Validity of Cardiopulmonary Exercise Testing for Assessing Aerobic Capacity in Neuromuscular Diseases.

OBJECTIVES: To determine the content validity of cardiopulmonary exercise testing (CPET) for assessing peak oxygen uptake (VO2peak) in neuromuscular diseases (NMD). DESIGN: Baseline assessment of a randomized controlled trial. SETTING: Academic hospital. PARTICIPANTS: Eighty-six adults (age: 58.0±13.9 y) with Charcot-Marie-Tooth disease (n=35), postpolio syndrome (n=26), or other NMD (n=25). INTERVENTION: Not applicable. MAIN OUTCOME MEASURES: Workload, gas exchange variables, heart rate, and ratings of perceived exertion were measured during CPET on a cycle ergometer, supervised by an experienced trained assessor. Muscle strength of the knee extensors was assessed isometrically with a fixed dynamometer. Criteria for confirming maximal cardiorespiratory effort during CPET were established during 3 consensus meetings of an expert group. The percentage of participants meeting these criteria was assessed to quantify content validity. RESULTS: The following criteria were established for maximal cardiorespiratory effort: a plateau in oxygen uptake (VO2plateau) as the primary criterion, or 2 of 3 secondary criteria: (1) peak respiratory exchange ratio (RERpeak) ≥1.10 (2), peak heart rate ≥85% of predicted maximal heart rate; and (3) peak rating of perceived exertion (RPEpeak) ≥17 on the 6-20 Borg scale. These criteria were attained by 71 participants (83%). VO2plateau, RERpeak ≥1.10, peak heart rate ≥85%, and RPEpeak ≥17 were attained by 31%, 73%, 69%, and 72% of the participants, respectively. Peak workload, VO2peak, and knee extension muscle strength were significantly higher, and body mass index was lower (all P<.05), in participants with maximal cardiorespiratory effort than other participants. CONCLUSIONS: Most people with NMD achieved maximal cardiorespiratory effort during CPET. This study provides high quality evidence of sufficient content validity of VO2peak as a maximal aerobic capacity measure. Content validity may be lower in more severely affected people with lower physical fitness.

Updated size index valid for both neurogenic and myogenic changes.

BACKGROUND: Size index (SI) is a motor unit potential (MUP) parameter in concentric needle electromyography calculated from amplitude and area/amplitude, which can sensitively discriminate between control and neurogenic MUPs. In this study, we investigated the application of SI to myogenic MUPs based on expanded data. METHODS: MUPs were collected from the biceps brachii (BB) and tibialis anterior (TA) muscles. Muscles showing unequivocal neurogenic or myogenic changes by visual inspection were selected for patients. In addition to the original SI, a revised SI (rSI) was defined using the logarithmic scale for area/amplitude. The coefficient for area/amplitude was varied and that achieving the best sensitivity both for BB and TA was selected. RESULTS: Analyzed were 1619, 340, and 498 MUPs from the BB of 26, 10, and 14 subjects (control, neurogenic, and myogenic), respectively, and 1245, 536, and 473 MUPs from the TA of 23, 18, and 13 subjects (control, neurogenic, and myogenic), respectively. For neurogenic MUPs, the original SI and the newly defined rSIn were similarly sensitive (82.1% and 81.8% sensitivity for SI and rSIn, respectively, for BB, and 68.1% and 69.6% for TA), and were more sensitive than area (72.6% for BB and 57.6% for TA), the most sensitive parameter among conventional ones. For myogenic MUPs, the sensitivity of rSIm was 9.0% for BB and 24.5% for TA, which was not significantly different from duration (7.4% for BB and 21.8% for TA), the most sensitive parameter among conventional ones. CONCLUSIONS: SI, rSIn, and rSIm are promising as new MUP parameters.

The Perception of Physical Activity in Ambulatory Persons with Late Effects of Polio: A Qualitative Study.

Maintaining regular physical activity (PA) can be challenging for persons with late effects of polio. This qualitative study of ambulatory persons with late effects of polio explored their perceptions of PA, as well as facilitators of and barriers to PA. Semistructured interviews were conducted with 15 persons and analyzed with content analysis using the International Classification of Functioning, Disability and Health (ICF) as a framework. The participants described positive perceptions of PA and its health benefits. PA was used to prevent further decline in functioning, and the type and frequency of activities had changed over time. Past experiences and personal characteristics impacted PA. Support from close relatives, knowledgeable health care professionals, mobility devices, and accessible environments facilitated PA, whereas impairments, inaccessible environments, and cold weather were the main barriers. To perform PA regularly, persons with late effects of polio may benefit from individualized advice based on their disability and personal and environmental factors.

