Post-Polio Syndrome Revisited.
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ABSTRACT:
SUBMITTER: Punsoni M
PROVIDER: S-EPMC10123742 | biostudies-literature | 2023 Apr
REPOSITORIES: biostudies-literature
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Post-Polio Syndrome Revisited.
Punsoni Michael M Lakis Nelli S NS Mellion Michelle M de la Monte Suzanne M SM
Neurology international 20230413 2
Post-polio syndrome (PPS) is characterized by recrudescence or worsening of motor neuron disease symptoms decades after recovery from acute paralytic poliovirus infection, i.e., poliomyelitis. PPS afflicts between 25% and 40% of poliomyelitis survivors and mimics motor neuron diseases (MNDs), such as amyotrophic lateral sclerosis (ALS), due to its selective impairment, degeneration, or death of motor neurons in the brainstem and spinal cord. Herein, we report a case of PPS in a 68-year-old man w ...[more]
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Project description:BackgroundPost-polio syndrome is characterised by symptoms of fatigue, pain and new-onset neuromuscular weakness, and emerges decades after the initial poliovirus infection. We sought to evaluate the only post-polio syndrome specific self-management programme in the United Kingdom.MethodsThis was a retrospective study of patients who had completed a residential self-management programme led by a multi-disciplinary clinical team. Following a confirmed diagnosis of post-polio syndrome by rehabilitation and neurology specialists, patients were offered to participate in the programme. Although group-based, patients also received individually tailored support on physical exercise and fatigue management. Physical effects, physical function, psychosocial well-being measures were assessed at baseline and 6 months follow-up. Knowledge was tested at baseline and immediately following the programme. Statistical comparisons were made using paired t-test and Wilcoxon signed rank test according to the data distribution.ResultsOver a period of 17 years, 214 participants (median age 61.3 years old, 63% female) attended 31 programmes. At 6 months the following post-polio syndrome specific symptoms improved significantly: fatigue, as measured by the Multidimensional Assessment of Fatigue scale [37.6 (7.1) vs. 34.2 (9.3), P=0.005]; and pain [15.0 (6.1) vs. 13.1 (6.7), P=0.001], atrophy [10.0 (8.0-12.0) vs. 9.0 (7.0-11.0), P=0.002] and bulbar symptoms [3.0 (1.0-5.0) vs. 2.0 (0-4.0), P=0.003] as measured by the Index of Post-polio Sequelae scale. Knowledge related to post-polio syndrome also significantly increased [14.0 (11.0-16.0) vs. 17.0 (16.0-19.0), P=0.001]. Participants were able to walk at a faster speed over 10 meters [0.77 (0.59-1.00) vs. 0.83 (0.67-1.10) m/s, P=0.003] and walked longer distances during the 2-minute walk test [76.9 (31.7) vs. 82.0 (38.4) m, P=0.029]. Depression and anxiety scores did not change over time [PHQ-9, 2.0 (0.3-10.8) vs. 2.0 (0.3-6.8), P=0.450; GAD-7, 2.0 (0-7.0) vs. 1.0 (0-3.0), P=0.460] nor was there change in self-reported quality of life {60 [50-70] vs. 60 [55-70], P=0.200}.ConclusionsThis study suggests that a post-polio syndrome self-management programme led to improvement in symptoms, knowledge and walking speed, but not quality of life. Anxiety and depression scores remained low.
