Post-polio Syndrome Patients Research Articles

Risk factors for post-polio syndrome among an Italian population: a case–control study

Post-polio syndrome (PPS) is a clinical syndrome of new weakness, fatigue and musculoskeletal pain occurring in a variable proportion of polio survivors decades after acute disease. To date, several risk factors for PPS development have been reported, although the etiology of this disorder remains elusive. Using a case-control design, we aimed to assess risk indicators for PPS in a group of Italian polio survivors. Subjects with prior poliomyelitis attending the rehabilitation hospital of Malcesine, Italy, were the target population. Patients with PPS, diagnosed according to the European Federation of Neurological Societies criteria, served as cases, while patients not meeting diagnostic criteria for PPS were used as controls. All subjects were assessed through a structured questionnaire made of 82 questions and neurological examination. The association with investigated risk factors (sex, age at polio onset, age at onset of symptoms, extension and severity of polio, employment) was analyzed by the calculation of the odds ratio. A total of 161 out of 391 eligible patients met the adopted diagnostic criteria for PPS, giving a frequency of 41.2%. Symptoms most frequently complained by PPS patients were loss of muscle strength, loss of resistance, loss of muscle volume and generalized fatigue. Female gender, the presence of respiratory disturbance during the acute phase of polio and the use of orthoses and aids during the recovery and stabilization represented independent risk factors for PPS in the studied population.

Post-polio syndrome: impact of hope on quality of life

Purpose: To determine the effect of future-oriented coping strategies on the quality of life (QOL) of individuals with post-polio syndrome (PPS). Methods: A correlative study, in which a cohort of 61 patients was surveyed and a group of 40 healthy, age-matched individuals served as controls. Patients were surveyed as to their QOL, levels of hope and utilization of proactive coping, employment status and degree of functionality. Results: PPS patients had lower total, physical and mental QOL indices compared to controls. Future-oriented coping strategies associated with hope were positively associated with physical and mental QOL in the PPS group, but not in the controls. In a multivariate analysis, hope and employment status predicted higher QOL among those with PPS. Conclusions: Future-oriented coping strategies, particularly hope are distinctively associated with improved QOL benefits in PPS patients. Fostering future-oriented coping related to hope may improve the self-perceived mental and physical status of patients with PPS.Implications for RehabilitationCoping styles of individuals with post-polio can be conceptualized in terms of positive-optimistic psychological constructs rather than the traditional negative frame of reference.Hope is distinctively associated with improved quality of life among post-polio patients.Enhancing hope through psychotherapy may improve mental and physical health of individuals with postpolio.

Leukocyte myeloperoxidase and pathogenesis of the post-polio syndrome

Dear Sir, In 2006 the European Federation of Neurological Societies (EFNS) provided guidelines [1] on the diagnosis and management of the post-polio syn-drome (PPS). Thus the diagnosis of PPS should be reserved only for patients who clinically and/or by laboratory means present a confi rmed history of paralytic polio. Secondly, they should have recovered completely or almost completely and have stayed stable neurologically and functionally for at least 15 y after the acute poliovirus infection. Symptoms and signs of PPS include fatigue and gradually increasing muscular weakness, muscle atrophy and pain, some-times combined with arthralgia and intolerance to cold. Furthermore, according to the criteria adopted by the EFNS consensus group, symptoms and clini-cal observations must not be explicable by reference to another neurological diagnosis. PPS is by defi nition related to a previous paretic poliovirus infection, but the pathogenesis of the pro-gressive chronic muscle weakness is still essentially unknown. Several hypotheses for the pathophysiol-ogy of PPS have been discussed. One of these refers to the possible persistence of poliovirus in the central nervous system (CNS). The advances made in molecular poliovirus in the 1990s, with new cell and animal models – studies reviewed by Blondel et al. [2] – suggest that at least under certain experimental conditions the poliovirus infection might persist for a considerable time without killing cultured cells or infected animals. Using the polymerase chain reac-tion (PCR), attempts were made to trace poliovirus RNA sequences in cerebrospinal fl uid (CSF), blood lymphocytes and post-mortem PPS tissues. Initially, observations in PPS patients [3,4] were interpreted as compatible with persisting CNS polio or other enterovirus infection. However, the possible associa-tion of PPS and poliovirus persistency has gradually lost most of its relevance. Thus it has not been pos-sible, as yet, to associate the development of PPS with a persistent CNS poliovirus infection [5,6]. Hypothetically, cell destructive immune reactions might in a multitude of ways contribute to motor neuron degeneration such as that seen in PPS. A plethora of immune reactions may be pivotal, affect-ing life-span and functions of motor neurons. In par-ticular, reactions related to autoimmunity have been looked for, testing possible implications of B- as well as T-cell-mediated immunity. PPS patients display CSF mononuclear cells expressing mRNAs for tumour necrosis factor alpha (TNF- α), interferon gamma (IFN- γ), interleukin (IL)-4 and IL-10 [7]. Although the impact of cytokines on immune reac-tions and infl ammation in PPS has not been studied in detail, it may be assumed that while IL-4 and IL-10 will modify and suppress adverse infl amma-tory reactions, the presence of TNF- α and IFN- γ may promote this response. The neutrophil and monocyte nicotinamide ade-nine dinucleotide phosphate (NADPH) oxidase has a central role in infl ammation by the release of reac-tive oxygen species ( ‘ oxygen radicals ’ ) such as super-oxide anions and hydrogen peroxide (H

