Residual complaints after neuralgic amyotrophy.

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.

Living with myotonic dystrophy; what can be learned from couples? A qualitative study.

BACKGROUND: Myotonic dystrophy type 1 (MD1) is one of the most prevalent neuromuscular diseases, yet very little is known about how MD1 affects the lives of couples and how they themselves manage individually and together. To better match health care to their problems, concerns and needs, it is important to understand their perspective of living with this hereditary, systemic disease. METHODS: A qualitative study was carried out with a purposive sample of five middle-aged couples, including three men and two women with MD1 and their partners. Fifteen in-depth interviews with persons with MD1, with their partners and with both of them as a couple took place in the homes of the couples in two cities and three villages in the Netherlands in 2009. RESULTS: People with MD1 associate this progressive, neuromuscular condition with decreasing abilities, describing physical, cognitive and psychosocial barriers to everyday activities and social participation. Partners highlighted the increasing care giving burden, giving directions and using reminders to compensate for the lack of initiative and avoidant behaviour due to MD1. Couples portrayed the dilemmas and frustrations of renegotiating roles and responsibilities; stressing the importance of achieving a balance between individual and shared activities. All participants experienced a lack of understanding from relatives, friends, and society, including health care, leading to withdrawal and isolation. Health care was perceived as fragmentary, with specialists focusing on specific aspects of the disease rather than seeking to understand the implications of the systemic disorder on daily life. CONCLUSIONS: Learning from these couples has resulted in recommendations that challenge the tendency to treat MD1 as a condition with primarily physical impairments. It is vital to listen to couples, to elicit the impact of MD1, as a multisystem disorder that influences every aspect of their life together. Couple management, supporting the self-management skills of both partners is proposed as a way of reducing the mismatch between health services and health needs.

Implementation of multidisciplinary advice to allied health care professionals regarding the management of their patients with neuromuscular diseases.

PURPOSE: Patients with neuromuscular diseases (NMDs) do not always receive appropriate allied health care. This is partially because of the large heterogeneity among these conditions, some of which are quite rare. Individual allied health care professionals, therefore, have relatively little opportunity to develop relevant experience with these patients. To overcome this problem, we developed specialist multidisciplinary advice regarding management of patients with NMD for occupational therapy (OT), physical therapy (PT) and speech therapy (ST) in a primary care or rehabilitation setting. The aims of the study were to explore to what extent this allied health care advice was implemented with a focus on the amount of therapy received and to explore possible barriers to implementation. METHODS: One-hundred two patients visited the Neuromuscular Centre Nijmegen for OT, PT and ST consultations. Integrated allied health care advice was written on the basis of these consultations and a multidisciplinary meeting. All patients, their therapists and rehabilitation physicians received this advice. Following the advice, questionnaires were sent out at baseline and at 6 months follow up, collecting data on implementation of the amount of therapy suggested and on possible barriers for adherence. RESULTS: Advice for ST and OT was fully implemented in primary care, but only partially (58%) in a rehabilitation setting. Advice to reduce the amount of PT was implemented in only 15% of the cases. Possible barriers were related to the advice itself (feasibility of treatment duration, correctness and completeness), the patient (motivation) and the professional (experience in treatment of NMDs). Therapists expressed a desire to have the opportunity to discuss the treatment advice with the multidisciplinary team. CONCLUSIONS: The extent to which multidisciplinary advice was implemented differed for OT and ST compared to PT and for the setting (primary care or rehabilitation). Possible barriers were identified at different levels. We recommend follow-up telephone calls to provide therapists opportunity for discussion.

Computer-aided visualization of muscle weakness distribution.

We present a computer program for visualizing muscle weakness distribution in patients with neuromuscular disorders. Ordinal muscle strength data can be computed in the program. Data are visualized as the prime movers of the testmovements in an image of the human body and a separate image of the face. Severity is indicated in shades of grey and black indicates complete loss of muscle power. This visualization facilitates the diagnosis and follow-up of patients and is an excellent tool for patient education. Moreover, the program can also be used to investigate and further refine the phenotypic description of these disorders.