Post-polio syndrome. Cases report and review of literature.

It is estimated that around 15 million people survived polio infection worldwide since early twentieth century. In 1950 effective vaccination was used for first time. Since that time number of affected people decreased. The last epidemic of Haine-Medine disease in Poland was in 1950s. Another rare cases of infections were observed till 1970s. About at least 15 years after polio virus infection, slowly progressive muscle limbs paresis with muscle atrophy, joints pain, paresthesia were observed in polio survivors. That constellation of symptoms was called post-polio syndrome (PPS). PPS frequency among people after paralytic and nonparalytic polio infectious is ranged from 30% to 80%. Fatigue that leads to physical and mental activity deterioration is another important symptom that is observed in 90% of patients with PPS. Etiology of disease remains elusive. Probably it is an effect of spine frontal horns motoneurons damage during acute virus polio infection that leads to overloading and degeneration of remaining ones. The most important risk factors of PPS are female sex and respiratory symptoms during acute polio infection. Electromyography is an important part of PPS diagnostic process. Electrophysiological abnormalities are seen in clinically affected and unaffected muscles. The most frequent are fasciculations and fibrillations during rest activity, extension of motor unit area, time duration and amplitude. In this study we described three cases of people who developed PPS years after Haine-Medine disease and correlation between their EMG results and clinical status. We also analyzed electromyography results both after one month since first PPS signs occurred as well as after few years. Presentation of dynamic changes in EMG was the most important aim of that study.

Survey of young patients with polio and a foreign background at a Swedish post-polio outpatient clinic.

Nowadays, polio survivors aged under 60 years are non-native Swedes which pose new aspects and challenges to a post-polio outpatient clinic. To analyze the medical data, walking aids, occupational, and family situation in non-native polio survivors aged less than 60 years at a Swedish post-polio outpatient clinic. Retrospective data analysis. Data were retrieved from medical records at the post-polio outpatient clinic. Actual age, age at acute polio infection, walking capacity, pain, concomitant diseases, working and family situation, and ethnical origin were analyzed. Data are presented in numbers and percentage. 153 patients were included. Mean age was 45 (17-60) years, and mean age at acute polio infection was 2 (0-12) years. Paresis of the lower extremities was the most common disability. 10 % were wheelchair dependent. Pain occurred in 70 % with a mean intensity of 55 measured with the visual analog scale. Hypertension was the most common concomitant disease. Half of the polio survivors were working at least part time, and roughly half were singles. Data were comparable with data earlier published in Swedish native polio survivors. Non-native polio survivors aged under 60 years showed similarities in age at acute polio infection, paresis, prevalence, and intensity of pain when compared with native Swedish polio survivors. They were, however, younger, and were less often working and married/cohabitants than native Swedish polio survivors. The results of this study underline the importance of social and vocational rehabilitation tailoring rehabilitation suitable for polio survivors with a foreign background.

Falls among adults aging with disability.

OBJECTIVE: To investigate the prevalence of and risk factors for falling among individuals aging with multiple sclerosis (MS), muscular dystrophy (MD), postpolio syndrome (PPS), and spinal cord injury (SCI). DESIGN: Cross-sectional survey data from 2009 to 2010 were analyzed. We used forward logistic regression models to examine whether risk factors such as age, sex, mobility level, years since diagnosis, vision, balance, weakness, number of comorbid conditions, and physical activity could distinguish participants who reported falling from those who did not. SETTING: Surveys were mailed to community-dwelling individuals who had 1 of 4 diagnoses (MS, MD, PPS, or SCI). The survey response rate was 91%. PARTICIPANTS: A convenience sample of community-dwelling individuals (N=1862; age, 18-94y) with MS, MD, PPS, or SCI in the United States. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURE: Self-reported fall within the last 6 months. RESULTS: Fall prevalence for people with MS (54%), MD (70%), PPS (55%), and SCI (40%). Across all 4 groups, fall rates peaked in middle age (45-64y) and among people with moderate mobility limitations. Seven risk factors differentiated participants who fell from those who did not: mobility level, imbalance, age, curvilinear age (age(2)), number of comorbid conditions, duration of diagnosis, and sex. The models differed across diagnostic groups. CONCLUSIONS: People aging with long-term physical disabilities experience unique challenges that affect their risk of falls. A better understanding of the frequency, severity, and risk factors of falls across diagnostic groups is needed to design and implement customized, effective fall prevention and management programs for these individuals.

Polio survivors' perceptions of the meaning of quality of life and strategies used to promote participation in everyday activities.

