Functional exercise capacity in maximal and submaximal activities of individuals with polio sequelae.

PURPOSE: Poliomyelitis is an infectious disease that can cause total paralysis. Furthermore, poliomyelitis survivors may develop new signs and symptoms, including muscular weakness and fatigue, years after the acute phase of the disease, i.e., post-polio syndrome (PPS). Thus, the objective was to compare the functional exercise capacity during maximal and submaximal exercises among individuals with polio sequelae (without PPS diagnosis), PPS, and a control group. METHODS: Thirty individuals participated in three groups: a control group (CG, n = 10); a group of individuals with polio sequelae but without PPS diagnosis (PG, n = 10); and a PPS group (PPSG, n = 10). All participants underwent (i) a cardiopulmonary exercise test to determine their maximal oxygen uptake ([Formula: see text]) and (ii) a series of functional field tests (i.e., walking test, sit-to-stand test, and stair climbing test). RESULTS: [Formula: see text]O2max was 30% lower in PPSG than in CG and PG. Regarding functional field tests, walking and stair climbing test performances were significantly different among all groups. The PPSG sit-to-stand performance was lower than CG. CONCLUSION: The sequelae of paralytic poliomyelitis impair functional exercise capacity obtained from maximal and submaximal tests, especially in patients with PPS. Furthermore, submaximal variables appear to be more negatively impacted than maximal variables.

Describing post-polio syndrome.

INTRODUCTION: Patients presenting sequelae of poliomyelitis may present new symptoms, known as post-polio syndrome (PPS). OBJECTIVE: To identify the clinical and functional profile and epidemiological characteristics of patients presenting PPS. PATIENTS AND METHODS: We performed a retrospective study of 400 patients with poliomyelitis attended at the Institut Guttmann outpatient clinic, of whom 310 were diagnosed with PPS. We describe patients' epidemiological, clinical, and electromyographic variables and analyse the relationships between age of poliomyelitis onset and severity of the disease, and between sex, age of PPS onset, and the frequency of symptoms. RESULTS: PPS was more frequent in women (57.7%). The mean age at symptom onset was 52.4 years, and was earlier in women. Age at primary infection > 2 years was not related to greater poliomyelitis severity. The frequency of symptoms was: pain in 85% of patients, loss of strength in 40%, fatigue in 65.5%, tiredness in 57.8%, cold intolerance in 20.2%, dysphagia in 11.7%, cognitive complaints in 9%, and depressive symptoms in 31.5%. Fatigue, tiredness, depression, and cognitive complaints were significantly more frequent in women. Fifty-nine percent of patients presented electromyographic findings suggestive of PPS. CONCLUSIONS: While the symptoms observed in our sample are similar to those reported in the literature, the frequencies observed are not. We believe that patients' clinical profile may be very diverse, giving more weight to such objective parameters as worsening of symptoms or appearance of weakness; analysis of biomarkers may bring us closer to an accurate diagnosis.

Describiendo el síndrome pospolio / Describing post-polio syndrome

Introducción: Las personas con secuelas de poliomielitis pueden presentar nuevos síntomas que constituirían el síndrome pospolio (SPP). Objetivo Identificar el perfil clínico y funcional, y las características epidemiológicas de personas que padecen SPP. Pacientes y métodos: Estudio retrospectivo de 400 pacientes afectados de poliomielitis visitados en consulta externa del Institut Guttmann, de los cuales a 310 se les diagnosticó SPP. Se describieron variables epidemiológicas, clínicas y electromiográficas. Se analizó la relación entre edad de adquisición de la polio y gravedad de la misma, así como entre el sexo y la edad de aparición del SPP y la frecuencia de síntomas. Resultados: Se observó mayor frecuencia de SPP en mujeres (57,7%). La edad media de inicio de la clínica fue 52,4 años, más precoz en mujeres. Edad de primoinfección mayor de 2 años no se relacionó con mayor gravedad de la polio. La frecuencia de síntomas fue: dolor 85%, pérdida de fuerza 40%, fatiga 65,5%, cansancio 57,8%, intolerancia al frío 20,2%, disfagia 11,7%, quejas cognitivas 9%, síntomas depresivos 31,5%. La fatiga, el cansancio, la depresión y las quejas cognitivas fueron significativamente más frecuentes en mujeres. El 59% de los pacientes presentaban hallazgos electromiográficos sugestivos de SPP. Conclusiones: El tipo de sintomatología que presentaba nuestra muestra es similar a la publicada, no así en la frecuencia de la misma. Creemos que el perfil clínico de los pacientes podría ser muy diverso, y dar mayor peso a parámetros objetivos como el empeoramiento o la aparición de debilidad y el estudio de biomarcadores podría acercarnos más a un diagnóstico preciso. (AU)