Development of a tool to guide referral of patients with neuromuscular disorders to allied health services. Part one.

PURPOSE: Reasons for referral of patients with chronic, slowly progressive neuromuscular disorders (NMD) to occupational therapy (OT), physical therapy (PT) and speech therapy (ST) are often unclear. One-off consultations by OT, PT and ST can help patients and physicians to decide if therapy is needed. We present a construct for a questionnaire, the Perceived Limitations in Activities and Needs Questionnaire (PLAN-Q). Its aim is to signal problems and needs of patients with chronic, slowly progressive NMD with a view to referral for one-off consultations by OT, PT and ST. We report on the construct, item pool, response options and the item reduction procedure as the first development stages for the PLAN-Q. METHODS: The International Classification of Functioning, Disability and Health (ICF) served as source for items and response options. Five clinical health professionals of the Neuromuscular Center Nijmegen (NMCN) formed an expert panel. They were asked to reach consensus on construct, item pool and response options. Subsequently a sample of patients with NMD (n=21) that attended the NMCN was asked for their approval of the concept questionnaire. Data of a large cohort (n=208) of patients with a broad variety of chronic NMD were used to carry out item reduction. RESULTS: The expert panel reached consensus on the selection of 56 items covering eight activity categories relevant to OT, PT, and ST. They also reached consensus on response options regarding 'capacity' and 'need for therapy or advice'. The patient sample approved the concept PLAN-Q. The item reduction procedure resulted in a reduction to 25 items covering eight activity categories. CONCLUSION: The PLAN-Q has proceeded through the first stages of development and is now ready for further assessment of reliability and validity.

Development of a tool to guide referral of patients with neuromuscular disorders to allied health services. Part two.

PURPOSE: The Perceived Limitations and Needs Questionnaire (PLAN-Q) was developed to guide referral for a one-off consultation by occupational therapy (OT), physical therapy (PT), and speech therapy (ST) consultation, to provide a tailor-made advice on allied health interventions. This article reports on the testing of validity and reliability of the PLAN-Q. METHODS: In the validation study, 208 patients with a broad spectrum of neuromuscular disorders completed the PLAN-Q, Medical Outcome Study short-form 36-item version (SF-36), and the Impact of Participation on Autonomy (IPA) questionnaires. A subsection of 51 patients, whose physical condition was stable, participated in the evaluation of the intra-rater reliability of the questionnaire. The theoretical construct was tested with factorial analysis, subscales were constructed and reliability and validity of the PLAN-Q subscales were assessed. RESULTS: Factorial analysis resulted in an 18-item self-report questionnaire. Items were grouped into four subscales ('physical capacity', 'transferring', 'oropharyngeal capacity', and 'hand-use'), each with two dimensions ('capacity' and 'need for help'). The internal consistency of all subscales was good (Cronbach's alpha: 0.77-0.94) as well as the intra-rater reliability of the subscales 'physical capacity' and 'transferring' in the 'capacity' dimension (Kappa: 0.70-0.75). The 'need' dimension showed poor intra-rater reliability suggesting that 'need for help' is a variable phenomenon that changes between two points of measurement. The construct validity of the subscales against the SF-36 and the IPA was satisfactory. CONCLUSION: The PLAN-Q is a valid self-report instrument that measures patients' perception of capacity and needs in domains relevant to referral for a one-off OT, PT, or ST consultation.

Exercise therapy and other types of physical therapy for patients with neuromuscular diseases: a systematic review.

OBJECTIVE: To summarize and critically appraise the available 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, Medline, CINAHL, EMBASE (Rehabilitation and Physical Medicine), and reference lists of reviews and articles. STUDY SELECTION: Randomized clinical trials (RCTs), controlled 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 physical 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 included 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 analysis. 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 ("indications 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 effects of exercise therapy were negligible. CONCLUSIONS: The available evidence is limited, but relevant for clinicians. Future studies should be preferably multicentered, 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.

Referral of patients with neuromuscular disease to occupational therapy, physical therapy and speech therapy: usual practice versus multidisciplinary advice.

UNLABELLED: 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.