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A 73-year-old man presented with muscle weakness and atrophy of his right arm. Atrophy of his left brachia and left calf had occurred 13 years before without any improvement or deterioration. His sister and cousin had a history of paralytic poliomyelitis. Serum poliovirus type 2 neutralizing antibody was elevated to 128×. Electromyography revealed chronic denervation potentials not only in the muscles affected previously but also in the unaffected muscles. Acute and chronic denervation potentials were found in the newly affected muscle. Postpolio syndrome should be considered in patients with unilateral muscular atrophy even when they have no history of paralytic poliomyelitis.

Objective. Many patients with postpolio syndrome (PPS) experience pain. In this study, we aimed to review previous studies to investigate the effectiveness of intravenous immunoglobulin (IVIG) for managing pain in patients with PPS. We performed a narrative review. Methods. In PubMed, we searched for the keywords ((Immunoglobulin OR IVIG) AND (poliomyelitis OR poliomyelitis syndrome)). We included articles in which IVIG was infused in patients with PPS and pain severity was measured before and after treatment. Results. In the results, five articles (4 randomized controlled trials and 1 prospective observational study) were included in this review. Four of the studies reported that IVIG had a positive pain-reducing effect in patients with PPS. In addition, 4 studies evaluated the outcomes related to muscle strength and function. Of these studies, 3 showed some improvement in measurements for muscle strength and function. Conclusion. In conclusion, IVIG might be one of the beneficial options for managing pain in PPS. Pain reduction might be responsible for the improvement of muscle strength or function. To confirm the benefits of IVIG in reducing pain, more high-quality studies are required.

Objective: To compare the effects of an upper limb videogame-based training with a training based on similar motor demands on upper limb function, dexterity, functionality, balance, fatigue, and pa...

OBJECTIVE Obesity is a major and functionally important problem in polio survivors. The aim of this study was to investigate the prevalence of obesity using body mass index and percentage body fat in polio survivors and to analyze the relationship between obesity and mobility. DESIGN Eighty-four polio survivors were included. Anthropometric parameters, knee extensor strength, and the Short Physical Performance Battery were evaluated. A questionnaire was used to explore the late effects of poliomyelitis. Obesity was determined using both body mass index and percentage body fat. RESULTS The prevalence of obesity in polio survivors was 39.3% and 81.5% using the body mass index and percentage body fat criteria, respectively. The Short Physical Performance Battery scores were significantly different between the obese and nonobese groups as determined by percentage body fat (P < 0.05). Only percentage body fat was significantly associated with mobility after controlling for the confounding variables in obese polio survivors (P < 0.05). CONCLUSIONS Obesity in polio survivors was underestimated when the body mass index criteria were used. Percentage body fat was a significantly associated factor for mobility in obese polio survivors. Obesity determined by percentage body fat criteria is useful to address obesity-related problems in polio survivors.

Objective: Aging with physical disability disrupts one’s ability to achieve valued goals due to changes in symptoms and function. It is unclear how to cope optimally in this context. This study examined whether two possible strategies—tenacious goal pursuit (TGP) and flexible goal adjustment (FGA)—were associated with reduced pain interference and depressive symptoms and greater well-being, and protected against pain intensity, and FGA was more protective with increasing age and worse physical function. Method: Middle-aged adults with muscular dystrophy, multiple sclerosis, post-polio syndrome, or spinal cord injury ( N = 874; MAGE = 58.3 years, range = 46-68; MDISEASEDURATION = 26.2 years, range = 2-67) completed two questionnaires, a year apart. Results: TGP and FGA use was associated with greater well-being. FGA use predicted decreased depressive symptoms. Concurrent use of both predicted decreased pain interference. Discussion: Adults with disability employ a variety of goal management strategies. Findings support TGP and FGA as potential intervention targets for healthy aging with disabilities.

Background Resilience is a psychological construct referring to one’s positive adaptation in response to adversity. Evidence suggests that resilience may contribute to various function domains in adults with chronic physical disabilities. Purpose To test hypothesized temporal associations between resilience and four function domains (anxiety, depression, social role satisfaction, and physical function) in individuals with chronic physical disabilities. Methods Participants were 1,574 adults with one of four chronic physical disabilities (spinal cord injury, muscular dystrophy, multiple sclerosis, or postpolio myelitis syndrome) who were participating in a large, ongoing USA-based longitudinal survey study. Three surveys were mailed on an approximately yearly basis. Resilience was assessed using the Connor–Davidson Resilience Scale 10-item (CDRSC-10) and each function domain was assessed using the respective Patient Reported Outcome Measurement System (PROMIS) short-form. Results Cross-lagged path models evidenced statistically significant reciprocal relationships between resilience and each function domain except physical function. The standardized lagged coefficients corresponding to resilience predicting social role satisfaction (T1–T2 = 0.09, T2–T3 = 0.09) had similar effect sizes as those corresponding to social role satisfaction predicting resilience (T1–T2 = 0.11, T2–T3 = 0.04), although resilience was a slightly stronger predictor in the second lag. In models assessing psychological function, resilience was a stronger predictor of later psychological function (resilience-to-anxiety, T1–T2 = −0.15, T2–T3 = −0.11; resilience-to-depression, T1–T2 = −0.21, T2–T3 = −0.13) than the inverse (anxiety-to-resilience, T1–T2 = −0.11, T2–T3 = −0.06; depression-to-resilience, T1–T2 = −0.12, T2–T3 = −0.05). Conclusions The study findings suggest that resilience is a significant prospective predictor of psychological and social function over time in individuals with chronic physical disabilities.

BACKGROUND: Sense of Coherence (SOC) is important for successful adaptation in persons with life-long disabilities. A previous study has shown that persons with Late Effects of Polio (LEoP) have a level of SOC indicating that they generally have the ability to understand, handle and being motivated when dealing with stressful events and problems arising in their lives. However, no study has explored the associations between SOC, sociodemographics and disability related factors in persons with LEoP.OBJECTIVE: To explore the associations between SOC, sociodemographics and disability related factors in persons with LEoP.DESIGN: A cross-sectional design.SETTING: University hospital outpatient clinic.STUDY PARTICIPANTS: Ninety-three persons (mean age 74 years, 52% women) with LEoP.MAIN OUTCOME MEASUREMENTS: Swedish versions of the Sense of Coherence scale (SOC-13), Self-reported Impairments in Persons with late effects of Polio (SIPP) scale, Reintegration to Normal Living Index (RNL-I) (subscales "Daily functioning" and "Perceptions of Self") and Satisfaction With Life Scale (SWLS).METHODS: The participants responded to a postal survey with background information and the four rating scales. To determine factors associated with SOC, a hierarchical regression analysis was performed with SOC as the dependent variable.RESULTS: Higher age, less bothered by symptoms of LEoP, better perceptions of self and higher life satisfaction were significantly associated with a greater SOC score. The final model explained 60% of the variance in the SOC (R2 Adj= 0.60, p

MUNIX method (Motor Unit Number Index) had been not used to assess number of motor neurons in post-polio syndrome in contrary to needle electromyography.To confirm if MUNIX reflects motor unit loss and clinical stage and to assess difference in MUNIX and EMG results between muscles in different stage.132 Muscles (MUNIX) and 96 (EMG) in 12 patients were studied and divided into groups: with normal strength(N), stable weakness and atrophy(S), new weakness and atrophy(W).In PPS group MUNIX global was 561.36 ± 282.6 (right 6 muscles) and 561.27 ± 281.1 (left) significantly lower than in control group (six muscles 1139.6 ± 164.5) (p 0.05). MUNIX global correlated with MRC global. MUNIX was greater in muscles with normal strength (95-100% of normal values) than in those with stable weakness (48%-0% of normal values) and new weakness (65%-0% of normal values). Respectively to clinical stage of muscle MUP (motor unit potential) amplitude increased to 350% of normal value, from 250% to 110%, and from 300% to 700%. No correlation was found between MUP parameters and MRC values.MUNIX reflects motor dysfunction and could be a good biomarker for loss of motor neurons in PPS.