INTRODUCTION: The term 'post-polio syndrome' (PPS) is used to describe new and late manifestations of poliomyelitis that occur later in life. Research in this area has focused upon health status rather than its effect on quality of life. AIM: To gain an in-depth understanding of the meaning of quality of life for polio survivors and to determine the type of strategies that are used by people with PPS and the support that they consider as important to facilitate participation in everyday life activities that have an impact on their quality of life. METHOD: Six focus groups were conducted with 51 participants from two regions in England. Data were audio-taped and analysed using thematic analysis. RESULTS: Our research found that polio survivors used terms used to describe quality of life which could be associated with that of happiness. Our research has identified resolvable factors that influence quality of life namely inaccessible environments, attitudes of health-care professionals and societal attitudes. Polio survivors have tried alternative therapies, chiefly acupuncture and massage, and found them to be effective in enhancing their quality of life. CONCLUSION: It is suggested that health-care professionals should consider factors which influence happiness and implement a person-centred approach with the views of the polio survivor being listened to. The three factors that influenced quality of life could be resolved by health-care professionals and by society. With regard to strategies used, we suggest that polio survivors should have access to the treatments that they perceive as important, although further research is required to design optimal interventions for this client group.

Physical Activity and the Association With Self-Reported Impairments, Walking Limitations, Fear of Falling, and Incidence of Falls in Persons With Late Effects of Polio.

The purpose of this study was to determine the association between physical activity and self-reported disability in ambulatory persons with mild to moderate late effects of polio (N = 81, mean age 67 years). The outcome measures were: Physical Activity and Disability Survey (PADS), a pedometer, Self-Reported Impairments in Persons with Late Effects of Polio Scale (SIPP), Walking Impact Scale (Walk-12), Falls Efficacy Scale-International (FES-I), and self-reported incidence of falls. The participants were physically active on average 158 min per day and walked 6,212 steps daily. Significant associations were found between PADS and Walk-12 (r = -.31, p < .001), and between the number of steps and SIPP, Walk-12, and FES-I (r = -.22 to -.32, p < .05). Walk-12 and age explained 14% of the variance in PADS and FES-I explained 9% of the variance in number of steps per day. Thus, physical activity was only weakly to moderately associated with self-reported disability.

Post poliomyelitis syndrome: A rare sequel of acute poliomyelitis.

Post poliomyelitis syndrome (PPS) is a rare sequel of acute poliomyelitis, usually seen 30-40 years after an acute episode. It is characterized by new muscle weakness seen in survivors of acute poliomyelitis. We describe a rare case of a 50 year old man; with a previous history of poliomyelitis in right lower limb who now presented with complaints of progressive left lower limb weakness for past two years. The diagnosis was made on the basis of clinical suspicion and EMG findings. PPS is not a well recognized disease in Pakistan and due to the lack of documentation; its true prevalence is not known. Though, over the years, cases of Poliomyelitis have decreased worldwide, however, PPS still remains a constant challenge for the physicians. This report highlights the impact of the disease on the quality of life of patients suffering from PPS and emphasis on the need for new therapeutic approach.

Determining the anaerobic threshold in postpolio syndrome: comparison with current guidelines for training intensity prescription.

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.

Neurobiological modulation with REAC technology: enhancing pain, depression, anxiety, stress, and quality of life in post-polio syndrome subjects.

Post-polio syndrome (PPS) brings new challenges for polio survivors, including muscle decline, pain, depression, and diminished quality of life. This study explored the potential of REAC neuromodulatory treatments to ease pain, improve mood, and enhance quality of life in PPS patients. 17 individuals with PPS (average age 54.8) received three REAC treatments: Neuro Postural Optimization, Neuro Psycho Physical Optimization, and Neuro Psycho Physical Optimization-Cervico Brachial. Pain, depression, anxiety, stress, and quality of life were assessed before and after using established scales. REAC treatments significantly reduced pain across various dimensions, along with depression, anxiety, and stress levels. Additionally, patients reported improved physical and psychological quality of life. This study suggests REAC neuromodulatory treatments as a promising non-invasive option to improve pain, emotional well-being, and quality of life in individuals with PPS.

Post-polio syndrome - somatosensory dysfunction and its relation to pain: a pilot study with quantitative sensory testing.

