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Background: Cognitive behavioural therapy does not reduce fatigue in post-polio syndrome, but is effective in facioscapulohumeral dystrophy. This difference in efficacy might be explained by a different role of cognitions in these conditions., Objective: To compare fatigue-related cognitions between patients with post-polio syndrome and facio-scapulohumeral dystrophy., Subjects: Patients with post-polio syndrome (n = 21) and facioscapulohumeral dystrophy (n = 24) allocated to a cognitive behavioural therapy intervention in 2 identical trials., Methods: Assessed cognitions included: sense of control over fatigue; catastrophizing; acceptance; focusing on fatigue; and perceived social support. Group differences in cognitions (independent t-tests or Mann-Whitney U tests) and group differences in the association of cognitions with fatigue (linear regression models) were studied., Results: No differences in cognitions were found between the 2 groups (p > 0.18). Furthermore, there were no cognition-by-group interaction effects, except for "perceived social support", for which a different association with fatigue was found between the 2 groups (p = 0.01). However, univariate models revealed no associations per group., Conclusion: Fatigue-related cognitions in severely fatigued patients with post-polio syndrome are not clearly different from that in facioscapulohumeral dystrophy. Thus, the lack of efficacy of cognitive behavioural therapy in post-polio syndrome cannot be attributed to unique cognitive characteristics of this population.

Objective: To explore reasons for the lack of efficacy of a high intensity aerobic exercise program in post-polio syndrome (PPS) on cardiorespiratory fitness by evaluating adherence to the training program and effects on muscle function., Design: A process evaluation using data from an RCT., Patients: Forty-four severely fatigued individuals with PPS were randomized to exercise therapy (n = 22) or usual care (n = 22)., Methods: Participants in the exercise group were instructed to exercise 3 times weekly for 4 months on a bicycle ergometer (60-70% heart rate reserve)., Results: The attendance rate was high (median 89%). None of the participants trained within the target heart rate range during >75% of the designated time. Instead, participants exercised at lower intensities, though still around the anaerobic threshold (AT) most of the time. Muscle function did not improve in the exercise group., Conclusion: Our results suggest that severely fatigued individuals with PPS cannot adhere to a high intensity aerobic exercise program on a cycle ergometer. Despite exercise intensities around the AT, lower extremity muscle function nor cardiorespiratory fitness improved. Improving the aerobic capacity in PPS is difficult through exercise primarily focusing on the lower extremities, and may require a more individualized approach, including the use of other large muscle groups instead., Trial Registration: Netherlands National Trial Register NTR1371.

Background: People with postpolio syndrome (PPS) commonly experience severe fatigue that persists over time and negatively affects functioning and health-related quality of life (HRQoL)., Objectives: To study the efficacy of exercise therapy (ET) and cognitive behavioral therapy (CBT) on reducing fatigue and improving activities and HRQoL in patients with PPS., Methods: We conducted a multicenter, single-blinded, randomized controlled trial. Over 4 months, severely fatigued patients with PPS received ET, CBT, or usual care (UC). The primary end point (fatigue) was assessed using the subscale fatigue severity of the Checklist Individual Strength (CIS20-F). Secondary end points included activities and HRQoL, which were assessed with the Sickness Impact Profile and the 36-Item Short-Form, respectively. End points were measured at baseline and at 4, 7, and 10 months., Results: A total of 68 patients were randomized. No differences were observed between the intervention groups and UC group for fatigue (mean differences in CIS20-F score = 1.47, 95%CI = -2.84 to 5.79, for ET versus UC; and 1.87, 95%CI = -2.24 to 5.98, for CBT versus UC), activities, or HRQoL., Conclusions: Our results demonstrate that neither ET nor CBT were superior to UC in reducing fatigue in severely fatigued PPS patients. Further research should investigate explanations for the lack of efficacy of these 2 currently advised approaches in clinical practice, which may provide clues to improving treatment aimed at reducing fatigue in PPS., (© The Author(s) 2015.)

Background: Many persons affected with poliomyelitis develop post-polio syndrome (PPS) later in their life. Recently, the effectiveness of Exercise Therapy (ET) and Cognitive Behavioural Therapy (CBT) for PPS has been evaluated in a randomized controlled trial, but did not show a decrease in fatigue or improvement in secondary endpoints like Quality of Life and self-perceived activity limitations. The aim of this explorative study was to gain insight in the perceived effects and experiences of the interventions from the perspectives of the patients and therapists., Methods: Qualitative data were collected through semi-structured interviews with 17 patients and 7 therapists. All participants were involved in the trial. A thematic analysis of the data was performed., Results: Some patients experienced a short term enhanced endurance and a better use of energy during the day. However, in general patients did not experience a long lasting reduction of fatigue from the CBT or ET. Mainly patients of the CBT, but also some patients of the ET described an increase of self-esteem and self-acceptance. As a result, patients were sometimes better able to perform physical activities during the day. In contrast to the CBT, the ET was in general perceived by the patients as an intensive therapy, which was difficult to fit into their daily routine. Therapists of both the CBT and the ET struggled with a low intrinsic motivation of the patients in the study. This made it sometimes difficult for the therapists to follow the protocol., Conclusion: Confirming the negative quantitative study outcome, the qualitative results did not demonstrate lasting effects on fatigue. Patients did, however, experience some benefits on self-esteem and acceptance of the disease. This study showed that it is of great importance to work with feasible interventions; they should fit the patients' needs on a practical (fit into their daily routine) and mental (fit their need for support) level.

A key feature of post-polio syndrome (PPS) is progressive loss of muscle strength. In other chronic diseases systemic inflammation has been linked to muscle wasting. In this study plasma TNF-α, IL-6, IL-8, and leptin levels were significantly increased in PPS-patients compared to healthy controls. There was however no association between these raised systemic levels of inflammatory mediators and long-term decline in quadriceps strength or other clinical parameters. In conclusion, there is evidence for systemic inflammation in PPS, yet the relationship with clinical deterioration remains tenuous., (Copyright © 2015 Elsevier B.V. All rights reserved.)