Background Literature indicates that individuals with long-term residual lower extremity (LE) weakness after polio have decreased bone mineral density (BMD) deficiencies related to muscle weakness. Where weakness is asymmetrical, bone densitometry (BDt) measured only on the stronger LE may misclassify BMD. Objective To determine (1) whether femoral neck BMD differed from side to side in individuals with asymmetrical LE muscle weakness, and (2) the proportion of individuals at risk for underdiagnosis of low bone density or osteoporosis given unilateral assessment of the femoral neck. Design Retrospective study. Setting Outpatient postpolio center. Participants Patients >18 years old with complete relevant data. Main Outcome Measures BDt T scores, BMD categories based on standard T-score ranges, and side of LE weakness determined by a strength score. Results Forty-three subjects had at least 1 femoral neck T score and bilateral LE strength scores. Fourteen (32.5%) had BDt only on their weaker LE and 14 (32.5%) had BDt only on their stronger LE. Of the 15 subjects with BDt done on both femoral necks, T scores (mean [standard deviation]) were lower in the weaker LE (–1.73 [1.09]) than the stronger LE (–0.88 [1.0]) (P = .001). Classification of low bone density or osteoporosis was more frequent based on T scores taken on a weaker LE (48.3% and 24.1%, respectively) than from T scores from a stronger LE (41.4% and 6.9%, respectively). Conclusions In this small sample, using strong-limb T scores resulted in fewer individuals categorized as having low bone density or osteoporosis than when weak-limb T scores were used. Underestimating BMD loss may lead to undertreatment and increased risk of morbidity, mortality, and costs associated with femoral neck fractures in this high-fall-risk group. Level of Evidence III

This single-centered, randomized, double-blind, placebo-controlled study reports the results of L -Citrulline treatment for 24 weeks in patients with post-polio syndrome (PPS). Twenty-nine patients were randomized and assigned into receiving a treatment of 15 g L -Citrulline or placebo. The primary endpoint was the change of the 6 min walking distance test. Secondary endpoints included motor function measure, quantitative muscle strength, quantitative MRI and self-reported impairment questionnaires. Patients receiving L -Citrulline walked 17.5 longer in the 6 min walking distance test when compared to placebo group, however not statistically significant (95% CI = -14.69; 49.68, p = 0.298). None of the secondary endpoints showed a statistically significant change in the L -Citrulline group when compared to placebo group. The motor function measure showed a change of -0.78 (95% CI= [-3.39; 1.83] p = 0.563). Muscle degeneration of leg muscles assessed with quantitative MRI indicated no significant change (estimate= -0.01, 95% CI =-0.13; 0.11, p = 0.869). L -Citrulline was safe and well tolerated. In conclusion, administration of 15 g L -Citrulline daily for 24 weeks to patients with PPS showed no beneficial treatment effect in timed muscle function.

BACKGROUND Sense of coherence (SOC), comprising "comprehensibility", "manageability" and "meaningfulness", is important for successful adaptation in persons with late effects of polio (LEoP) and can be used as an estimate of the ability to cope with stressors. Coping behaviours are the actions a person performs to reduce stress and can be divided into problem-focused coping, emotion-focused coping and less useful coping. Our knowledge is very limited of what coping behaviours persons with LEoP use to manage their life situation as well as the association between SOC and coping behaviours. OBJECTIVE The aims of this cross-sectional study were to assess coping behaviours and to explore the association between SOC and coping behaviours in persons with LEoP. METHODS In total, 93 ambulant persons (52% women, mean [SD] age 74 [8] years) with clinically and electromyographically verified LEoP responded to a postal survey with the Sense of Coherence Scale 13 items (SOC-13) and the Brief Cope Scale. Three linear regression analyses were used to explore the association between SOC and problem-focused coping, emotion-focused coping and less useful coping, controlling for age and sex. RESULTS The most-used coping behaviours belonged to the categories problem-focused and emotion-focused coping. We found a significant negative association between SOC and less useful coping; less useful coping explained 34% of the variance of SOC (adj R2 = 0.34, p

Background Sleep disturbances, especially sleep disordered breathing and sleep movement disorders, seem to be highly prevalent among aging polio survivors. They could contribute to late functional deterioration, fatigue, poor quality of life and negative health outcomes, thereby increasing cardiovascular risk. Objectives This review focused on current knowledge of the prevalence of sleep disorders in polio survivors, their features, predictive factors and management. Data sources Articles were searched in PubMed and the Cochrane Library up to March 2018. Study eligibility criteria, participants and interventions Articles needed to 1) be written in English; 2) include only participants with previous poliomyelitis or post-polio syndrome diagnosis; and 3) involve any form of sleep disorders. Articles about isolated fatigue or non-specific sleep complaints as well as non-polio specific articles (neuromuscular disorders) were not included in the qualitative analysis. Results Among 166 studies identified, 41 were included in this review. The prevalence of sleep apnea syndrome, nocturnal alveolar hypoventilation and restless legs syndrome seemed higher than in the general population (from 7.3% to 65%, 15% to 20% and 28% to 63%, respectively). This review highlights the lack of randomised studies assessing sleep disorder management in this specific population. Limitations Because of the small number of eligible publications, none was excluded for methodological limitations, and only a qualitative analysis was provided. Conclusions and implications Follow-up of polio survivors should include systematic screening for sleep disorders because they are associated with adverse consequences. Sleep disorder evaluation and management should improve the long-term survival and quality of life of polio survivors. Methodologically robust clinical trials are needed, but the decreasing prevalence and large clinical spectrum of the disease may complicate the creation of comparable groups.

BACKGROUND: Dorsiflexion-restricting ankle-foot orthoses (DR-AFOs) are often prescribed in polio survivors with calf muscle weakness to reduce or solve gait problems. However, orthoses are sometimes not being used and/or users are dissatisfied with the usability. AIM: To compare the usability of custom-made DR-AFOs provided in clinical care between users and discontinued users who have calf muscle weakness due to polio. DESIGN: Cross-sectional survey. SETTING: Outpatient post-polio university hospital clinic in the Netherlands. POPULATION: All polio survivors with calf muscle weakness, provided with a DR-AFO between 2004 and 2015 in our outpatient clinic. METHODS: DR-AFO use and usability according to the ISO 9241-11 standard were evaluated with a questionnaire sent out by postal mail. RESULTS: Forty of 57 questionnaires were returned. Five respondents did not fulfil the eligibility criteria. DR-AFO use among the 35 eligible respondents was 74%. Compared to discontinued users, users were significantly more often male (users: 16 of 26 vs. discontinued users: 0 of 9, P=0.001), more limited in their walking ability without DR-AFO (P=0.007), perceived more effectiveness, both overall (P=0.001) and on their personal goals of use (P=0.006), and were more satisfied with orthosis-related aspects (P=0.011), such as comfort. CONCLUSIONS: Almost three quarters of the polio survivors used their orthosis. Use was related to several aspects of usability, indicating that it is important to consider usability in the prescription process of DR-AFOs for polio survivors with calf muscle weakness. CLINICAL REHABILITATION IMPACT: When prescribing DR-AFOs, it is important to consider that the orthosis is most likely used when the experienced walking problems are large and the DR-AFO reduces these problems. We recommend discussing the patient's personal goals for DR-AFO use and the anticipated improvement on the individual's walking problems as well as possible hindrance of the orthosis during daily life activities. Furthermore, providers may need to pay extra attention to females and should particularly take care that the experienced fit and comfort are satisfactory. Incorporating these suggestions in clinical practice may further improve DR-AFO use among polio survivors with calf muscle weakness.