| S-EPMC7642628 | biostudies-literature
Project description:To examine the risk of post-polio syndrome (PPS) in immigrant groups using native Swedish-born individuals as referents. This is a retrospective study. The study population included all individuals aged 18 years and older registered in Sweden. PPS was defined as having at least one registered diagnosis in the Swedish National Patient Register. The incidence of post-polio in different immigrant groups, using Swedish-born individuals as referents, was assessed by Cox regression, with hazard ratios (HRs) and 99% confidence intervals (CI). The models were stratified by sex and adjusted for age, geographical residence in Sweden, educational level, marital status, co-morbidities, and neighbourhood socioeconomic status. In total 5300 post-polio cases were registered, 2413 males and 2887 females. Fully adjusted HRs (99% CI) in immigrants versus Swedish-born were 1.77 in men (1.52-2.07) and 1.39 (1.19-1.62) in women. Statistically significant excess risks of post-polio were found in the following subgroups: men and women from Africa, HRs (with 99% CI) 7.40 (5.17-10.59) and 8.39 (5.44-12.95), respectively, and Asia, HRs 6.32 (5.11-7.81) and 4.36 (3.38-5.62) respectively, and in men from Latin America, HR 3.66 (2.17-6.18). It is of importance to be aware of risks of PPS in immigrants settled in Western countries, and that it is more common in immigrants from regions of the world where polio is still prevalent. Patients with PPS need treatment and proper follow-up until polio has been eradicated through global vaccination programs.
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Project description:ObjectivesTo understand the quality-of-life implications for post-polio syndrome survivors.MethodsFor this qualitative study, a focus group methodology, with a case study design, was used. The data were analyzed for themes using a social ecological conceptual framework.ResultsThree focus groups were conducted with a total of 24 participants (N?=?24). Participants defined quality of life as being able to engage in meaningful activities of daily living. Participants shared experiences of adapting to declining physical health and embracing new activities of daily living. They expressed hope and shared stories that demonstrated resiliency.ConclusionThe literature states that individuals aging with post-polio syndrome express concern that health providers often know little about their disability condition. The participants in this study reflected this experience by sharing feelings of misunderstanding from the medical community and expressing a desire to have more dynamic education for health providers.
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Project description:BackgroundPost-polio syndrome (PPS) is characterized by new or worsening muscular weakness, atrophy, muscle and joint pain, and muscle fatiguability decades after paralytic poliomyelitis infection.Case descriptionA 56-year-old man was diagnosed with paralytic poliomyelitis at the age of five, which left him with flaccid paralysis and weakness of the right leg. One year before seeking chiropractic care, the patient saw his primary care physician with neck pain, low back pain, and fatigue. At the time, he had been diagnosed with degenerative spondylosis and was being treated with tricyclic antidepressants, clonazepam, and tramadol. Despite taking the drugs, his spinal pain and fatigability deteriorated, and he acquired head ptosis during the following six months. As a result, he sought chiropractic care for second opinion. Due to the patient's failure to respond to oral analgesics, radiographs were performed, which revealed degenerative spondylosis, cervical flexion deformity, right pelvic drop, and right thoracolumbar scoliosis. The patient met the PPS diagnostic criteria. PPS related isolated neck extensor myopathy (INEM) was impressed. Multimodal intervention including cervical and lumbar manipulation, spinal traction, micro-vibration deep muscle massage, and core muscle training was provided. As a result of 40-month treatment, the patient reported full resolution of physical complaints. Head posture restored, cervical curvature retrieved and pelvic obliquity relatively corrected.ConclusionsSurvivors of paralytic polio are especially vulnerable to developing leg weakness and length discrepancy, pelvic obliquity, asymmetric axial loading, and trunk muscular imbalance. The current case demonstrates a rare myopathy in a patient at post-polio stage, as well as the restoration of neck function with chiropractic intervention.
| S-EPMC10130685 | biostudies-literature
Project description:Worldwide efforts to eradicate polio caused a tipping point in polio vaccination strategies. A switch from the oral polio vaccine, which can cause circulating and virulent vaccine derived polioviruses, to inactivated polio vaccines (IPV) is scheduled. Moreover, a manufacturing process, using attenuated virus strains instead of wild-type polioviruses, is demanded to enhance worldwide production of IPV, especially in low- and middle income countries. Therefore, development of an IPV from attenuated (Sabin) poliovirus strains (sIPV) was pursued. Starting from the current IPV production process based on wild type Salk strains, adaptations, such as lower virus cultivation temperature, were implemented. sIPV was produced at industrial scale followed by formulation of both plain and aluminium adjuvanted sIPV. The final products met the quality criteria, were immunogenic in rats, showed no toxicity in rabbits and could be released for testing in the clinic. Concluding, sIPV was developed to manufacturing scale. The technology can be transferred worldwide to support post polio-eradication biosafety goals.