Background: Postpolio syndrome (PPS) may affect survivors of paralytic poliomyelitis and is characterised by a complex of neuromuscular symptoms leading to a decline in physical functioning. The effectiveness of pharmacological treatment and rehabilitation management in PPS is not yet established. This is an update of a review first published in 2011., Objectives: To systematically review the evidence from randomised and quasi-randomised controlled trials for the effect of any pharmacological or non-pharmacological treatment for PPS compared to placebo, usual care or no treatment. , Search Methods: We searched the following databases on 21 July 2014: Cochrane Neuromuscular Disease Group Specialized Register, the Cochrane Central Register of Controlled Trials (CENTRAL), MEDLINE, EMBASE, PsycINFO and CINAHL Plus. We also checked reference lists of all relevant articles, searched the Database of Abstracts of Reviews of Effects (DARE), the Health Technology Assessment (HTA) Database and trial registers and contacted investigators known to be involved in research in this area., Selection Criteria: Randomised and quasi-randomised trials of any form of pharmacological or non-pharmacological treatment for people with PPS. The primary outcome was self perceived activity limitations and secondary outcomes were muscle strength, muscle endurance, fatigue, pain and adverse events., Data Collection and Analysis: We used standard methodological procedures expected by The Cochrane Collaboration., Main Results: We included 10 pharmacological (modafinil, intravenous immunoglobulin (IVIg), pyridostigmine, lamotrigine, amantadine, prednisone) and three non-pharmacological (muscle strengthening, rehabilitation in a warm climate (that is temperature ± 25°C, dry and sunny) and a cold climate (that is temperature ± 0°C, rainy or snowy), static magnetic fields) studies with a total of 675 participants with PPS in this review. None of the included studies were completely free from any risk of bias, the most prevalent risk of bias being lack of blinding.There was moderate- and low-quality evidence that IVIg has no beneficial effect on activity limitations in the short term and long term, respectively, and inconsistency in the evidence for effectiveness on muscle strength. IVIg caused minor adverse events in a substantial proportion of the participants. Results of one trial provided very low-quality evidence that lamotrigine might be effective in reducing pain and fatigue, resulting in fewer activity limitations without generating adverse events. Data from two single trials suggested that muscle strengthening of thumb muscles (very low-quality evidence) and static magnetic fields (moderate-quality evidence) are safe and beneficial for improving muscle strength and pain, respectively, with unknown effects on activity limitations. Finally, there was evidence varying from very low quality to high quality that modafinil, pyridostigmine, amantadine, prednisone and rehabilitation in a warm or cold climate are not beneficial in PPS., Authors' Conclusions: Due to insufficient good-quality data and lack of randomised studies, it was impossible to draw definite conclusions about the effectiveness of interventions for PPS. Results indicated that IVIg, lamotrigine, muscle strengthening exercises and static magnetic fields may be beneficial but need further investigation to clarify whether any real and meaningful effect exists.

Post-polio syndrome is characterised by progressive muscle weakness and other symptoms which can limit physical mobility. We assessed the rate of decline in mobility over 10 years in relation to strength decline; and investigated potential predictors for the rate of decline of walking capacity, a measure of mobility, in 48 patients with post-polio syndrome and proven quadriceps dysfunction at baseline. Average walking capacity and self-reported physical mobility declined over 10 years, by 6 and 14%, respectively. Concomitantly people lost an average of 15% of isometric quadriceps strength. Significantly more people used walking aids offering greater support at follow-up. Notably, there was much individual variation, with 18% of participants losing a substantial amount of walking capacity (27% decline) and concomitant self-reported physical mobility (38% decline). Loss of quadriceps strength only explained a small proportion of the variance of the decline in walking capacity (R = 11%) and the rate of decline could not be predicted from baseline values for strength, walking capacity, self-reported physical mobility or basic demographics. The individual variability, yet lack of predictive factors, underscores the need for personally tailored care based on actual functional decline in patients with post-polio syndrome., (Copyright © 2014. Published by Elsevier B.V.)

Objectives: To compare the content of the Fatigue Severity Scale and the subscale "subjective experience of fatigue" of the Checklist Individual Strength, and (ii) to assess the reliability of both questionnaires in polio survivors., Design: Repeated-measures at a 3-week interval., Subjects: Consecutive series of 61 polio survivors., Methods: Concepts contained in the questionnaire items were linked to the International Classification of Functioning, Disability and Health (ICF), using standardized linking rules. Reliability analyses included tests of internal consistency, test-retest reliability and measurement error., Results: Questionnaires differed in the extent to which they measured other than fatigue-related aspects of functioning (represented ICF components: "Body functions": 50% and 80%, "Activities and Participation": 30% and 0%, for the Fatigue Severity Scale and Checklist Individual Strength, respectively). Internal consistency and test-retest reliability were considered acceptable, while measurement error was large (Cronbach's α: 0.90 and 0.93, intraclass correlation coefficient: 0.80 and 0.85, smallest detectable change: 28.7% and 29.4% for the Fatigue Severity Scale and Checklist Individual Strength, respectively)., Conclusion: Considering the acceptable clinimetric properties, we conclude that both the Fatigue Severity Scale and the Checklist Individual Strength can be applied in research on post-poliomyelitis syndrome when measuring fatigue. However, because the 2 questionnaires differ in content they cannot be used interchangeably.

Objective: To assess the reliability of contractile properties of the knee extensor muscles in 23 individuals with post-polio syndrome (PPS) and 18 age-matched healthy individuals., Methods: Contractile properties of the knee extensors were assessed from repeated electrically evoked contractions on 2 separate days, with the use of a fixed dynamometer. Reliability was determined for fatigue resistance, rate of torque development (MRTD), and early and late relaxation time (RT50 and RT25), using the intraclass correlation coefficient (ICC) and standard error of measurement (SEM, expressed as % of the mean)., Results: In both groups, reliability for fatigue resistance was good, with high ICCs (>0.90) and small SEM values (PPS: 7.1%, healthy individuals: 7.0%). Reliability for contractile speed indices varied, with the best values found for RT50 (ICCs>0.82, SEM values <2.8%). We found no systematic differences between test and retest occasions, except for RT50 in healthy subjects (p = 0.016)., Conclusions: In PPS and healthy individuals, the reliability of fatigue resistance, as obtained from electrically evoked contractions is high. The reliability of contractile speed is only moderate, except for RT50 in PPS, demonstrating high reliability., Significance: This was the first study to examine the reliability of electrically evoked contractile properties in individuals with PPS. Our results demonstrate its potential to study mechanisms underlying muscle fatigue in PPS and to evaluate changes in contractile properties over time in response to interventions or from natural course.