Postpolio syndrome (PPS) is a disabling process characterized by progressive muscle weakness and atrophy that typically emerges decades after an initial poliomyelitis infection. Although the exact incidence of PPS is unknown, it is estimated that 25% to 40% of all poliomyelitis survivors are affected. Patients with PPS may have increased sensitivity to numerous anesthetic agents including neuromuscular blocking drugs. A case report of a patient with PPS undergoing general anesthesia for a cystoscopy procedure is presented. Because of a previous general anesthetic using traditional muscle relaxant reversal of neostigmine, which resulted in prolonged paralysis (6-8 hours) and an intensive care unit admission, sugammadex was used in the cystoscopy procedure. Excellent results were achieved. Following extubation, the patient sustained adequate respiratory effort demonstrated by respiratory rate, end-tidal carbon dioxide, and oxygen saturation within normal limits. Sugammadex administration led to a markedly improved outcome for a patient with a disabling muscle-weakening neurologic disorder. Use of this medication may be of value to other anesthesia providers caring for patients with PPS in an operative setting.

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.

Background As many as 60-80% of persons with a history of polio myelitis develop new symptoms, such as new or increased muscle weakness, muscle and joint pain, and fatigue several decades later, called postpolio syndrome (PPS). This may affect their ability to perform activities of daily living (ADL). It is still unclear if the patient's symptom is getting worse and in that case how much/fast the decline is. Aim The aim of the present study was to evaluate long-term changes in disability in community dwelling patients with prior poliomyelitis, in contact with a polio clinic 14-16 years post their first assessment. Design A cross sectional longitudinal study. Setting Polio clinic. Population Fifty-two persons recruited from an earlier 4-year follow-up participated in the study. Methods A questionnaire was mailed prior to the visit at the polio clinic. Physical testing was performed by measuring muscle strength, walking speed and handgrip force. Results Overall there was a small change in muscle strength. A significant reduction in the right leg was found for flexion 60° and in dorsal flexion. For the left leg a significant reduction was found for plantar flexion. In the walking tests, a significant reduction was seen for spontaneous walking speed. No significant interaction between decrease in spontaneous walking speed and the variables age, BMI and flexion 60° and dorsal flexion in the right leg was seen. Conclusions This cross-sectional longitudinal study shows small changes in muscle strength and disability. The results may imply that symptoms associated with late effects of polio are not progressing as fast as we had previously thought. Clinical rehabilitation impact When health care professionals meet persons with late effects of polio the knowledge of long-term consequences of deterioration is important. Knowing that the deterioration is not as fast as previously thought, can help us to support the person in having a healthy lifestyle, stay active and encourage to perform adapted physical training.

The prognosis of patients with left ventricular hypertrabeculation/noncompaction (LVHT) is assessed controversially. LVHT is associated with other cardiac abnormalities and with neuromuscular disorders (NMD). Aim of the study was to assess cardiac and neurological findings as predictors of mortality rate in adult LVHT-patients. Included were patients with LVHT diagnosed between 1995 and 2019 in 1 echocardiographic laboratory. Patients underwent a baseline cardiologic examination and were invited for a neurological investigation. In January 2020, their survival status was assessed. End points were death or heart transplantation. LVHT was diagnosed by echocardiography in 310 patients (93 female, aged 53 ± 18 years) with a prevalence of 0.4%/year. A neurologic investigation was performed in 205 patients (67%). A specific NMD was found in 33 (16%), NMD of unknown etiology in 123 (60%) and the neurological investigation was normal in 49 (24%) patients. During follow-up of 84 ± 71 months, 59 patients received electronic devices, 105 patients died, and 6 underwent heart transplantation. The mortality was 4.7%/year, the rate of heart transplantation/death 5%/year. By multivariate analysis, the following parameters were identified to elevate the risk of mortality/heart transplantation: increased age (p = 0.005), inpatient (p = 0.001), presence of a specific NMD (p = 0.0312) or NMD of unknown etiology (p = 0.0365), atrial fibrillation (p = 0.0000), ventricular premature complexes (p = 0.0053), exertional dyspnea (p = 0.0023), left bundle branch block (p = 0.0201), and LVHT of the posterior wall (p = 0.0158). In conclusion, LVHT patients should be systematically investigated neurologically since neurological co-morbidity has a prognostic impact.

Introduction Chronic pain and fatigue are the main symptoms of postpoliomyelitis syndrome (PPS). This study aimed to evaluate the efficacy and safety of an anthroposophic multimodal treatment for chronic pain in PPS outpatients. Methods A twelve‐week, four‐arm, randomized, double‐blind, placebo‐controlled, phase 2 prospective clinical trial was designed to compare four groups (n = 48): groups A and B received daily active experimental transdermal gel (ETG) or placebo gel (PTG), respectively; groups C and D received weekly external therapies, art therapies, and neurofunctional reorganization, plus either daily ETG or PTG, respectively. The pain symptoms were evaluated through a visual analogue scale (VAS), the McGill questionnaire, and thermography. Quality of life and resilience were evaluated by the WHOQOL‐BREF and Antonovsky sense of coherence questionnaires applied at baseline and after the interventions. Results No related adverse events occurred, and 10% of the patients reports dysphagia improvement. In the groups C and D, pain reduction was statistically significant in both the placebo group (p = .02, d = 1.315) and in the ETG (p = .005, d = 2.035). However, following the week‐to‐week evolution of pain with the concomitant use of the ETG, this significant pain reduction occurred earlier from the 4th week and continued to decrease (p = .016, d = 1.369). In the group that received the complete multimodal treatment, the greatest significant benefit in increasing quality of life occurred in the physical domain and elevation in resilience with an emphasis on meaning and comprehension domains. Conclusions The anthroposophic multimodal treatment group presented both safety and efficacy as an analgesic in the groups that received the nonpharmacological therapies, much earlier when associated with the ETG. The multimodal approach corresponded to the pattern of better efficacy for both pain reduction and improvement in quality of life and resilience., This study aimed to evaluate the efficacy and safety of an anthroposophic multimodal treatment for muscle and joint chronic pain in outpatients with PPS. This twelve‐week, four‐arm, parallel, randomized, double‐blind, placebo‐controlled clinical trial which were applied to evaluate pain, quality of life and resilience at baseline and after the interventions. The treatment showed increased safety and efficacy for pain and improvement of the quality of life and resilience specially in the groups that received the nonpharmacological interventions, earlier when associated to transdermal gel.