Analysis of sleep characteristics in post-polio syndrome patients

The main post-polio syndrome (PPS) symptoms are new weakness, new atrophy, fatigue, pain and sleep disturbances. Polysomnography is the gold standard for sleep analysis. To analyze sleep patterns in PPS patients. Sixty patients (mean age 46.8+/-11.3 years) at the Federal University of São Paulo (UNIFESP/EPM) complaining of sleep disturbances were evaluated by means of polysomnography, performed at the Sleep Institute. Sleep efficiency was lower due to high sleep latency and arousal index. The apnea and hypopnea index (AHI) and the periodic limb movements (PLM) index were higher. Sleep architecture was also impaired. There were no abnormalities of oxygen saturation, carbon dioxide levels, respiratory rate or heart rate. New post-polio sleep disturbances were isolated symptoms. It appears that these symptoms were not due to post-polio features, but rather, that they were due to dysfunction of the surviving motor neurons in the brainstem. Abnormal dopamine production, which is responsible for many sleep-related breathing disorders and abnormal movements, may also have been implicated in the present findings.

Sleep disorders frequency in post-polio syndrome patients caused by periodic limb movements

Post-polio syndrome (PPS) in individuals with polio longer than 15 years is characterized by weakness and/or muscle fatigue, deficit of deglutition and breath and periodic limb movements (PLM) during sleep. We undertook a review of 99 patients with PPS, and assessed the frequency of PLM through polysomnographic recordings at our sleep disorders unit. The total number of PLM, total time of sleep (TTS), efficiency of sleep (EfS), awaking index (AI) and apnea-hypopnea index (AHI) were analyzed. Sixteen patients presented PLM in excess of 5 for the entire night. When comparing these with the group without PLM, a correlation was found (p=0.001). Significant difference was found for the correlation of the parameters: IAH, ID, TTS and EfS when compared the two groups. There is a close relationship between PPS and PLM.

Tratamiento de la fatiga en el síndrome pospoliomielitis. Revisión sistemática

Fatigue is the most common symptom and the most disabling in patients with post-polio syndrome. To analyze the effectiveness of various treatments used to improve fatigue syndrome patients post-polio. Systematic review. Is defined a bibliographic search strategy in Medline (from 1961), EMBASE (from 1980), ISI Web of Knowledge and Cochrane Library, Cochrane Central Register of Controlled Trials (CENTRAL), AMED (January 1985), EMI and Physiotherapy Evidence Database (PEDro) until February 2008, the population defined (post-polio syndrome patients) and intervention (any treatment for fatigue in these patients). Outcome were selected as different scales of fatigue and fatigue or vitality dimension scales quality of life. Clinical trials were selected. We retrieved 396 articles, of which 23 were analyzed in detail. Finally, 19 were included in the analysis, a total of 705 patients. Lamotrigine, bromocriptine, aerobics and flexibility exercises, hydrokinesitherapy and technical aids are treatment techniques that reduce more fatigue in these patients.