Objective: To investigate whether strength decline in post-polio syndrome (PPS) results from excessive distal axonal degeneration of enlarged motor units., Methods: We assessed changes over 10 years in isometric quadriceps strength, mean motor unit action potential (MUAP) size, root mean squared (RMS) amplitude, and level of interference (LOI) in 47 patients with PPS and 12 healthy controls, using high density surface EMG. At baseline, all patients had symptomatic quadriceps dysfunction, evidenced by transmission defects on single-fibre EMG., Results: MU size and strength declined significantly by 20% and 15%, respectively in patients with PPS. Those with the largest initial MU sizes exhibited the greatest losses of mean MU size (27%) and proportional decreases in quadriceps strength (23%). Initial strength, change in LOI and change in RMS amplitude together explained 35% of the variability in strength changes in patients. MU size of controls did not change, although they lost 29% strength., Conclusions: MU size and strength declined concomitantly in a homogeneous cohort of patients with PPS and quadriceps dysfunction., Significance: This long term follow-up study provides evidence that size diminution of enlarged MUs combined with a reduced number of active MUs contributes to the gradual strength decline in PPS., (Copyright © 2013 International Federation of Clinical Neurophysiology. Published by Elsevier Ireland Ltd. All rights reserved.)

Objectives: To determine whether the anaerobic threshold (AT) can be identified in individuals with postpolio syndrome (PPS) using submaximal incremental exercise testing, and to compare current guidelines for intensity prescription in PPS with the AT., Design: Cohort study., Setting: Research laboratory., Participants: Individuals with PPS (N=82)., Interventions: Not applicable., Main Outcome Measures: Power output, gas exchange variables, heart rate, and rating of perceived exertion (RPE) were measured in an incremental submaximal cycle ergometry test. Two independent observers identified the AT. Comparison of current guidelines for training intensity prescription in PPS (40%-60% heart rate reserve [HRR] or RPE of 12) with the AT was based on correlations between recommended heart rate and the heart rate at the AT. In addition, we determined the proportion of individuals that would have been recommended to train at an intensity corresponding to their AT., Results: The AT was identified in 63 (77%) of the participants. Pearson correlation coefficients between the recommended heart rate and the heart rate at the AT were lower in cases of 40% HRR (r=.56) and 60% HRR (r=.50) than in cases of prescription based on the RPE (r=.86). Based on the RPE, 55% of the individuals would have been recommended to train at an intensity corresponding to their AT. This proportion was higher compared with 40% HRR (41%) or 60% HRR (18%) as criterion., Conclusions: The AT can be identified in most individuals with PPS offering an individualized target for aerobic training. If the AT cannot be identified (eg, because gas analysis equipment is not available), intensity prescription can best be based on the RPE., (Copyright © 2014 American Congress of Rehabilitation Medicine. Published by Elsevier Inc. All rights reserved.)

The present study investigated whether intrinsic fatigability of the muscle fibers is reduced in patients with post-polio syndrome (PPS). This may contribute to the muscle fatigue complaints reported by patients with PPS. For this purpose, we assessed contractile properties and fatigue resistance of the knee extensor muscles using repeated isometric electrically evoked contractions in 38 patients with PPS and 19 age-matched healthy subjects. To determine whether any difference in fatigue resistance between both groups could be attributed to differences in aerobic capacity of the muscle fibers, 9 patients with PPS and 11 healthy subjects performed the same protocol under arterial occlusion. Results showed that fatigue resistance of patients with PPS was comparable to that in controls, both in the situation with intact circulation and with occluded blood flow. Together, our findings suggest that there are no differences in contractile properties and aerobic muscle capacity that may account for the increased muscle fatigue perceived in PPS., (Copyright © 2013 Elsevier B.V. All rights reserved.)

Objectives: To study the severity and 5-year course of fatigue in patients with late-onset sequelae of poliomyelitis (LOSP) and to identify physical and psychosocial determinants of fatigue., Design: Prospective cohort study with 5 measurements over 5 years., Setting: University hospital., Participants: Patients with LOSP (N=168); 89% of the subjects completed the study., Interventions: Not applicable., Main Outcome Measures: Fatigue assessed with the Fatigue Severity Scale (FSS). Potential determinants were perceived physical functioning, bodily pain and mental health, extent of paresis, walking capacity, comorbidity, sleeping disorders, coping, and social support. Associations were investigated by multivariable longitudinal analysis using generalized estimating equations., Results: The mean FSS score ± SD at baseline was 5.1±1.4, which did not change significantly during the 5-year follow-up. Reduced physical functioning, increased bodily pain, reduced sleep quality, more psychologic distress, and higher task-oriented coping were independently associated with fatigue. The extent of paresis and walking capacity were strongly associated with physical functioning., Conclusions: Fatigue is severe and persistent in patients with LOSP due to physical and psychologic factors, which has implications for counseling and treatment. In addition to the commonly applied interventions targeting physical aspects, psychologic interventions are a potential area for reducing fatigue., (Copyright © 2011 American Congress of Rehabilitation Medicine. Published by Elsevier Inc. All rights reserved.)