OBJECTIVE This longitudinal study examined the unique relationship between anxiety, symptoms (pain intensity, sleep disturbance, fatigue severity), and function domains (self-reported cognitive function, physical function, satisfaction with social roles) in individuals with chronic physical conditions, independent of depressive symptoms. METHOD Three surveys were mailed on an approximately yearly basis to community-dwelling adults with one of four chronic physical conditions (spinal cord injury, multiple sclerosis, muscular dystrophy, postpoliomyelitis syndrome). The first survey was completed by 1594 individuals (T1). Of these, 1380 completed the second survey (T2), and 1272 completed the third survey (T3). RESULTS Mixed growth curve models evidenced significant concurrent and longitudinal associations between anxiety and each symptom and function domain, independent of depression severity. The largest unique association found was between anxiety and self-reported cognitive function; inclusion of the measure of anxiety in the model improved model fit substantially over depression alone, χ²[2] = 104.40, p < .001. Both anxiety and depression exhibited similar effect sizes in their unique relationships with each symptom and function domain measure. However, depression was more strongly associated with satisfaction with social roles and physical function than was anxiety. CONCLUSIONS The findings showed that anxiety assessed at one point in time demonstrated significant and unique associations with concurrent and subsequent symptom and function domains. Given that anxiety has been inadequately studied (and perhaps evaluated and treated) in rehabilitation populations, the current findings suggest that researchers and clinicians should broaden their scope when assessing and treating psychological distress to incorporate anxiety and related disorders. (PsycINFO Database Record (c) 2018 APA, all rights reserved).

The prevalence of carpal tunnel syndrome (CTS) in patients with postpolio syndrome occurs at a rate of 22%. Irrespective of those with CTS, 74% of postpolio patients weight bear through their arms for ambulation or transfers. As open carpal tunnel release is performed along the weight-bearing region of the wrist, their functional independence may be altered while recovering. This case demonstrates that ultrasound-guided carpal tunnel release was successfully performed in a patient with postpolio syndrome allowing him to immediately weight bear through his hands after the procedure so he could recover at home. LEVEL OF EVIDENCE: V.

Background: Previous studies suggest that patients with a history of poliomyelitis (PM) later in life experience a variety of symptoms. These studies were carried out in patients who later in life were admitted to hospital or became members of polio societies and may therefore not be representative of all polio patients. Little data have been published concerning patients actually discharged from hospital with a diagnosis of acute paralytic PM. Objectives: The aim of this study was to compare the prevalence of late symptoms in individuals with a history of paralytic PM with that of controls, and to study whether late symptoms in individuals with a history of PM were associated with symptoms at the acute stage of polio, and finally to compare the prevalence of symptoms in polio patients with postpolio syndrome (PPS) with the prevalence of symptoms in polio patients without PPS. Methods: A questionnaire concerning various symptoms was sent to a previously established cohort of patients, who during the polio epidemics were discharged from the Department of Infectious Disease at Blegdamshospitalet, Copenhagen, with a diagnosis of paralytic PM, and to age- and gender-matched controls without PM. Information about symptoms at the acute stage of disease was obtained from hospital records. Logistic regression analysis with adjustment for age and gender was applied to compare the occurrence of late symptoms in cases and controls and within the above-mentioned groups of individuals with a history of PM. Results: (i) Compared with controls, individuals with a history of polio significantly more often reported muscle symptoms, pain, neuropathic sensory symptoms, and bulbar symptoms; (ii) the occurrence of symptoms did not seem to be related to symptoms of the initial PM; and (iii) symptom prevalence was significantly higher in individuals with a history of polio who reported PPS as compared with those who did not. Conclusion: Our data indicate that individuals with a history of PM late in life experience a variety of symptoms that cannot be attributed to lesions of the anterior horn. Furthermore, late symptoms do not seem to be related to initial symptoms of the acute stage of PM but to reported PPS. The last finding supports the perception that the cause of PPS is not just normal ageing.

The objective was to identify gait patterns in polio survivors with calf muscle weakness and associate them to underlying lower extremity impairments, which are expected to help in the search for an optimal orthosis. Unilaterally affected patients underwent barefoot 3D-gait analyses. Gait pattern clusters were created based on the ankle and knee angle and ankle moment shown in midstance of the affected limb. Impairment clusters were created based on plantarflexor and knee-extensor strength, and ankle and knee joint range-of-motion. The association between gait patterns and underlying impairments were examined descriptively. The Random Forest Algorithm and regression analyses were used to predict gait patterns and parameters. Seven gait patterns in 73 polio survivors were identified, with two dominant patterns: one with a mildly/non-deviant ankle angle, ankle moment and knee angle (n = 23), and one with a strongly deviant ankle angle and a mildly/non-deviant ankle moment and knee angle (n = 18). Gait pattern prediction from underlying impairments was 49% accurate with best prediction performance for the second dominant gait pattern (sensitivity 78% and positive predictive value 74%). The underlying impairments explained between 20 and 32% of the variance in individual gait parameters. Polio survivors with calf muscle weakness who present a similar impairment profile do not necessarily walk the same. From physical examination alone, the gait pattern nor the individual gait parameters could be accurately predicted. The patient's gait should therefore be measured to help in the prescription and evaluation of orthoses for these patients

Background New or increased impairments may develop several decades after an acute poliomyelitis infection. These new symptoms, commonly referred to as late effects of polio (LEoP), are characterised by muscular weakness and fatigue, generalised fatigue, pain at rest or during activities and cold intolerance. Growing older with LEoP may lead to increased activity limitations and participation restrictions, but there is limited knowledge of how these persons perceive the practical and psychological consequences of ageing with LEoP and what strategies they use in daily life. The aim of this qualitative study was therefore to explore how ageing people with LEoP perceive the their situation and what strategies they use for managing daily life. Methods Seven women and seven men (mean age 70 years) were interviewed. They all had a confirmed history of acute poliomyelitis and new impairments after a stable period of at least 15 years. Data were transcribed verbatim and analysed using systematic text condensation. Results The latent analysis resulted in three categories ‘Various consequences of ageing with LEoP’, ‘Limitations in everyday activities and participation restrictions’, and ‘Strategies for managing daily life when ageing with LEoP’ and 12 subcategories. The new impairments led to decreased physical and mental health. The participants perceived difficulties in performing everyday activities such as managing work, doing chores, partaking in recreational activities and participating in social events, thereby experiencing emotional and psychological distress. They managed to find strategies that mitigated their worries and upheld their self-confidence, for example finding practical solutions, making social comparisons, minimising, and avoidance. Conclusion Ageing with LEoP affected daily life to a great extent. The participants experienced considerable impact of the new and increased impairments on their life situation. Consequently, their ability to participate in various social activities also became restricted. Social comparisons and practical solutions are strategies that facilitate adaptation and acceptance of the new situation due to LEoP. This emphasises the need to design rehabilitation interventions that focus on coping, empowerment and self-management for people ageing with LEoP.

