The total Abilhand median score was 38 (range 13–44) corresponding with a 86.4% of the maximum score. Especially activities above shoulder level are problematic. Patterns of limitations in arm function are further analyzed as are correlations with disease course, age, and gender. The project McArm is a national Dutch ‘Pieken in de Delta’ project. It is partially funded by: Agentschap.nl, an agency of the Dutch Ministry of Economic Affairs, Agriculture and Innovation, the Province of Noord Brabant and the Province of Limburg.