Background: Postpolio syndrome (PPS) may affect survivors of paralytic poliomyelitis and is characterised by a complex of neuromuscular symptoms leading to a decline in physical functioning. The effectiveness of pharmacological treatment and rehabilitation management in PPS is not yet established., Objectives: To review systematically the effects of any treatment for PPS compared to placebo, usual care or no treatment.  , Search Strategy: We searched the following databases on 1 October 2010: Cochrane Neuromuscular Disease Group Specialized Register, the Cochrane Central Register of Controlled Trials, MEDLINE, EMBASE, PsycINFO and CINAHL Plus from inception to September 2010., Selection Criteria: Randomised and quasi-randomised trials of any form of pharmacological or non-pharmacological treatment for people with PPS. The primary outcome was self-perceived activity limitations and secondary outcomes were muscle strength, muscle endurance, fatigue, pain and adverse events., Data Collection and Analysis: Two authors independently selected eligible studies, assessed risk of bias and extracted data., Main Results: Nine pharmacological (modafinil, intravenous immunoglobulin, pyridostigmine, lamotrigine, amantadine, prednisone) and three non-pharmacological (muscle strengthening, rehabilitation in a warm climate (i.e. temperature ± 25°C, dry and sunny) and a cold climate (i.e. temperature ± 0°C, rainy or snowy), static magnetic fields) studies were included in this review. None of the included studies was completely free from any risk of bias and the most prevalent risk of bias was lack of blinding.There is moderate quality evidence that intravenous immunoglobulin has no beneficial effect on activity limitations and there is inconsistency in the evidence for effectiveness on muscle strength and pain. Results of one trial provide very low quality evidence that lamotrigine might be effective in reducing pain and fatigue, resulting in fewer activity limitations. Data from two single trials suggest that muscle strengthening of thumb muscles (very low quality evidence) and static magnetic fields (moderate quality evidence) are beneficial for improving muscle strength and pain, respectively, with unknown effects on activity limitations. Finally, there is evidence varying from very low quality to high quality that modafinil, pyridostigmine, amantadine, prednisone and rehabilitation in a warm or cold climate are not beneficial in PPS., Authors' Conclusions: Due to insufficient good quality data and lack of randomised studies it is impossible to draw definite conclusions on the effectiveness of interventions for PPS. Results indicate that IVIG, lamotrigine, muscle strengthening exercises and static magnetic fields may be beneficial but need further investigation.

Purpose: This study aimed to explore the perceived work ability of patients with postpoliomyelitis syndrome (PPS), to identify barriers and factors that are impeding or conducive, respectively, to work participation, and to identify possible interventions., Methods: Qualitative cross-sectional interview study with 17 subjects with PPS, 12 women and 5 men, mean age 49 years (SD: 11). Semi-structured interviews were held with the subjects in their homes, with the aid of a topic list. The COREQ criteria list for qualitative research was used as guideline in design and analysis., Results: Thirteen out of 17 subjects rated their work ability six or higher on a scale from 0 to 10. Most subjects worked in an administrative, educational or managerial function. Five subjects stopped working, four worked between 0 and 20 h/week and eight worked between 20 and 40 h/week. Factors conducive to working were physical adaptations in the workplace, accessibility of the workplace and high decision latitude. Barriers to full work ability in PPS patients were high physical job demands, low social support and the symptoms of PPS, especially fatigue and pain., Conclusions: These findings suggest that work-oriented interventions aimed at preventing PPS patients from dropping out of the workforce should primarily focus on reducing physical job demands, arranging adaptations in the workplace and increasing job control.

Objectives: Many polio survivors have symptoms that are known risk factors for falls in elderly people. This study aims to determine the: (i) frequency; (ii) consequences; (iii) circumstances; and (iv) factors associated with falls in polio survivors., Methods: A survey was conducted among 376 polio survivors. Participants completed a falls history questionnaire and additional information was obtained from their medical files., Results: Of the 305 respondents, 74% reported at least one fall in the past year and 60% two or more. Sixteen percent of fallers described a major injury after a fall in the last year and 69% reported fear of falling. One-third of fallers had reduced the amount they walked because of their fear of falling. Most reported falls in a familiar environment (86%), during ambulation (72%) and in the afternoon (50%). Quadriceps weakness of the weakest leg (Medical Research Council (MRC) ≤ 3), fear of falling and complaints of problems maintaining balance were independently associated with both falls and recurrent falls, while increasing age and medication use were not., Conclusion: The high rate of falls and consequences thereof, merit the implementation of fall intervention strategies. To maximize effect, they should be tailor-made and target the fall mechanisms specific to polio survivors.

Background: Postpoliomyelitis Syndrome (PPS) is a complex of late onset neuromuscular symptoms with new or increased muscle weakness and muscle fatigability as key symptoms. Main clinical complaints are severe fatigue, deterioration in functional abilities and health related quality of life. Rehabilitation management is the mainstay of treatment. Two different therapeutic interventions may be prescribed (1) exercise therapy or (2) cognitive behavioural therapy (CBT). However, the evidence on the effectiveness of both interventions is limited. The primary aim of the FACTS-2-PPS trial is to study the efficacy of exercise therapy and CBT for reducing fatigue and improving activities and quality of life in patients with PPS. Additionally, the working mechanisms, patients' and therapists' expectations of and experiences with both interventions and cost-effectiveness will be evaluated., Methods/design: A multi-centre, single-blinded, randomized controlled trial will be conducted. A sample of 81 severely fatigued patients with PPS will be recruited from 3 different university hospitals and their affiliate rehabilitation centres. Patients will be randomized to one of three groups i.e. (1) exercise therapy + usual care, (2) CBT + usual care, (3) usual care. At baseline, immediately post-intervention and at 3- and 6-months follow-up, fatigue, activities, quality of life and secondary outcomes will be assessed. Costs will be based on a cost questionnaire, and statistical analyses on GEE (generalized estimated equations). Analysis will also consider mechanisms of change during therapy. A responsive evaluation will be conducted to monitor the implementation process and to investigate the perspectives of patients and therapists on both interventions., Discussion: A major strength of the FACTS-2-PPS study is the use of a mixed methods design in which a responsive and economic evaluation runs parallel to the trial. The results of this study will generate new evidence for the rehabilitation treatment of persons with PPS., Trial Registration: Dutch Trial Register NTR1371.