Post-polio syndrome: Pathophysiological hypotheses, diagnosis criteria, medication therapeutics

Post-polio syndrome (PPS) refers to a clinical disorder affecting polio survivors with sequelae years after the initial polio attack. These patients report new musculoskeletal symptoms, loss of muscular strength or endurance. PPS patients are tired, in pain and experience new and unusual muscular deficits, on healthy muscles as well as deficient muscles initially affected by the Poliovirus. Once a clinical diagnosis is established, the therapeutic options can be discussed. Some pathophysiological mechanisms have been validated by research studies on PPS (inflammatory process in cerebrospinal fluid [CSF] and cytokines of the immune system). Several studies have been conducted to validate medications (pyridostigmine, immunoglobulin, coenzyme Q10) or physical exercises protocols. This article focuses on the relevance and efficacy that can be expected from these therapeutics. Very few studies reported some improvements. Medications combined to individual and supervised exercise training programs are promising therapeutic strategies for PPS patients care management.

Effects of the use of MIG3 bioceramics fabrics use - long infrared emitter - in pain, intolerance to cold and periodic limb movements in post-polio syndrome

The main post-polio syndrome (PPS) symptoms are new-onset weakness, new-onset atrophy, fatigue, cold intolerance, and pain associated with sleep disturbances. The polysomnographic study is the gold pattern to analyze sleep disorders. To assess pain, intolerance to cold and periodic limb movements (PLM) index before and after the use of MIG3 bioceramic fabrics over 4 weeks. 12 patients with PPS from UNIFESP/EPM. All patients were submitted to polysomnography and infra-red examinations with answered scales of pain and intolerance to cold before and after the use of MIG3 bioceramics fabrics. There were significant decreases in pain and PLM index. MIG3 bioceramic fabrics can help in the treatment of pain and PLM in PPS patients.

Identification of novel candidate protein biomarkers for the post-polio syndrome — Implications for diagnosis, neurodegeneration and neuroinflammation

Survivors of poliomyelitis often develop increased or new symptoms decades after the acute infection, a condition known as post-polio syndrome (PPS). The condition affects 20-60% of previous polio patients, making it one of the most common causes of neurological deficits worldwide. The underlying pathogenesis is not fully understood and accurate diagnosis is not feasible. Herein we investigated whether it was possible to identify proteomic profile aberrations in the cerebrospinal fluid (CSF) of PPS patients. CSF from 15 patients with well-defined PPS were analyzed for protein expression profiles. The results were compared to data obtained from nine healthy controls and 34 patients with other non-inflammatory diseases which served as negative controls. In addition, 17 samples from persons with secondary progressive multiple sclerosis (SPMS) were added as relevant age-matched references for the PPS samples. The CSF of persons with PPS displayed a disease-specific and highly predictive (p=0.0017) differential expression of five distinct proteins: gelsolin, hemopexin, peptidylglycine alpha-amidating monooxygenase, glutathione synthetase and kallikrein 6, respectively, in comparison with the control groups. An independent ELISA confirmed the increase of kallikrein 6. We suggest that these five proteins should be further evaluated as candidate biomarkers for the diagnosis and development of new therapies for PPS patients.

Basal Metabolic Rate and Body Composition in Patients with Post-Polio Syndrome

The aim of this study was to compare basal metabolic rate (BMR) of post-polio syndrome (PPS) patients with healthy individuals and to determine its correlation to body composition. BMR (kcal/day) was determined by indirect calorimetry and body composition by dual energy X-ray absorptiometry. BMR was lower in the PPS patient group than in the control group, although it was similar in both groups when adjusted for body surface area, total body mass (TBM), lean body mass (LBM) and fat-free mass (FFM). PPS patients also showed reduced TBM, LBM and FFM in relation to controls. As muscle energy expenditure while at rest contributes only 20% to the BMR, a proportional reduction in BMR and FFM or LBM could suggest that muscle mass or other factors may interfere more than predicted. It was concluded that the prediction of BMR from the Harris-Benedict equation in PPS patients must be carefully reviewed.

Immediate effects of a controllable knee ankle foot orthosis for functional compensation of gait in patients with proximal leg weakness

Application of intermittent control of the knee joint stiffness in a knee ankle foot orthosis (KAFO) during gait is proposed. The approach combines inertial sensors and an actuator system in order to apply compensation in quadriceps weakness with a wearable device. Two methods, segment-angular rotation based and segment-angular velocity based, are analysed for the control of the knee joint state (intermittent stiffness) based on the inertial sensors signals. Protocolled tests are developed with two post-polio syndrome patients (PPS). In this study, the cases of gait with free-swinging leg and safe stance with the orthotic system are presented in terms of quantified kinematics (average peak angle of knee flexion of 50 degrees ) and evidences of reduction of frequent compensations (e.g. leg lateral movement) in post-polio syndrome patients. The results from immediate inspection indicate an important improvement of the gait patterns in two patients with proximal leg weakness by means of compensations applied by the wearable orthosis.