Introduction: Patients presenting sequelae of poliomyelitis may present new symptoms, known as post-polio syndrome (PPS). Objective: To identify the clinical and functional profile and epidemiological characteristics of patients presenting PPS. Patients and methods: We performed a retrospective study of 400 patients with poliomyelitis attended at the Institut Guttmann outpatient clinic, of whom 310 were diagnosed with PPS. We describe patients’ epidemiological, clinical, and electromyographic variables and analyse the relationships between age of poliomyelitis onset and severity of the disease, and between sex, age of PPS onset, and the frequency of symptoms. Results: PPS was more frequent in women (57.7%). The mean age at symptom onset was 52.4 years, and was earlier in women. Age at primary infection > 2 years was not related to greater poliomyelitis severity. The frequency of symptoms was: pain in 85% of patients, loss of strength in 40%, fatigue in 65.5%, tiredness in 57.8%, cold intolerance in 20.2%, dysphagia in 11.7%, cognitive complaints in 9%, and depressive symptoms in 31.5%. Fatigue, tiredness, depression, and cognitive complaints were significantly more frequent in women. Fifty-nine percent of patients presented electromyographic findings suggestive of PPS. Conclusions: While the symptoms observed in our sample are similar to those reported in the literature, the frequencies observed are not. We believe that patients’ clinical profile may be very diverse, giving more weight to such objective parameters as worsening of symptoms or appearance of weakness; analysis of biomarkers may bring us closer to an accurate diagnosis. (AU)

Postpolio Syndrome from Non-paralytic Poliovirus Infection.

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.

[Post-polio Syndrome in the Perioperative Phase]. / Das Post-Polio-Syndrom in der perioperativen Phase.

Due to the numerous poliomyelitis epidemics that have continued over the last decades and the post-polio syndrome (PPS) that occurs 10 - 30 years after poliomyelitis infection, the prevalence of PPS is also expected to increase in Europe. At the same time, due to the musculoskeletal disorders associated with the underlying disease, PPS patients often require surgery for which special anaesthetic requirements must be taken into account. In this analysis we summarise the current evidence and recommendations.

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.

Whole body vibration on people with sequelae of polio.

PURPOSE: 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). METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

[Polio and post-polio syndrome, viewed by patients and health professionals in primary care]. / Polio y postpolio. Visión de pacientes y profesionales en atención primaria.

OBJECTIVE: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

OBJETIVO: 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. METODOS: 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. RESULTADOS: 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). CONCLUSIONES: 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.

Postpolio syndrome and the late effects of poliomyelitis. Part 1. pathogenesis, biomechanical considerations, diagnosis, and investigations.

Postpolio syndrome (PPS) is characterized by new muscle weakness and/or muscle fatigability that occurs many years after the initial poliomyelitis illness. Many theories exist regarding the pathogenesis of PPS, which remains incompletely understood. In contrast, the late effects of poliomyelitis are often a consequence of biomechanical alterations that occur as a result of polio-related surgeries, musculoskeletal deformities, or weakness. Osteoporosis and fractures of the polio-involved limbs are common. A comprehensive clinical evaluation with appropriate investigations is essential to fulfilling the established PPS diagnostic criteria. PPS is a diagnosis of exclusion in which a key clinical feature required for the diagnosis is new muscle weakness and/or muscle fatigability that is persistent for at least 1 year. Electromyographic and muscle biopsy findings including evidence of ongoing denervation cannot reliably distinguish between patients with or without PPS. Muscle Nerve 58:751-759, 2018.

Post-polio syndrome and the late effects of poliomyelitis: Part 2. treatment, management, and prognosis.

Post-polio syndrome (PPS) is characterized by new muscle weakness and/or muscle fatigability that occurs many years after the initial poliomyelitis illness. An individualized approach to rehabilitation management is critical. Interventions may include rehabilitation management strategies, adaptive equipment, orthotic equipment, gait/mobility aids, and a variety of therapeutic exercises. The progression of muscle weakness in PPS is typically slow and gradual; however, there is also variability in both the natural history of weakness and functional prognosis. Further research is required to determine the effectiveness of selected medical treatment. Muscle Nerve 58:760-769, 2018.

Problems in Comparing Jitter Values Obtained with Voluntary Activation and Electrical Stimulation.