Objective: To select a questionnaire and walking capacity test based on comparison of clinimetric properties and mutual association to be used as "core" qualifiers for physical functioning in patients with late-onset sequelae of poliomyelitis., Design: Repeated-measures at 3-week intervals., Subjects: An unselected sample of 57 patients with late-onset sequelae of poliomyelitis from a prospective cohort study., Methods: Physical functioning scales from Short Form-36 (SF36-PF), Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC-PF) and Nottingham Health Profile (NHP-PM). Timed-Up-and-Go test, 10-m walking at self-preferred and maximum speed, and 2-min walk test., Results: Test-retest reliability of SF36-PF and WOMAC-PF was good (intraclass correlation coefficient (ICC) 0.92 and 0.89, respectively), sufficient for NHP-PM (ICC 0.74) and excellent for walking tests (ICC 0.93-0.96). The smallest detectable changes were 18.4 and 16.5, respectively, for WOMAC-PF and SF36-PF, and 26.7 for NHP-PM. The smallest detectable change was best for the 2-min walk test (22.9 m). Correlation coefficients between questionnaires and walking tests ranged from 0.32 to 0.69, with the highest correlation between the SF36-PF and 2-min walk test., Conclusion: The SF36-PF and 2-min walk test are recommended as core qualifiers for physical functioning, the major increasing disability in late-onset sequelae of poliomyelitis, to assess perceived physical performance and walking capacity in research and clinical practice.

Objective: To investigate the effects of total-contact fitted carbon-composite knee-ankle-foot orthoses (KAFOs) on energy cost of walking in patients with former polio who normally wear a conventional leather/metal KAFO or plastic/metal KAFO., Design: A prospective uncontrolled study with a multiple baseline and follow-up design. Follow-up measurements continued until 26 weeks after intervention., Participants: Twenty adults with polio residuals (mean age 55 years)., Intervention: Each participant received a new carbon-composite KAFO, fitted according to a total-contact principle, which resulted in a rigid, lightweight and well-fitting KAFO., Outcome Measures: Energy cost of walking, walking speed, biomechanics of gait, physical functioning and patient satisfaction., Results: The energy cost decreased significantly, by 8%, compared with the original KAFO. Furthermore, the incremention energy cost during walking with the carbon-composite KAFO was reduced by 18% towards normative values. An improvement in knee flexion, forward excursion of the centre of pressure, peak ankle moment, and timing of peak ankle power were significantly associated with the decrease in energy cost. Walking speed and physical functioning remained unchanged., Conclusion: In patients with former polio, carbon-composite KAFOs are superior to conventional leather/metal and plastic/metal KAFOs with respect to improving walking efficiency and gait, and are therefore important in reducing overuse and maintaining functional abilities in polio survivors.

Objective: To investigate the impact of age and co-morbidity on the functional independence and perceived physical functioning of patients with sequelae of poliomyelitis., Design: Cross sectional study., Subjects: A convenience sample of 168 patients with sequelae of poliomyelitis, aged 45-85 years, recruited from 2 university hospitals., Methods: Outcome measures were Functional Independence Measure (FIMTM) for functional independence, Short Form-36 (SF-36) for physical functioning and general mental health, Cumulative Illness Rating Scale (CIRS) for co-morbidity., Results: FIMTM scores were significantly lower for the 65-85 year age group than for the 45-54 year age group. No differences in the SF-36 were found between the age groups, except that the SF-36 general mental health sub-scale score was significantly better in the 65-85 year age group than in the 45-54 year age group. The CIRS score increased significantly with age. Linear regression showed that age, gender, polio severity, and 4 co-morbidity scores ("cardiac", "vascular", "endocrine, metabolic"and "muscle, bone, skin") were significantly and inversely associated with functional independence and physical functioning., Conclusion: The level of functional independence of elderly former poliomyelitis patients is lower than that of younger patients. Specific attention should be paid to co-morbidity and ageing in this increasingly older population of polio survivors, since they negatively affect functional independence and perceived functioning.

Post-polio syndrome (PPS) is characterized by new or increased muscular weakness, atrophy, muscle pain and fatigue several years after acute polio. The aim of the article is to prepare diagnostic criteria for PPS, and to evaluate the existing evidence for therapeutic interventions. The Medline, EMBASE and ISI databases were searched. Consensus in the group was reached after discussion by e-mail. We recommend Halstead's definition of PPS from 1991 as diagnostic criteria. Supervised, aerobic muscular training, both isokinetic and isometric, is a safe and effective way to prevent further decline for patients with moderate weakness (Level B). Muscular training can also improve muscular fatigue, muscle weakness and pain. Training in a warm climate and non-swimming water exercises are particularly useful (Level B). Respiratory muscle training can improve pulmonary function. Recognition of respiratory impairment and early introduction of non-invasive ventilatory aids prevent or delay further respiratory decline and the need for invasive respiratory aid (Level C). Group training, regular follow-up and patient education are useful for the patients' mental status and well-being. Weight loss, adjustment and introduction of properly fitted assistive devices should be considered (good practice points). A small number of controlled studies of potential-specific treatments for PPS have been completed, but no definitive therapeutic effect has been reported for the agents evaluated (pyridostigmine, corticosteroids, amantadine). Future randomized trials should particularly address the treatment of pain, which is commonly reported by PPS patients. There is also a need for studies evaluating the long-term effects of muscular training.

Objectives: To describe the energy demands of walking in subjects with postpoliomyelitis syndrome (PPS) in comparison with the demands in healthy subjects, and to assess the reproducibility of walking energy measurements., Design: Four repeated measurements with a 1-week interval between each measurement., Setting: Outpatient clinic of a university hospital., Participants: Fourteen subjects with PPS and 14 age- and sex-matched healthy subjects., Interventions: Not applicable., Main Outcome Measures: Walking speed and energy cost of walking. The correlation parameter was lower-extremity muscle strength sum (MSS). The reproducibility parameters were standard error (SE) of measurement and smallest detectable difference (SDD)., Results: Walking speed in subjects with PPS (61.8 m/min) was significantly lower (-28%) and energy cost (4.8 J.kg(-1).m(-1)) was significantly higher (40%) than in healthy subjects. MSS correlated strongly with energy cost (r=-.84, P=.000), explaining 71% of the variance. The SE of measurement of energy cost measurements ranged between 1.7% and 3.4% for PPS subjects and between 1.2% and 2.4% for healthy subjects. The SDD ranged between 4.6% and 9.4% for PPS subjects and between 3.3% and 6.6% for healthy subjects, depending on the number of repeated measurements that were considered., Conclusions: Energy cost of walking in subjects with PPS is strongly related to the extent of muscle weakness in the lower extremities. Although variability was greater for PPS subjects than for healthy subjects, reproducibility of energy cost measurements was high. Therefore, metabolic assessment of energy cost of walking is a sensitive tool that can reveal clinically relevant changes even in the condition of a person with PPS.