Circulating insulin-like growth factors and related binding proteins are selectively altered in amyotrophic lateral sclerosis and multiple sclerosis

Objective To provide a detailed profile of the peripheral IGF system in the neurological conditions; amyotrophic lateral sclerosis (ALS), post polio syndrome (PPS) and multiple sclerosis (MS). To determine whether subsets of patients within the disease groups could be identified in whom one or more components of the IGF regulatory system are altered compared to healthy control subjects matched for age, sex and BMI. Design Three cohorts of patients were recruited, 28 with ALS, 18 with PPS and 23 with MS. Patients were individually matched to a healthy control based on sex, age (±3yr), and BMI (±2.5 kg·m −2). The concentration (ng/ml) of serum IGF-I, IGF-II, IGFBP-1, IGFBP-2 and IGFBP-3 and acid-labile subunit (μg/ml) was determined by IRMA. Results In ALS patients, there was an increase of 11% in [IGF TOTAL] ( p = 0.042) ([IGF TOTAL] = [IGF-I] + [IGF-II]) and [IGFBP-1] was decreased by 34% ( p = 0.050) compared to matched controls. In “surviving” ALS patients, defined as those ALS patients with long disease duration (+2 SD from the mean survival time for Irish patients post diagnosis), there was an increase in [IGF-I] 36% ( p = 0.032) and a large decrease in [IGFBP-1] –58% ( p = 0.020) compared to controls. These differences were not evident in pre-agonal ALS patients. The concentration of serum IGF-I was 38% ( p = 0.018), acid-labile subunit 17% ( p = 0.044) and IGFBP-2 43% ( p = 0.035) higher in MS patients compared to controls. When stratified for interferon-beta (IFN-β) use, we observed an increase in serum [IGF-I] 52% ( p = 0.013) and [IGF TOTAL] 19% ( p = 0.043) in MS patients undergoing IFN-β treatment, but MS patients not undergoing IFN-β treatment had similar IGF and IGFBP concentration to controls. Serum [IGFBP-3] 18% ( p = 0.033), [IGFBP-2] 86% ( p = 0.015) and (acid-labile subunit) 33% ( p = 0.012) was also higher in IFN-β patients compared to controls. Stratified by stage of disease the most significant increase in components of the peripheral IGF system was attributed to relapsing-remitting MS patients treated with IFN-β. All components of the peripheral IGF system in PPS patients were similar to controls. Conclusions The increase in circulating IGF-I and a reduction in regulatory binding protein IGFBP-1 in ALS patients with a “stable” disease profile suggest a potential change in peripheral IGF bioavailability in these subjects. In MS, we report a change in a number of components of the peripheral IGF system, the observed increase in IGF-I in patients treated with IFN-β being of most significance as a potential therapeutic biomarker.

Virology of the Post-Polio Syndrome

The three poliovirus serotypes (PVs) cause acute paralytic poliomyelitis. Decades after being hit by polio, survivors may develop a condition known as post-polio syndrome (PPS). PPS is characterized by extreme fatigue, progressing muscular weakness and chronic pain. The pathogenesis is unclear and, thus, empirical therapies are employed. PVs are known to be able to persist in infected host cells both in vitro and in vivo. The understanding of PV genomes has made it possible to set up sensitive and specific molecular tests capable of detecting minute amounts of virus in samples from PPS patients. Current data indicate that complete PV genomes (or genomic fragments) remain present, decades after acute paralysis, in the CNS of these patients. Virus persistence is hypothesized to bring about chronic inflammation, immune-mediated injury and decreased expression of neurotrophic factors. Establishing a pathogenetic link between PV persistence and PPS would be extremely relevant to the development of an etiologic therapy aimed at virus eradication.