Objectives To determine if resilience is uniquely associated with functional outcomes (satisfaction with social roles, physical functioning, and quality of life) in individuals with physical disabilities, after controlling for measures of psychological health (depression and anxiety) and symptom severity (pain, fatigue, and sleep disturbance); and to examine the potential moderating effect of sex, age, and diagnosis on the hypothesized associations between resilience and function. Design Cross-sectional survey study. Setting Surveys were mailed (81% response rate) to a community sample of 1949 individuals with multiple sclerosis, muscular dystrophy, postpoliomyelitis syndrome, or spinal cord injury. Participants were recruited through the Internet or print advertisement (28%), a registry of previous research participants who indicated interest in future studies (21%), a departmental registry of individuals interested in research (19%), disability-specific registries (18%), word of mouth (10%), or other sources (3%). Participants Convenience sample of community-dwelling adults aging with physical disabilities (N=1574), with a mean Connor-Davidson Resilience Scale (10 items) score of 29. Interventions Not applicable. Main Outcome Measures Patient-Reported Outcomes Measurement Information System measures of Satisfaction with Social Roles and Activities and Physical Functioning, the World Health Organization's brief Older People's Quality of Life Questionnaire, and the Connor-Davidson Resilience Scale (10 items). Results After controlling for age, age squared, sex, diagnosis, psychological health, and symptom severity, resilience was significantly and positively associated with satisfaction with social roles (β=.17, P P P >.05). For every 1-point increase in scores of resilience, there was an increase of .50 in the quality of life score and .20 in the satisfaction with social roles score. Sex also moderated the association between resilience and satisfaction with social roles (F 1,1453 =4.09, P =.043). Conclusions The findings extend past research, providing further evidence indicating that resilience plays a unique role in nonphysical functional outcomes among individuals with physical disabilities.

The purpose of this study was to determine if falls in polio survivors, with or without post-polio syndrome (PPS), are related to number of medications taken, use of anti-depressant or psychoactive medications, or self-report of depression. A survey was sent to 300 members of a regional polio support group, asking them to document their fall history, medications used, and the presence of depression. Depression was measured by self-report and with the Geriatric Depression Scale, short form (GDS-15). One hundred and seventy-two usable surveys were returned with 146 of those completing the medication list. Sixty-two percent reported at least one fall in the past year. The multiple logistic regression was significant (p = 0.023), and it indicated depression to be a significant predictor (p = 0.012) of falls in polio survivors with and without PPS. The number of total medications or anti-depressant or psychoactive medications used was not related to fall incidence. Routine screening and treatment for depression may be one aspect of fall prevention which can be implemented through primary care.

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.

To design a disease-specific quality of life (QoL) questionnaire for people with post-polio syndrome (PPS).Qualitative interviews were conducted with 45 people with PPS to identify themes and derive potential items reflecting impact upon QoL. After cognitive debriefing, these were made into a questionnaire pack along with comparative questionnaires and posted to 319 patients. The 271 (85%) returned questionnaires were subjected to exploratory factor analysis (EFA) and Rasch analysis.A 25 item scale, the post-polio quality of life scale (PP-QoL), showed good fit to the Rasch model (conditional chi-square p = 0.156), unidimensionality (% t-tests 2.0: CI 0.7-3.8), and Cronbach's alpha of 0.87. With the latent estimate transformed to a 0-100 scale, the mean score was 56.9 (SD 18.5) with only 3.3% of respondents at the floor or ceiling of the scale. Test-retest reliability showed an intraclass correlation coefficient (ICC) (2.1) of 0.916, and correlation of 0.85.The disease-specific PP-QoL demonstrated excellent reliability, appropriate concurrent validity, and satisfied the standards of the Rasch model. It enables examination of the impact of health status upon perceived QoL, and the impact of rehabilitation interventions. The scale is freely available for academic or not-for-profit users to improve research in this neglected, disabling condition. Implications for Rehabilitation In post-polio syndrome (PPS), existing work examines aspects of health-related quality of life (HRQoL), such as activity limitations. A disease-specific QoL measure would enable researchers to model the impact of health status, such as fatigue or mobility restrictions, upon QoL in PPS. The post-polio quality of life scale (PP-QoL) is based on the patients' lived experience, meets Rasch standards and is free for use for academic and not-for-profit researchers. The raw score is reliable for individual use in clinical settings, and interval scale transformation is available for parametric applications and the calculation of change scores.

Objective Individuals living with physical disability due to early acquired or traumatic conditions often experience a range of psychological and physical health problems that are associated with their condition but are not directly caused by it. Known as "secondary health conditions," these problems can interact with existing functional limitations and other medical comorbidities to limit social participation. The current study assessed the concurrent and longitudinal associations between secondary health conditions, chronic medical comorbidities, and functional limitations, with a PROMIS® measure of social role participation. Methods A longitudinal survey study of community-dwelling adults with one of four chronic physical conditions (multiple sclerosis, muscular dystrophy, spinal cord injury, postpoliomyelitis syndrome). The baseline survey (T1) was mailed to 2041 individuals, and1862 baseline surveys were completed and returned (91% response rate). The follow-up survey (T2) was mailed roughly three years later; 1594 completed and returned the T2 survey (86% of T1 survey completers). Results Multiple linear regression analyses revealed that secondary health conditions, functional impairments, and chronic medical comorbidities accounted for 52% of the variance in satisfaction with social roles concurrently at T1. The amount of variance of change in satisfaction with social roles over the ∼3-year period accounted for by these variables was 3%. Functional limitations and more psychologically oriented secondary conditions were the strongest predictors of satisfaction with social roles. Conclusions Findings suggest that, for people with disabilities, addressing psychologically oriented secondary health conditions may be as important as functional impairment in predicting long-term social health. (PsycINFO Database Record (c) 2019 APA, all rights reserved).

Background There is substantial evidence that pain intensity and sleep are related, with findings generally suggesting more support for the influence of sleep on pain intensity than vice versa. However, the strength and direction of the relationship has been found to vary among different populations, with few studies in individuals with chronic physical disabilities. Objective Examine the directionality of the sleep and pain relationship in individuals with chronic physical disabilities. Methods Cross-lagged effects models were generated using data from a longitudinal observational survey study of individuals (N = 1660) with multiple sclerosis (MS), muscular dystrophy (MD), post-polio syndrome (PPS), and spinal cord injury (SCI). Models evaluated the correlational effects of sleep disturbance and pain intensity, as well as the cross-lagged effects of sleep disturbance to pain intensity and vice versa. Results The effects of pain on sleep were stronger than sleep on pain, although the magnitude of the effects were both relatively weak. Analyses within individual samples were consistent with the overall sample results for MS, MD, and PPS. In the SCI sample the magnitude and direction of the cross-lagged model paths were more variable than in the other samples. Conclusions The relationship between pain intensity and sleep disturbance appears bi-directional, but the effects are small in a sample of individuals with long-term disabilities. The temporal effects of pain on sleep disturbance appear stronger than the effects of sleep disturbance on pain intensity. Future research is needed to better understand this relationship in the context of pain and/or sleep disturbance treatments.