Objectives: To review systematically studies of late-onset polio sequelae on the course of functional status and muscle strength over time and to identify prognostic factors of change., Data Sources: We conducted a computerized literature search up to July 2004 in MEDLINE, EMBASE, CINAHL, Web of Science, PsychInfo, and the Cochrane controlled trial register using the key words: postpolio, postpoliomyelitis, postpoliomyelitis syndrome, post poliomyelitis muscular atrophy, and poliomyelitis., Study Selection: Reports were selected by 1 reviewer if the study involved subjects with a history of poliomyelitis, the outcome measures described functional status or muscle strength, and follow-up was for at least 6 months., Data Extraction: Studies were summarized with regard to population, design, sample size, outcome measures, results, and methodologic scores. Overlap in populations between studies was checked., Data Synthesis: Of 71 potentially relevant studies, 19 were included (2 on functional status, 15 on muscle strength, 2 on both muscle strength and functional status). Two studies on the course of functional status had sufficient quality and reported inconsistent results. Four studies on the course of muscle strength had sufficient quality. Two studies reported a decline in strength and 2 reported no change. Decline in strength was only reported in studies with a follow-up period longer than 2 years. One study reported extent of paresis as a prognostic factor for change in perceived physical mobility., Conclusions: Conclusions cannot be drawn from the literature with regard to the functional course or prognostic factors in late-onset polio sequelae. The rate of decline in muscle strength is slow, and prognostic factors have not yet been identified. Long-term follow-up studies with unselected study populations and age-matched controls are needed, with specific focus on prognostic factors.

Objective: To compare walking test results with walking in daily life, and to investigate the relationships between walking tests, walking activity in daily life, and perceived mobility problems in patients with post-poliomyelitis syndrome., Subjects: Twenty-four ambulant patients with post-poliomyelitis syndrome., Methods: Walking tests were performed at self-preferred and maximal speed. Walking activity was measured with an ambulatory activity monitor. Heart rate, step cadence and walking speed in the test and in daily life were compared. Walking speed in daily life was represented by the intensity of walking. Perceived mobility problems were assessed with the Nottingham Health Profile., Results: Heart rate during walking was lower in the test at self-preferred speed than in daily life (mean difference: 11.3+/-10.4; p=0.001). Self-preferred walking speed in the test and in daily life correlated significantly (r=0.55; p=0.04). In a sub-group with a test performance below the median value, test performance correlated significantly with walking activity. No significant correlation was found between perceived mobility problems and walking activity., Conclusion: Walking in daily life may be more demanding than walking under standardized conditions. Patients with post-poliomyelitis syndrome with the lowest test performance walked less in daily life. Patients do not necessarily match their activity pattern to their perceived mobility problems.

Objective: To assess the reproducibility of walking performance, heart rate, and perceived exertion at self-preferred speed and maximal walking speed in patients with the postpoliomyelitis syndrome (PPS)., Design: Repeated measurement at a 3-week interval., Setting: University hospital., Participants: Convenience sample of 65 patients with PPS., Interventions: Not applicable., Main Outcome Measures: Walking performance: the distance walked in 2 minutes at a self-preferred speed and the time needed to walk 75 m at maximal speed, heart rate, and rating of perceived exertion (RPE) on an 11-point scale., Results: Test-retest reliability of walking performance was excellent for both tests (intraclass correlation coefficient [ICC] range, .94-.97). No systematic differences existed between test and retest. The smallest detectable change for an individual was 15% for both tests. Test-retest reliability for heart rate was good (ICC=.86) but moderate for RPE (Spearman rho range, .67-.70). The smallest detectable change for RPE was between 4 and 6 scale points. The variability in walking performance was significantly correlated with the variability in heart rate at self-preferred speed (r =.36, P <.01) but not with the variability in RPE (r =.20, P =.11)., Conclusions: Both walking tests showed good reproducibility and may be appropriate to monitor (individual) changes in walking capacity in patients with PPS. Because of its moderate reproducibility, RPE does not seem to be suitable to monitor physical exertion. The usefulness of an objective measure such as heart rate for this purpose needs further investigation.

The purpose of this study was to identify optimal ways to detect neurogenic changes with high-density surface electromyography (HD-sEMG). For this purpose, we searched for the variables that most clearly discriminated between postpoliomyelitis and healthy subjects. We obtained HD-sEMG from the quadriceps muscle at different force levels in nine subjects with postpoliomyelitis syndrome and in matched healthy controls. Single motor unit action potentials (MUAPs), extracted from the HD-sEMG signal and the raw signal itself, were analyzed. Areas under the curve of the extracted MUAP waveform, indicating motor unit size, perfectly separated both groups. Raw signal analysis showed significant differences between groups for the monopolarly recorded amplitude up to 60% of maximal force and for the level of interference at higher force levels (40-100% force). We conclude that with HD-sEMG it is possible to detect neurogenic motor unit changes noninvasively, both by analysis of the raw signal itself and by analysis of extracted single MUAPs. The diagnostic yield of the single MUAP analysis is clearly higher. These findings point toward applications for clinical practice and invite further studies exploring the diagnostic value of HD-sEMG., (Copyright 2004 Wiley Periodicals, Inc.)