OBJECTIVE: To explore and characterize somatosensory dysfunction in patients with post-polio syndrome and chronic pain, by conducting examinations with Quantitative Sensory Testing. DESIGN: A cross-sectional, descriptive, pilot study conducted during 1 month. SUBJECTS/PATIENTS: Six patients with previously established post-polio syndrome and related chronic pain. METHODS: All subjects underwent a neurological examination including neuromuscular function, bedside sensory testing, a thorough pain anamnesis, and pain drawing. Screening for neuropathic pain was done with 2 questionnaires. A comprehensive Quantitative Sensory Testing battery was conducted with z-score transformation of obtained data, enabling comparison with published reference values and the creation of sensory profiles, as well as comparison between the study site (more polio affected extremity) and internal control site (less affected extremity) for each patient. RESULTS: Derived sensory profiles showed signs of increased prevalence of sensory aberrations compared with reference values, especially Mechanical Pain Thresholds, with significant deviation from reference data in 5 out of 6 patients. No obvious differences in sensory functions were seen between study sites and internal control sites. CONCLUSION: Post-polio syndrome may be correlated with a mechanical hyperalgesia/allodynia and might be correlated to a somatosensory dysfunction. With lack of evident side-to-side differences, the possibility of a generalized dysfunction in the somatosensory system might be considered.

Self-reported depression and physical activity in adults with mobility impairments.

OBJECTIVE: To test hypothesized associations between depression and physical activity among adults with multiple sclerosis (MS), spinal cord injury (SCI), muscular dystrophy (MD), and postpolio syndrome (PPS). DESIGN: Cross-sectional survey. SETTING: Survey responses collected from individuals in the Washington state area (participants with SCI) and across the United States (participants with MS, MD, and PPS). PARTICIPANTS: Convenience sample of participants were surveyed (N=1676; MD, n=321; PPS, n=388; MS, n=556; SCI, n=411). INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: The Patient Health Questionnaire-9 (PHQ-9) assessing depressive symptoms and the International Physical Activity Questionnaire (IPAQ) and Godin Leisure Time Exercise Questionnaire (GLTEQ) assessing physical activity. RESULTS: The average age was 56 years, 64% were women, 92% were white, 86% had a high school degree or higher, and 56% walked with an assistive device or had limited self-mobility. The IPAQ and GLTEQ explained a small but statistically significant and unique amount of the variance in PHQ-9 scores in all diagnostic groups, with no significant differences in the relation by condition, age, or mobility status (IPAQ R(2)=.004; GLTEQ R(2)=.02; both P<.02). CONCLUSIONS: Both physical activity measures demonstrated a small but statistically significant association with depression in all 4 diagnostic groups. Research is needed to determine longitudinal relations and whether physical activity interventions could promote improved mood in adults with physical disabilities.

Living with polio and postpolio syndrome in the United Kingdom.

The term Postpolio Syndrome (PPS) is used to describe new and late manifestations of poliomyelitis that occur later in life in polio survivors. Polio had been eradicated in the United Kingdom (UK) and most of Europe, although this is not the case in all countries. Research in this area has tended to focus upon the impact of polio and PPS on health status and functional health rather than its overall effect on people's lives. This study's two main aims were to explore the ways in which polio and PPS in the UK has affected the respondents' lives and to ascertain their views about how the quality of life could be improved. The two questions were as follows: (1) How has the health of people with polio and PPS affected their quality of life? (2) What would people with polio and PPS change to improve their quality of life? Deductive content analysis using existing qualitative data from a cross-sectional survey of 336 returned questionnaires from persons with polio and PPS was carried out. The average age of the participants was 54 years. Our research found that polio survivors valued social occupations and participation in family life. Our research has also shown that healthcare professionals still do not understand polio and PPS and this lack of understanding influences their clients' quality of life. Finances and accessibility of environments also influence participation in chosen occupations. Rehabilitation programmes for people with polio and PPS need to be targeted towards maintaining and improving accessible environments and participation in chosen occupations, and healthcare professionals need to ensure that persons with polio and PPS are referred to persons with specific expertise in this area.

Anesthetic implications of postpolio syndrome: new concerns for an old disease.

Poliomyelitis was pandemic in the United States and much of the world in the first half of the 20th century. The uses of polio vaccines have essentially eradicated the disease in the United States today. But poliovirus infection survivors who had experienced a paralytic attack can see a return of some symptoms, which is a syndrome called postpolio syndrome (PPS). The anesthetist must preoperatively assess reported amounts of patient physical activity and patient age, which can indicate the amount of muscle degeneration that may have already occurred. Patients with PPS demonstrate altered respiratory function, cold intolerance, a risk for aspiration, and experience chronic pain in muscles and joints. Patients with PPS display an increased sensitivity to some anesthetic agents such as long-acting narcotics and potent inhaled anesthetic gases with a high blood-gas partition coefficient, along with report of increased fatigue, weakness, and somnolence after anesthesia. Anesthesia care must center on the preservation of muscle function postoperatively. The anesthetist should consider the use of short-acting anesthetic agents, increased doses of analgesics, the use of warming devices, and careful attention to patient positioning. Prolonged postoperative care and hospital admission after surgery are possible.