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.

Ultrasound-Guided Carpal Tunnel Release Using Dynamic Expansion of the Transverse Safe Zone in a Patient With Postpolio Syndrome: A Case Report.

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.

Measuring Physical and Cognitive Fatigue in People With Post-Polio Syndrome: Development of the Neurological Fatigue Index for Post-Polio Syndrome (NFI-PP).

BACKGROUND: Fatigue in post-polio syndrome (PPS) has been shown to affect quality of life adversely. There is currently no disease-specific measure of fatigue for PPS. OBJECTIVE: To develop a scale to measure fatigue in PPS that meets rigorous psychometric standards. DESIGN: Qualitative followed by validation and test-retest studies. SETTING: Polio clinic followed by national questionnaire studies. PARTICIPANTS: A total of 45 participants from polio clinic for qualitative; 319 participants from clinic or self-referral for validation study, of whom 87 completed the retest questionnaire. METHODS: Draft questionnaire items on PPS fatigue were derived from transcripts of qualitative interviews. After cognitive debriefing, the draft measure was administered by mail along with comparator questionnaires to a new sample. MAIN OUTCOME MEASUREMENTS: Draft PPS fatigue measure, Fatigue Severity Scale, and visual analog scale for fatigue. RESULTS: Analysis of 271 of 319 (85%) questionnaires identified a 2-factor solution (RMSEA 0.058). For the physical subscale, a 20-item scale showed good fit (χ2P = .189), strict unidimensionality (t-test 5.17%), and reliability 0.91. For the cognitive subscale, a 7-item scale showed excellent fit (χ2P = .917), strict unidimensionality (t-test 5.2%), and reliability 0.89. Evidence of a "difficulty factor" emerged also supporting a total score that showed good fit (χ2P = .151), strict unidimensionality (t-test 0.4%), and reliability consistent with group use at 0.73. Test-retest correlations for all scales were greater than 0.85. Standard error of measurement on metric ranges was 5.4 for total, 2.9 for physical, and 1.69 for cognitive domains. With the latent estimate of the total score transformed to a 0-100 scale, the mean score was 49.5 (SD 6.9). Spearman correlations with the Fatigue Severity Scale and visual analog scale were 0.60 and 0.55, respectively. CONCLUSIONS: A patient-derived Neurological Fatigue Index for PPS, with physical and cognitive subscales and a total score, has demonstrated good reliability, appropriate concurrent validity, and satisfies the Rasch measurement model. A raw-score to interval scale transformation is available for parametric applications and the calculation of change scores. LEVEL OF EVIDENCE: III.

Relationship of depression and medications on incidence of falls among people with late effects of polio.

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.

Treatment with L-citrulline in patients with post-polio syndrome: study protocol for a single-center, randomised, placebo-controlled, double-blind trial.

BACKGROUND: Post-polio syndrome (PPS) is a condition that affects polio survivors years after recovery from an initial acute infection by the Poliomyelitis virus. Most often, patients who suffered from polio start to experience gradual new weakening in muscles, a gradual decrease in the size of muscles (muscle atrophy) and fatigue years after the acute illness. L-citrulline is known to change muscular metabolism synthesis by raising nitric oxide (NO) levels and increasing protein synthesis. This investigator-initiated, randomised, placebo-controlled, double-blind, trial aims to demonstrate that L-citrulline positively influences muscle function and increases muscular energy production in patients with PPS. METHODS/DESIGN: Thirty ambulant PPS patients will be recruited in Switzerland. Patients will be randomly allocated to one of the two arms of the study (placebo:verum 1:1). After a 24-week run-in phase to observe natural disease history and progression, participants will be treated either with L-citrulline or placebo for 24 weeks. The primary endpoint is change in the 6-min Walking Distance Test. Secondary endpoints will include motor function measure, quantitative muscle force, quantitative muscle magnetic resonance imaging and magnetic resonance spectroscopy and serum biomarker laboratory analysis DISCUSSION: The aim of this phase IIa trial is to determine if treatment with L-citrulline shows a positive effect on clinical function and paraclinical biomarkers in PPS. If treatment with L-citrulline shows positive effects, this might represent a cost-efficient symptomatic therapy for PPS patients. TRIAL REGISTRATION: ClinicalTrial.gov, ID: NCT02801071 . Registered on 6 June 2016.

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.

Outcomes of total hip replacement in limbs affected by poliomyelitis.

INTRODUCTION: The 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. METHODS: A 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. RESULTS: 2 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. CONCLUSIONS: Total 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.