Post‐polio syndrome patients treated with intravenous immunoglobulin: a double‐blinded randomized controlled pilot study

Post-polio syndrome (PPS) is characterized by new muscle weakness, atrophy, fatigue and pain developing several years after the acute polio. Some studies suggest an ongoing inflammation in the spinal cord in these patients. From this perspective, intravenous immunoglobulin (IvIg) could be a therapeutic option. We performed a double-blinded randomized controlled pilot study with 20 patients to investigate the possible clinical effects of IvIg in PPS. Twenty patients were randomized to either IvIg 2 g/kg body weight or placebo. Primary endpoints were changes in pain, fatigue and muscle strength 3 months after treatment. Surrogate endpoints were changes in cerebrospinal fluid (CSF) cytokine levels. Secondary endpoints were pain, fatigue and isometric muscle strength after 6 months. Patients receiving IvIg reported a significant improvement in pain during the first 3 months, but no change was noted for subjective fatigue and muscle strength. CSF levels of tumour necrosis factor-alpha (TNF-alpha) were increased compared with patients with non-inflammatory neurological disorders. In conclusion, in this small pilot study no effect was seen with IvIg treatment on muscle strength and fatigue, however IvIg treated PPS patients reported significantly less pain 3 months after treatment. TNF-alpha was increased in the CSF from PPS patients. The results are promising, but not conclusive because of the low number of patients studied.

Knee orthosis for genu recurvatum of the post-polio syndrome patients

Knee orthosis for genu recurvatum of the post-polio syndrome patients

EFNS guideline on diagnosis and management of post‐polio syndrome. Report of an EFNS task force

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

Prior poliomyelitis—IvIg treatment reduces proinflammatory cytokine production

The postpolio syndrome (PPS) is characterized by progressive disabilities decades after recovery from the acute paralytic disease. There are reports on intrathecal inflammatory reactions in PPS, including increased expression of cytokines by cerebrospinal fluid (CSF) mononuclear cells (CSF-MC). This is potentially of relevance for the clinical condition. We here explored if cytokine expression in the CSF of PPS patients could be modulated by high-dose intravenous immunoglobulins (IvIg). The expression of TNF-α, IFN-γ, IL-10 and IL-4 mRNAs was measured by real-time RT-PCR in CSF and peripheral blood mononuclear cells (PBMC) of 16 PPS patients before, and 6–8 weeks after IvIg treatment, and in 26 patients with noninflammatory other neurological diseases (OND). TNF-α, IFN-γ and IL-10 CSF mRNA levels were elevated in samples from untreated persons with PPS compared to OND. Upon IvIg treatment, IFN-γ and TNF-α mRNA levels were dramatically reduced, while IL-10 remained unchanged. Placebo-controlled studies are now warranted to evaluate if IvIg treatment also has any effects on the clinical manifestations of PPS.

GM1 antibodies in post-polio syndrome and previous paralytic polio

We studied the relationship between post-polio syndrome (PPS) and GM1 antibodies, since such antibodies have been associated with PPS and motor neuron disorders. Sera from 144 patients with previous poliomyelitis (105 paralytic, 22 nonparalytic and 17 PPS), 60 with previous Guillain-Barré syndrome, 44 with amyotrophic lateral sclerosis (ALS) and 22 healthy blood donors were analyzed with ELISA for GM1 IgM, IgG and IgA antibodies. GM1 antibodies were present in 14% of the PPS patients, but the prevalence did not differ significantly from that of the other groups. Our study does not support the hypothesis that GM1 antibodies are involved in the pathogenesis of PPS.

A personality profile of patients diagnosed with post‐polio syndrome

Post-polio syndrome (PPS) refers to the late development of new neuromuscular symptoms in previously stable poliomyelitis patients. Whether psychological disturbance plays a role in the manifestation of symptoms in these patients is unclear. We examined 22 patients fulfilling the clinical criteria for PPS with the Minnesota Multiphasic Personality Inventory-II (MMPI-II), Beck Depression Inventory, Spielberger State-Trait Anxiety Scales, Chapman and Chapman Psychosis-Proneness Scales, Fatigue Scales, a neurobehavioral rating scale, and Cognitive Symptoms Self-Report Scales. The overwhelming majority of scale scores were within normal limits, and there was no indication that psychopathologic symptoms were associated with the development or severity of new muscle weakness in PPS patients. Women with PPS had significantly more somatic complaints, but were less socially isolated than men with PPS. This study confirms that the development or severity of new muscle weakness in carefully diagnosed PPS patients is not due to, or influenced by, underlying psychopathology.