Objectives: To determine what changes in maximal isometric strength can be detected in a symptomatic quadriceps muscle in patients with postpoliomyelitis syndrome (PPS) and to investigate the association between the variability in maximal strength and maximal voluntary activation (MVA)., Design: Repeated-measures over a 3-week interval., Setting: University hospital., Patients: Convenience sample of 65 patients with PPS., Intervention: Dynamometer testing., Main Outcome Measures: Maximal voluntary contraction (MVC) torque of the quadriceps was measured with a Kin-Com dynamometer and MVA was determined by twitch interpolation., Results: The mean difference between the 2 consecutive measurements was -0.7+/-12.8 Nm (95% confidence interval [CI], -3.9 to 2.5). The test-retest reliability was excellent for MVC torque (intraclass correlation coefficient [ICC]=.96; 95% CI,.93-.98) and moderate for MVA (ICC=.73; 95% CI,.56-.85). The smallest detectable change in MVC torque was 25% for an individual. The variability in MVA explained 18% of the variability in maximal strength., Conclusions: Variability in maximal quadriceps strength, measured with a fixed dynamometer, was large and partly related to variability in MVA. This implies that even with optimally standardized strength testing, a follow-up of many years is required to objectify progression of quadriceps weakness in an individual patient with PPS. To demonstrate changes in strength in groups of patients in follow-up or intervention studies, feasible sample sizes are required.

Objective: To assess the comparability and reproducibility of 4 questionnaires used to measure fatigue in postpoliomyelitis syndrome (PPS)., Design: Repeated-measures at a 3-week interval., Setting: University hospital., Participants: Convenience sample of 65 patients with PPS., Interventions: Not applicable., Main Outcome Measures: The Fatigue Severity Scale (FSS), the Nottingham Health Profile (NHP) energy category, the Polio Problem List (PPL) fatigue item, and the Dutch Short Fatigue Questionnaire (SFQ)., Results: Correlations of scores between questionnaires were all significant (P<.01) and ranged from.43 (between the NHP energy category and the PPL fatigue item) to.68 (between the PPL fatigue item and the SFQ). Scores on the second visit, normalized to a 0 to 100 scale, were: FSS, 78+/-15; NHP energy category, 47+/-35; PPL fatigue item, 81+/-17; and SFQ, 65+/-22. Except for the difference between the FSS and the PPL fatigue item, the differences in scores between the questionnaires were significant (P<.01). Scale analysis indicated that all questionnaires measured the same unidimensional construct. The reproducibility of the FSS, the PPL fatigue item, and the SFQ was moderate. The smallest detectable change was 1.5 points for the FSS, 2.0 points for the PPL fatigue item, and 1.9 points for the SFQ., Conclusions: Although the questionnaires measure the same fatigue construct in PPS, the results are not interchangeable because the ranges of measurement differ. The NHP energy category, in particular, appeared to have a high detection threshold. The moderate reproducibility of the questionnaires indicates a lack of precision, especially when applied at the individual patient level.

Objectives: To investigate the effect of pyridostigmine on fatigue, physical performance, and muscle function in subjects with postpoliomyelitis syndrome., Methods: 67 subjects with increased fatigue and new weakness in one quadriceps muscle showing neuromuscular transmission defects, were included in a randomised, double blind, placebo controlled trial of 60 mg pyridostigmine four times a day for 14 weeks. Primary outcome was fatigue (on the "energy" category of the Nottingham health profile). Secondary outcomes included two minute walking distance and quadriceps strength and jitter. Motor unit size of the quadriceps was studied as a potential effect modifier. The primary data analysis compared the changes from baseline in the outcomes in the last week of treatment between groups., Results: 31 subjects treated with pyridostigmine and 31 subjects treated with placebo completed the trial. No significant effect of pyridostigmine was found on fatigue. The walking distance improved more in the pyridostigmine group than in the placebo group (by 7.2 m (6.0%); p<0.01). Subgroup analysis showed that a significant improvement in walking performance was only found in subjects with normal sized motor units. Quadriceps strength improved more in the pyridostigmine group than in the placebo group (by 6.7 Nm (7.2%); p = 0.15). No effect of pyridostigmine was found on jitter., Conclusions: Pyridostigmine in the prescribed dose did not reduce fatigue in subjects with postpoliomyelitis syndrome. However, it may have a limited beneficial effect on physical performance, especially in subjects with neuromuscular transmission defects in normal sized motor units.

Quadriceps strength, maximal anatomical cross-sectional area (CSA), maximal voluntary activation (MVA), and maximal relaxation rate (MRR) were studied in 48 subjects with a past history of polio, 26 with and 22 without postpoliomyelitis syndrome (PPS), and in 13 control subjects. It was also investigated whether, apart from CSA, MVA and MRR were determinants of muscle strength. Polio subjects had significantly less strength, CSA, and MRR in the more-affected quadriceps than control subjects. MVA was reduced in 18 polio subjects and normal in all controls. PPS subjects differed from non-PPS subjects only in that the MVA of the more-affected quadriceps was significantly lower. Both CSA and MVA were found to be associated with muscle strength. Quadriceps strength in polio subjects was dependent not only on muscle mass, but also on the ability to activate the muscles. Since impaired activation was more pronounced in PPS subjects, the new muscle weakness and functional decline in PPS may be due not only to a gradual loss of muscle fibers, but also to an increasing inability to activate the muscles.

Objectives: To study prospectively the health status, and especially the physical functioning, of polio survivors with and without postpoliomyelitis syndrome (PPS), and to identify prognostic determinants of change in physical functioning., Design: Prospective cohort study; measurements at baseline and after 1, 2, and 6 years., Setting: University hospital in the Netherlands., Participants: Seventy-six subjects with PPS and 27 without PPS., Interventions: Not applicable., Main Outcome Measure: The Nottingham Health Profile (NHP) physical mobility category., Results: Subjects with PPS had significantly poorer health status than subjects without PPS. No significant differences in mean NHP physical mobility scores between baseline and 6 years were found; both groups had improved after 1 year, after which there was a slow decline over the next 5 years. During the first 2 years, strength measurements showed little decline that was not related to changes in NHP physical mobility score. A physical performance test revealed no mean change in the first 2 years, but the subgroup with a decline above the 75th percentile eventually deteriorated 10.5+/-16.3 points on the NHP physical mobility category (P=.01) at 6 years from baseline. This subgroup had more extensive paresis than the other subjects, although it was not significant (P=.07). The extent of paresis at baseline was the only prognostic determinant for an increase in NHP physical mobility problems after 6 years., Conclusions: Subjects with and without PPS did not differ with regard to changes in health status in a 6-year period. The fact that the extent of paresis was a prognostic factor for a decline in physical functioning is in accord with a (slow) decline in muscle mass, as a late effect of polio, that may lead to a decline in physical functioning as the reduced muscle capacity becomes less able to meet the demands of daily physical activities.