Objective: To explore the health situation and identify specific health challenges in non-Western immigrants with polio in Norway, by comparing their status with Western immigrants with polio and native Norwegians with polio. Design: A questionnaire covering demographics, polio history, life satisfaction, medical, psychological and social conditions was answered by 1,408 persons with polio, among them 34 immigrants from non-Western countries and 32 immigrants from Western countries. Results: The non-Western immigrant polio group had a mean age of 46 years, were highly educated, reported high frequency of mental health problems and only one-third was working. Mean age for contracting polio was 2.8 years. Only 30% was hospitalized in the acute phase and 80% reported severe leg weakness. Use of a powered wheelchair was reported by 72%. Post-polio symptoms had started at a mean age of 31 years. The non-Western immigrant group reported more fatigue, pain and loneliness, and a high proportion reported insufficient assistance from the public health system. Conclusion: The group of non-Western immigrants with polio in Norway reported more health and social problems than the group of Western immigrants with polio or the native Norwegian group with polio, even though they were younger and more highly educated. Their complex psychological and social situation requires active intervention from the health system, and health professionals need extra skills to deal most effectively with their situation. publishedVersion

IntroductionThe outcomes of total hip replacement in patients suffering from residual poliomyelitis are poorly covered in the literature. In this retrospective study we posed the question of whether total hip replacement performed for degenerative hip diseases in limbs with residual poliomyelitis could determine satisfactory mid-term clinical and radiographic results, with a reasonable complication rate.MethodsA retrospective study was carried out to assess the results of 14 total hip replacements performed on 14 patients with residual poliomyelitis on the involved limb from June 1999 to September 2011. Average age at the time of surgery was 51 years (range 26-66 years). Mean duration of follow-up was 92 months (range 52-156 months). Surgery was performed through a direct lateral approach on all hips. All but one were cementless implants.Results2 implants failed, 1 due to traumatic acetabular fracture 6 days after surgery, and 1 due to aseptic cup loosening 13 years after surgery. Surgery was uneventful in all patients except 1 (7%), who experienced a transient sensory sciatic nerve palsy. At the latest follow up Harris Hip Score was 83.3 (range 72-91) with a marked improvement when compared to preoperative score (average 52, range 32-78). No dislocations had occurred.ConclusionsTotal hip replacement can be considered a feasible option for hip osteoarthritis in patients with limbs affected by residual poliomyelitis. Longer follow-up studies are needed to assess the effectiveness of unconstrained total hip replacement in polio patients.

Background Adults with long-term disability are living longer and may experience accelerated aging. More information is needed to understand the incidence of chronic comorbid medical conditions in this population. Objective To examine the incidence, prevalence, age of onset and predictors of five chronic conditions in a sample of adults with long-term physical disability. Methods Longitudinal self-report surveys were mailed to 1594 adults with multiple sclerosis, muscular dystrophy, post-polio syndrome or spinal cord injury twice, 3.5 years apart. Survey questions assessed demographics (date of birth, sex, income, disability type, height/weight), self-reported diagnosis of coronary heart disease, hypertension, arthritis, diabetes and cancer, and health behaviors (alcohol use, smoking, physical activity). Results Over the course of the study, the most commonly reported new onset chronic comorbid medical condition was arthritis (percent incidence = 14%), followed by hypertension (9%) and cancer (7%). Report of a new condition was greatest in adults aged between 56 and 65 years, and risk factors included greater BMI, waist circumference, and the presence of another chronic comorbid medical condition at baseline. Conclusions Chronic comorbid medical conditions are prevalent in persons with long-term physical disability. Midlife appears to be the period of greatest risk for onset of a new condition, and risk for incidence increases in the presence of other chronic comorbid medical conditions. Modifiable risk factors include BMI and waist circumference. Future research should explore whether changes in modifiable factors at midlife or earlier (e.g., diet, exercise) would help prevent or delay onset of comorbid conditions in this population.

BACKGROUND: Many individuals with late effects of polio have difficulties participating in daily activities. The Reintegration to Normal Living Index (RNL-I) is a self-report questionnaire that evaluates perceived participation, but knowledge of the test-retest reliability and measurement errors in this population is lacking.OBJECTIVE: To evaluate the test-retest reliability of the RNL-I in adults with late effects of polio.DESIGN: A postal survey with a test-retest design.SETTING: University hospital outpatient clinic.PARTICIPANTS: Fifty-one adults (20 women and 31 men; mean age 72 years) with late effects of polio.MAIN OUTCOME MEASUREMENTS: The Reintegration to Normal Living Index (RNL-I).METHODS: The participants responded to the RNL-I twice, 3 weeks apart. Data were analyzed with the following statistical methods: percentage agreement (PA), quadratic kappa coefficients, the intraclass correlation coefficient (ICC), mean difference, standard error of measurement (SEM/SEM%), and the smallest real difference (SRD/SRD%).RESULTS: The PA (ie, the same scoring at both test occasions) was >70% for 10 of 11 items. The kappa coefficients showed good test-retest agreement (>0.61) for 7 items. The ICC was 0.88 and the mean difference was -0.74. The SEM (SEM%) was 7.4 (9.7%) and the SRD (SRD%) was 20.5 (27.0%).CONCLUSION: The RNL-I can be considered reliable for adults with mild to moderate late effects of polio. It can thereby be used to assess changes in perceived participation over time or after rehabilitation interventions, both for a group of individuals and a single individual.LEVEL OF EVIDENCE: III. (Less)

Comparing results from jitter studies performed with voluntary and electrical activation is difficult to perform quantitatively, particularly in complex signals as seen in reinnervation. High jitter values in individual spikes in these multispike signals can be missed with both activation methods, which introduces a bias towards more normal values.With voluntary activation, triggering on a spike from an abnormal end-plate in multispike potentials will overestimate individual jitter values and the number of abnormal jitter values.With electrical stimulation, artefactually-increased jitter may be caused by subliminal stimulation, which causes uncertainty at the stimulation point. Electrical stimulation also may activate many axons, causing signal summation, with erroneous or impossible jitter estimation.Awareness of such pitfalls can improve the correct performance and interpretation of jitter recordings. Quantitative comparisons of results can be made between studies in reinnervated muscle performed with the same activation method, but not between results obtained with different activation methods.

The purpose was to explore the feasibility of whole body vibration (WBV) on polio survivors with/without post-polio syndrome (PPS) by studying its effects on walking speed (10-m walk test), endurance (2-min walk test), pain severity/interference (Brief Pain Inventory [BPI]), sleep quality (Pittsburg Sleep Quality Index), fatigue (Fatigue Severity Scale), leg strength (manual muscle testing and hand-held dynamometry), and muscle cramping (written logs).Fifteen individuals completed the study, participating in eight sessions in two 4-week blocks. Participants started with ten 1-min vibration bouts/session, increasing to 20 min. Low (amplitude 4.53 mm, g force 2.21) and higher (amplitude 8.82 mm, g force 2.76) intensity blocked intervention occurred in random order crossover design. Blinded testing ensued before/after intervention blocks and at follow-up.No study-related adverse events occurred. Participants starting first with higher intensity intervention improved in walking speed (p = 0.017). BPI pain severity significantly improved (p = 0.049) after higher intensity intervention. No significant changes were found after low intensity vibration or in other outcome measures.WBV appears to be a safe exercise for this population. Long-term use in polio survivors needs to be researched, particularly in reducing barriers to participation to promote the physical aspects of health.