Functioning of the upper extremity in persons with late polio.

BACKGROUND: There are a number of people living with late effects of polio, but the extent of engagement with the upper extremity is unclear. The objective is to describe the polio involvement in persons attending the polio clinic and to assess the perceived problems in self-reported arm/hand function. MATERIAL AND METHODS: A 1-year sample of consecutively examined community-dwelling people at the polio clinic. Electromyography and muscle strength were assessed as part of the clinical examination. A questionnaire regarding perceived problems in different activities involving arm/hand function was sent out prior to appointment and brought to the clinic. RESULTS: A total of 186 persons were seen, and the majority was women (65%). The average age at examination was 60 years (SD 14), and the acute polio illness had occurred at 5 years of age (SD 7). Post-polio syndrome was present in 96%. Polio involvement in the upper extremities was seen in the majority, and this was often clinically unstable. Perceptions of problems in arm/hand activities did not always correlate with having had polio in the upper extremity or with mode of mobility. However, the grip force correlated significantly with the number of perceived problems. Bi-manual activities were more often perceived problematic. CONCLUSIONS: Polio involvement in the upper extremity is very common. There were no obvious correlations with the objective findings of polio and perceived problems. The polio survivors' adaptation requires more questions and assessments from the professional team to identify those with a possible need for intervention.

A brief history of postpolio syndrome in the United States.

This is an overview of the history of the late effects of polio in this country from 1980 to the present in the context of the broader and much longer history of acute poliomyelitis. Books, articles, conference proceedings, and other relevant historical resources that dealt with polio-related issues from January 1, 1980, through December 31, 2009, were reviewed. The mean number of articles published per year was calculated for 5-year intervals beginning in 1980; the number of postpolio support groups and polio-dedicated clinics was compiled from directories published annually by Post-Polio Health International at 5-year intervals from 1985 to 2010. Beginning in the mid-1980s, the number of articles published each year increased dramatically, peaking during the years 1995 to 1999 when a mean of 48.2 articles were published each year. This figure steadily declined over the next 14 years. Support groups and clinics showed a similar pattern of rise and fall, with a maximum of 298 support groups and 96 clinics in 1990 and a decline to 131 and 32, respectively, by 2010. During the 1980s and early 1990s, there was a period of optimism that energized research, clinical, and self-help initiatives. As the limits of these efforts became apparent during the late 1990s and early 2000s, resources and activities declined as the postpolio community continued to age and decrease in size. Regardless of these trends, there are still thousands of survivors who continue to require skilled physiatric management as they cope with advancing age and declining function.

Approach to Fatigue and Energy Conservation.

Fatigue, a common complaint in individuals with postpolio syndrome (PPS), is defined as an overwhelming sustained feeling of exhaustion and diminished capacity for physical and mental work. A comprehensive medical work-up is needed to rule out all other causes of fatigue. A sleep study should be considered for individuals with PPS who complain of fatigue. Self-reported outcome measures, such as the Fatigue Severity Scale, are reliable and valid tools to measure fatigue in this population. Fatigue management consists of individualized treatment of underlying medical conditions, energy conservation, pacing techniques, and lifestyle modifications.

Palliative Care for Polio and Postpolio Syndrome.

Palliative care is a team-based approach focusing on relief of physical, psychosocial, and existential distress and communication about serious illness. Patients with poliomyelitis and postpolio syndrome are at risk for contractures and can benefit from involvement of physical and occupational therapy. Hypersialorrhea can be treated with anticholinergic medications, botox, or radiation. Patients with dyspnea may require noninvasive positive pressure ventilation ± opioids or benzodiazepines. Constipation is often due to autonomic dysfunction and decreased mobility. There is a higher burden of anxiety. Early conversations about patients' goals and values as it relates to their health may help frame future decision-making.

Nerve Conduction Study/Electromyography: Common Neuropathies and Considerations for Patients with Polio.

Acute poliomyelitis is now extremely rare in the United States. Worldwide there are still sporadic outbreaks, which are typically treated with acute inoculation programs. Although polio has effectively been eradicated, the full scope of the disease and its myriad manifestations both in the acute phase and in the postpolio syndrome phase, remain areas of fertile research, debate, and stimulating topics.