| S-EPMC3861478 | biostudies-other
Project description:ObjectiveTo 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.DesignA process evaluation using data from an RCT.PatientsForty-four severely fatigued individuals with PPS were randomized to exercise therapy (n = 22) or usual care (n = 22).MethodsParticipants in the exercise group were instructed to exercise 3 times weekly for 4 months on a bicycle ergometer (60-70% heart rate reserve).ResultsThe attendance rate was high (median 89%). None of the participants trained within the target heart rate range during >75% of the designated time. Instead, participants exercised at lower intensities, though still around the anaerobic threshold (AT) most of the time. Muscle function did not improve in the exercise group.ConclusionOur results suggest that severely fatigued individuals with PPS cannot adhere to a high intensity aerobic exercise program on a cycle ergometer. Despite exercise intensities around the AT, lower extremity muscle function nor cardiorespiratory fitness improved. Improving the aerobic capacity in PPS is difficult through exercise primarily focusing on the lower extremities, and may require a more individualized approach, including the use of other large muscle groups instead.Trial registrationNetherlands National Trial Register NTR1371.
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Project description:BackgroundRevesz syndrome (RS) is an extremely rare variant of dyskeratosis congenita (DKC) with only anecdotal reports in the literature.MethodsTo further characterize the typical features and natural course of the disease, we screened the English literature and summarized the clinical and epidemiological features of previously published RS cases. In addition, we herein describe the first recorded patient in central Europe.ResultsThe literature review included 18 children. Clinical features are summarized, indicating a low prevalence of the classical DKC triad. All patients experienced early bone marrow failure, in most cases within the second year of life (median age 1.5 years; 95% CI 1.4-1.6). Retinopathy occurred typically between 6 and 18 months of age (median age 1.1 years; 95% CI 0.7-1.5). The incidence of seizures was low and was present in an estimated 20% of patients. The onset of seizures was exclusively during early childhood. The Kaplan-Meier estimate of survival was dismal (median survival 6.5 years; 95% CI 3.6-9.4), and none of the patients survived beyond the age of 12 years. Stem cell transplantation (SCT) was performed in eight children, and after a median of 22 months from SCT four of these patients were alive at the last follow up visit.ConclusionRS is a severe variant of DKC with early bone marrow failure and retinopathy in all patients. Survival is dismal, but stem cell transplantation may be performed successfully and might improve prognosis in the future.
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Project description:Individuals with post-polio syndrome (PPS) suffer from falls and secondary damage. To (i) analyze the correlation between spatio-temporal gait data and fall measures (fear and frequency of falls) and to (ii) test whether the gait parameters are predictors of fall measures in PPS patients. Spatio-temporal gait data of 50 individuals with PPS (25 males; age 65.9 ± 8.0) were acquired during gait and while performing the Timed Up-and-Go test. Subjects filled the Activities-specific Balance Confidence Scale (ABC Scale) and reported number of falls during the past year. ABC scores and number of falls correlated with the Timed Up-and-Go, and gait cadence and velocity. The number of falls also correlated with the swing duration symmetry index and the step length variability. Four gait variability parameters explained 33.2% of the variance of the report of falls (p = 0.006). The gait velocity was the best predictor of the ABC score and explained 24.8% of its variance (p = 0.001). Gait variability, easily measured by wearables or pressure-sensing mats, is an important predictor of falls in PPS population. Therefore, gait variability might be an efficient tool before devising a patient-specific fall prevention program for the PPS patient.
| S-EPMC8614826 | biostudies-literature