BACKGROUND: Sense of Coherence (SOC) is important for successful adaptation and mental well-being in people with life-long medical conditions. Late effects of polio (LEoP) often lead to a life-long disability, but no study has assessed SOC in this population. OBJECTIVE: To assess SOC in persons with LEoP and to explore the association between SOC, demographics (age, gender, marital status and level of education) and variables related to LEoP (age at polio onset, number of years from polio until onset of LEoP and self-rated disability). METHOD: Ninety-three community-dwelling persons with clinically verified LEoP responded to a postal survey with the Sense of Coherence Scale (SOC-13). A hierarchical multiple regression analysis was performed to explore the associations with SOC. RESULTS: SOC varied considerably among the participants. The mean and median SOC-13 total sum score was 71.8 and 76 points, which is similar to age-matched non-disabled people. The number of years before onset of LEoP and self-rated disability together with the participants' marital status and level of education explained 37% (p < 0.001) of the variance in SOC. CONCLUSION: Persons with LEoP have a level of SOC indicating that they generally have the ability to understand, handle and being motivated when dealing with stressful events and problems arising in their lives as a result of their disability. Being married and having a higher education, living many years before onset of LEoP and perceiving a mild to moderate disability contributed to a strong SOC. (Less)

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.A process evaluation using data from an RCT.Forty-four severely fatigued individuals with PPS were randomized to exercise therapy (n = 22) or usual care (n = 22).Participants in the exercise group were instructed to exercise 3 times weekly for 4 months on a bicycle ergometer (60-70% heart rate reserve).The attendance rate was high (median 89%). None of the participants trained within the target heart rate range during75% 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.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.Netherlands National Trial Register NTR1371.

Background: Sexuality is an important aspect of quality of life in individuals with disabilities, yet little is known about what factors contribute to sexual satisfaction as these individuals age. Method: Middle-aged adults with physical disabilities completed a cross-sectional survey that included measures of sexual activity, function, and satisfaction. Results: Consistent with studies of able-bodied adults, sexual function was the strongest predictor of satisfaction. However, depression also predicted sexual satisfaction for women. Use of aids for sexual activity varied by disability type and was generally associated with better function. Lowest levels of sexual satisfaction were reported by men with SCI. Conclusion: Depression may negatively impact sexual satisfaction in women, beyond contributions of sexual dysfunction, and effective use of sexual aids may improve function in this population.

To explore how the rehabilitation plan influences the rehabilitation process and its outcome in people with late effects of polio participating in an individualised goal-oriented interdisciplinary rehabilitation programme.Four women and two men with late effects of polio were interviewed before rehabilitation, at discharge, and at follow-up. Data were analysed according to the constant comparative method of grounded theory.The participants' experiences formed one core category: "The same starting point but different rehabilitation processes". Before rehabilitation, all participants experienced a similar starting point: Naïve understanding of rehabilitation. During rehabilitation, two separate processes followed. Four participants experienced their rehabilitation as being a mutually shared process that led to a process of change. They were actively engaged, using the rehabilitation plan, and working towards goals targeting a broad perspective of daily activities. The remaining two participants experienced their rehabilitation as a staff-directed process, with limited use of the rehabilitation plan, focusing on goals mainly related to body functions and self-care, not leading to any substantial changes.When clients experience that they develop a mutually shared rehabilitation process, based on a rehabilitation plan, they became more engaged in their rehabilitation and gained a better understanding of their participation during the process. Knowledge of the differences in how clients use the rehabilitation plan during the rehabilitation process can support their active engagement during rehabilitation. This, in turn, can promote a more holistic view among clients and professionals during the rehabilitation for people with late effects of polio. Implications for Rehabilitation Clients who experience a rehabilitation that is mutually shared with professionals, have a better understanding of their engagement during the rehabilitation process. When clients and professionals use the rehabilitation plan as a mutual tool, clients become more actively engaged in their rehabilitation process. A structured rehabilitation plan can serve as a map, and support clients' process of change during the rehabilitation process, also after the rehabilitation period is completed.

Background Lung volume recruitment (LVR) is a cough assistance technique used in persons with neuromuscular disorders (NMDs), most typically in those requiring noninvasive ventilation (NIV). Whether it may be useful in persons with NMDs who have milder respiratory impairment is unknown. Objective To assess the feasibility, impact on quality of life (QOL), and preliminary physiological effects of daily LVR in different categories of persons with NMDs who have an early stage of respiratory impairment. Design Feasibility study. Setting Academic tertiary care center. Participants Outpatients diagnosed with amyotrophic lateral sclerosis (n = 8), postpolio syndrome (n = 10), and myotonic dystrophy (n = 6) who had restrictive respiratory defects but were not yet using NIV. Methods Participants were asked to perform LVR up to 4 times daily and log their LVR use in a diary. Physiological measurements and questionnaires were completed at baseline and after 3 months. Main Outcome Measurements Compliance with LVR use was assessed, along with QOL and willingness to continue the treatment. Physiological measurements included forced vital capacity (FVC), lung insufflation capacity (LIC), and the LIC minus FVC difference. Results Of the 24 recruited subjects, 7 with amyotrophic lateral sclerosis, 7 with postpolio syndrome, and 5 with myotonic dystrophy completed the study (n = 19). At baseline, mean values for FVC and spontaneous peak cough flow were 59.9% predicted and 373.1 L/min, respectively. For subjects completing the study, 74% were willing to continue long-term LVR use, and QOL scores were not adversely affected by LVR in any NMD subgroup. The LIC-FVC difference increased from baseline to follow-up by a mean of 0.243 L ( P = .006) in all subjects (n = 19), suggesting a possible improvement in respiratory system mechanics. Conclusions In patients with NMDs who have early restrictive respiratory defects but do not yet require NIV, regular use of LVR is feasible with no negative impact on QOL over a 3-month period and may have physiological benefits. Further work is needed to determine whether early institution of LVR can improve respiratory system mechanics and help delay ventilatory failure in persons with NMDs.

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.

To compare the psychological health of the individuals with long-standing poliomyelitis, with or without post-polio syndrome (PPS), to the general population and to identify the role of work as well as other variables with regard to their psychological health.A cross-sectional study.One hundred and ninety-five polio patients attending postpolio clinic in Jerusalem.Emotional distress (ED) was measured using the general health questionnaire (GHQ-12). Demographic, medical, social and functional data were recorded using a specific structured questionnaire. Each polio patient was compared to four age- and sex-matched controls.ED was higher in the polio population as compared to the general population. Within the polio population ED was inversely correlated with work status. No correlation was found between ED and the functional level of polio participants and no difference was found in GHQ score between polio participants with or without post-polio. In addition, ED was less affected by subjective perception of physical health among polio patients as compared to the general population.Long-standing poliomyelitis is associated with decreased psychological health as compared to the general population. Yet, the resilience of polio survivors is manifested by their ability to block further decline of their psychological health in spite of deterioration in their physical health. Work appears as a significant source of resilience in the polio population. Implications for Rehabilitation Individuals with long-standing poliomyelitis often suffer from high emotional distress and may benefit from psychotherapy aimed at reducing distress. As active employment status is associated with increased mental health among polio survivors, encouraging participation at work needs to be a significant component of psychotherapeutic programs. Polio survivors, although physically disabled, may be relatively resilient, as their mental health is less affected by their negative health perception. This and other expressions of resilience may serve as a platform for increasing personal growth among them by implementing hope-oriented psychotherapy.