Objectives: To compare the submaximal exercise capacity of polio subjects with postpoliomyelitis syndrome (PPS) and without (non-PPS) with that of healthy control subjects, to investigate the relationship of this capacity with maximal short-term power and quadriceps strength, and to evaluate movement economy., Design: Cross-sectional survey., Setting: University hospital., Participants: Forty-three polio subjects (25 PPS, 18 non-PPS) and 12 control subjects., Interventions: Not applicable., Main Outcome Measures: Power output, oxygen uptake, and heart rate were measured in an incremental submaximal cycle ergometry test. Maximal short-term power was measured in 5-second all-out efforts. Knee extensor strength was measured on a chair dynamometer., Results: The mean submaximal power +/- standard deviation at 80% of heart rate reserve of 83.8 +/- 29.9 watts in the polio subjects was significantly less than the mean submaximal power of 142.1 +/- 30.4 watts in the control group. However, expressed as a percentage of the maximal short-term power, submaximal power did not differ between the groups. Strength and maximal short-term power correlated significantly (p < .005) with submaximal power (r = .64 and .76, respectively). The oxygen uptake was higher than theoretically expected for the given submaximal power output in polio subjects, and appeared to increase with increasing asymmetry in strength and power between legs. No differences were found between PPS and non-PPS subjects., Conclusion: The submaximal work capacity of polio subjects was severely reduced, mainly in association with the reduced muscle capacity. And, because of a reduced movement economy, their energy cost was elevated. Although muscle loads in activities such as walking and climbing stairs differ from cycling, they also may require elevated relative levels of effort, predisposing subjects to premature fatigue in sustained activity., (Copyright 2001 by the American Congress of Rehabilitation Medicine and the American Academy of Physical Medicine and Rehabilitation)

Objectives: To investigate the validity, the intraexaminer and interexaminer reproducibility, and the ability to detect change of a hand-held dynamometer (HHD) in strength measurements in former polio subjects., Design: HHD measurement of knee extensor strength was compared with the criterion standard of a chair dynamometer measurement in 49 subjects. The "break" method was used for HHD measurements. Reproducibility was studied for six lower extremity muscle groups in 28 subjects. The measurements were performed by one experienced and one inexperienced examiner on two separate occasions, with an interval of 1 week. The examiners were blinded to each other's and to previous results., Setting: University hospital., Subjects: Volunteer sample of former polio subjects., Results: For knee extension, the forces that could be measured with the HHD were limited to approximately 200N. Although the intraclass correlation coefficients were high (.75 to .98), the 95% limits of agreement between measurements showed large intervals for differences between two measurements (ratio intervals ranging from .76-1.52 to .52-2.77). The intraexaminer reproducibility for the experienced examiner was superior to that of the inexperienced examiner. The reproducibility of the inexperienced examiner showed systematic bias, with significantly higher strength values for the second session measurement of three muscle groups., Conclusion: The device has good validity in the lower force range. However, because the agreement between measurements was poor, it has limited ability to detect a change in muscle strength. Therefore, this method is unable to detect small changes in lower extremity muscle strength in former polio patients.

Objective: To investigate the prevalence of new neuromuscular symptoms, disabilities, and handicaps in a group of polio survivors., Design: A self-constructed health questionnaire about neuromuscular complaints and disability and handicap levels during the stable period after recovery from polio and at present., Subjects: Three hundred fifty subjects, derived from the 1,784 polio cases registered during the 1956 polio outbreak in The Netherlands., Results: Respondents totaled 260 (74%), 27 of whom denied or did not recall having had paralytic poliomyelitis. The remaining 233 subjects comprised the study group (mean age, 44yrs; range, 39 to 77; SD = 6.3). Frequency of all neuromuscular complaints at present time was significantly higher than that during the stable period after polio (range in p of .001 to .004). Fifty-eight percent of cases reported an increase in muscle weakness in comparison with muscle condition during the stable period. Fifty-six percent reported an increase in disabilities, mainly a restriction in gait functions. Fifty-three percent reported increased handicaps with regard to occupation and social integration, and there was an increased need for adaptive measures and devices., Conclusion: Nearly 60% of a sample of Dutch survivors of the 1956 polio outbreak experience late onset polio sequelae, resulting in increased severity of disabilities and handicaps.

Objectives: To compare perceived health problems and disability in former polio subjects with postpolio syndrome (PPS) and those without postpolio syndrome (non-PPS), and to evaluate perceived health problems, disability, physical performance, and muscle strength., Design: Cross-sectional survey; partially blinded data collection., Subjects: One hundred three former polio subjects, aged 32 to 60yrs. This volunteer sample came from referrals and patient contacts. Criterion for PPS: new muscle weakness among symptoms., Main Outcome Measures: Nottingham Health Profile (NHP), adapted D-code of the International Classification of Impairments, Disabilities and Handicaps, performance test, and muscle strength assessment., Results: PPS subjects (n = 76) showed higher scores (p < .001) than non-PPS subjects (n = 27) within the NHP categories of physical mobility, energy, and pain. On a 16-item Polio Problems List, 78% of PPS subjects selected fatigue as their major problem, followed by walking outdoors (46%) and climbing stairs (41%). The disabilities of PPS subjects were mainly seen in physical and social functioning. No differences in manually tested strength were found between patient groups. PPS subjects needed significantly more time for the performance test than non-PPS subjects and their perceived exertion was higher. Perceived health problems (NHP-PhysMobility) correlated significantly with physical disability (r = .66), performance-time (r = .54), and muscle strength (r = .38). With linear regression analysis, 54% of the NHP-PhysMobility score could be explained by the performance test (time and exertion), presence of PPS, and muscle strength, whereas strength itself explained only 14% of the NHP-PhysMobility score., Conclusions: PPS subjects are more prone to fatigue and have more physical mobility problems than non-PPS subjects. In former polio patients, measurements of perceived health problems and performance tests are the most appropriate instruments for functional evaluation.