Polio affects the quality of life of those who have suffered from it and causes health problems including the post-polio syndrome. The main goals of this work were to know the patients perspective of how they have been affected by the disease and establish the knowledge of post-polio syndrome among patients and primary health care professionals.Interpretive qualitative research based on the Grounded Theory carried out in two health-care centers in the city of Malaga, one of them with care-rural clinics. Four focal groups were established with the participation of thirteen patients and two focus groups with twenty-six professional participants. Intentional sampling is performed until saturation. The analysis follows an inductive strategy using the Atlas Ti5.2 software.The people affected by polio reports their personal histories of suffering counteracted by strong family support and an active coping attitude, marked by great effort exertion, willpower and endurance. These people made a positive assessment of their lives minimising the limitations. They presented compatible symptoms with post-polio syndrome, which remain unidentified due to the lack of knowledge of it among patients and health-care professionals. The health care provided was considered deficient due to several causes as for instance lack of involvement, communication problems.The day-to-day polio experience is focused on personal overcoming with major roles played by family support, difficult relationships with the healthcare system and lack of knowledge of the post-polio syndrome.La polio afecta a la calidad de vida de las personas que la han padecido y ocasiona problemas de salud entre los que se encuentra el síndrome postpolio. Los objetivos de este estudio fueron conocer la perspectiva de pacientes sobre cómo les ha afectado la enfermedad y describir el conocimiento sobre el síndrome postpolio de pacientes y profesionales de atención primaria.Investigación cualitativa interpretativa basada en la Teoría Fundamentada, realizada en dos centros de salud de la ciudad de Málaga, uno de ellos con consultorios rurales. Se realizaron cuatro grupos focales con participación de trece pacientes y dos grupos focales con veintiséis profesionales participantes. Muestreo intencional hasta saturación. El análisis siguió una estrategia inductiva con ayuda del programa Atlas Ti 5.2.Los pacientes relataron una historia personal de sufrimiento, contrarrestada por un fuerte apoyo familiar y afrontamiento activo (categoría central), marcado por el sobreesfuerzo, la superación y una alta resiliencia. Realizaron una valoración positiva de sus vi- das, minimizando las limitaciones. Presentaron síntomas compatibles con el síndrome postpolio, no identificado por el gran desconocimiento de pacientes y profesionales. La atención sanitaria fue considerada deficitaria (falta de implicación y problemas de comunicación).La vivencia de la polio está centrada en la superación personal, con gran relevancia del apoyo familiar, las difíciles relaciones con el sistema sanitario y el desconocimiento del síndrome postpolio.

Objective Few studies have quantified the prevalence of restless legs syndrome (RLS) in patients with post-polio syndrome (PPS). Our objective was to assess the prevalence and severity of RLS in patients with PPS and to examine the demographic characteristics of this population. Method This was a cross-sectional study conducted from April 2010 to May 2012 at the outpatient Neuromuscular Disorders clinic of Universidade Federal de Sao Paulo, Sao Paulo, Brazil. We evaluated 119 patients with PPS, consecutively recruited, and investigated for RLS based on the diagnostic criteria established by the International Restless Legs Syndrome Study Group (IRLSSG). Patients were evaluated with the Brazilian version of the IRLSSG severity scale. Results The prevalence of RLS was 36% ( n = 43; 32 women and 11 men). The ages at onset of RLS (median = 41 years) and PPS (median = 41 years) were concurrent, and the correlation between onset of symptoms of RLS and onset of symptoms of PPS was positive and very strong (Spearman r = 0.93, p = 0.01). The median RLS severity was 23 (range, 20–28). Low educational achievement and depression were predictive of RLS development. Conclusion In the largest population of patients with PPS studied to date, our results indicate a high prevalence of RLS, marked disease severity, and concomitant onset of both conditions in many patients with PPS. Further studies are needed to elucidate a possible pathophysiologic mechanism linking these two conditions. We suggest that all post-polio patients with sensory and motor complaints in the legs be investigated for RLS.

The objective of this study was to identify energy expenditure, retrospectively, in individuals with post-poliomyelitis syndrome (PPS) in the Brazilian population. Methods The Baecke questionnaire for the evaluation of habitual physical activity (HPA), assessment of quality of life (WHOQOL-Bref), and the Fatigue Severity Scale were administered to patients with PPS, poliomyelitis sequelae (PS) and to a control group (CG). Participated in the study 116 individuals (PPS=52,PS= 28,CG=36). Results Patients with PPS tended to increase their HPA from 10 to 20 years of age, compared with those in the PS group and the CG. In the period from 21 to 30 years of age, there was significant increase in the PPS group’s occupational physical activity compared to the PS group, and the occupational physical activity (21-30 years of age) correlated with the onset of symptoms of PPS. Conclusion Patients with PPS had a higher energy expenditure during life, especially in occupational physical activity at ages 21-30 years, suggesting this decade is critical for the development of PPS.

Contains fulltext : 176980.pdf (Publisher’s version ) (Open Access) 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.

To evaluate the association between self-reported severity of polio sequelae and current functional status among polio survivors.This was a cross sectional study of 195 polio survivors attending a polio outpatient clinic at a university hospital. The main outcome measures of demographic, medical, social, and functional data were gleaned from a questionnaire adapted for the polio population. The severity of polio sequelae was evaluated with the self-reported Index of Post-Polio Sequelae (IPPS).The mean age of our sample was 57.6 ± 10.5 years, 53% were men, 38% had acquired higher education and 37% were employed. We found significant correlations between the total IPPS score and independence in activity of daily living (P0.05), the use of walking aids (P0.005) and mobility in and out-of-doors (P0.0001). A positive correlation was also found between the total IPPS score and subjective assessment of physical and mental health (P0.0001).Higher disability in ADL and mobility and lower perception of physical and mental health in polio survivors were associated with a higher score on the IPPS, reflecting greater severity of polio sequelae. These findings demonstrate the IPPS as a useful tool in the clinical evaluation of the polio population, however further data is needed in order to determine if this index can assess clinically significant changes over time.

To investigate the health-related quality of life (QOL) in Swedish patients with post-polio syndrome (PPS), with a focus on sex and age. A total of 364 patients were recruited from five Swedish post-polio clinics. Analysis was carried out using SF-36 and data were compared with those of a normal population. QOL was significantly lower in PPS patients for all eight subdomains and the two main scores (physical compound score and mental compound score) when compared with the controls. Male patients had a significantly higher QOL than female patients for all subdomains and also for mental compound score and physical compound score, a phenomenon also observed in the normal population. There was a decrease in QOL in the physical domains and an increase in vitality with age. PPS decreases health-related QOL in both sexes, more in female patients. QOL for physical domains decreases whereas vitality increases with age in both sexes.

Objectives: To test a conceptual model of secondary health conditions, age, and function in persons aging with long-term physical disabilities. Methods: Surveys were collected from 1,862 adults with spinal cord injury, neuromuscular disease, multiple sclerosis, or post-polio syndrome. Structural equation modeling was used to build a model describing relationships among physical and psychosocial secondary health conditions, pain, functional impairments, chronic medical conditions, and age. Results: In total, 12 individual symptom or function domains (latent factors) were identified, grouped into 5 broader factors. Increasing age was associated with greater rates of physical and health problems and poorer function, and showed curvilinear relationships with pain and psychosocial difficulties. Discussion: These data support a biopsychosocial model of secondary health conditions in adults aging with physical disability and suggest a five-factor approach for conceptualizing secondary conditions and their impact. Results also emphasize the importance of age in symptom severity and impact.