Patients With Post-polio Syndrome Research Articles

Spinal cord gray matter atrophy is associated with functional decline in post-polio syndrome.

ObjectiveTo determine if patients with post‐polio syndrome (PPS) show spinal cord gray matter (SCGM) atrophy and to assess associations between SCGM atrophy, muscle strength and patient‐reported functional decline.MethodsTwenty patients diagnosed with PPS (March of Dimes criteria) and 20 age‐ and sex‐matched healthy controls (HC) underwent 3T axial 2D‐rAMIRA magnetic resonance imaging at the intervertebral disc levels C2/C3–C6/C7, T9/T10 and the lumbar enlargement level (T max) (0.5 × 0.5 mm2 in‐plane resolution). SCGM areas were segmented manually by two independent raters. Muscle strength, self‐reported fatigue, depression and pain measures were assessed.ResultsPost‐polio syndrome patients showed significantly and preferentially reduced SCGM areas at C2/C3 (p = 0.048), C3/C4 (p = 0.001), C4/C5 (p < 0.001), C5/C6 (p = 0.004) and T max (p = 0.041) compared to HC. SCGM areas were significantly associated with muscle strength in corresponding myotomes even after adjustment for fatigue, pain and depression. SCGM areaTmax together with age and sex explained 68% of ankle dorsiflexion strength variance. No associations were found with age at or time since infection. Patients reporting PPS‐related decline in arm function showed significant cervical SCGM atrophy compared to stable patients adjusted for initial disease severity.ConclusionsPatients with PPS show significant SCGM atrophy that correlates with muscle strength and is associated with PPS‐related functional decline. Our findings suggest a secondary neurodegenerative process underlying SCGM atrophy in PPS that is not explained by aging or residua of the initial infection alone. Confirmation by longitudinal studies is needed. The described imaging methodology is promising for developing novel imaging surrogates for SCGM diseases.

The effect of adding whole-body vibration exercises to home exercise program on muscle strength in patients with post-polio syndrome.

Objectives This study aims to understand the effectiveness of whole-body vibration (WBV) exercises performed with home exercise program in patients with post-polio syndrome (PPS) in terms of muscle strength, fatigue, the quality of life, and laboratory parameters. Patients and methods Between October 2015 and March 2016, a total of 14 patients (7 males, 7 females; mean age: 45.1±6.6 years; range, 34 to 57 years) who were diagnosed with PPS were included in the study. The patients were randomized into two groups: the first group received WBV, home exercise program and patient education while the second (control) group received home exercise program and patient education. The patients were evaluated by knee isometric and isokinetic peak torque, Fatigue Severity Scale (FSS), Fatigue Impact Scale (FIS), Nottingham Health Profile (NHP), serum creatine kinase, aspartate aminotransferase, and alanine aminotransferase at baseline and at the end of the treatment. Results At the end of the treatment, the exercise program administered to the patients was found to be effective in increasing knee muscle strength in PPS patients (p<0.05). This difference was more apparent on the sequela side, isometric extension peak torque in the group with WBV. There was no significant change in the markers of muscle damage or NHP in both groups. Conclusion Inclusion of WBV in home exercise program in PPS patients does not seem to be superior, except for reducing the effect of fatigue on the patient's life.

Treatment with L‐Citrulline in patients with post‐polio syndrome: A single center, randomized, double blind, placebo‐controlled trial

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.

Effectiveness of Intravenous Immunoglobulin for Management of Pain in Patients with Postpolio Syndrome.

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.

Neurological Symptoms in Danes with a History of Poliomyelitis: Lifelong Follow-Up of Late Symptoms, their Association with Initial Symptoms of Polio, and Presence of Postpolio Syndrome

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.

P39. The split hand syndrome in ALS and post-polio-syndrom

Introduction Electrodiagnostic evaluation for amyotrophic lateral sclerosis (ALS) relies on extensive measurements. As one diagnostic clue, the split-hand-index (SHI) was proposed. It compares the compound muscle action potential (CMAP) of the abductor pollicis brevis (APB) muscle with the CMAP of the abductor digiti minimi (ADM) muscle. In ALS, asymmetric atrophy of APB and ADM results in the index being reduced compared to the healthy population. This holds true despite the fact, that there is the same segmental innervation C8 for both examined muscles, as was previously discussed. Several studies have shown a diagnostic value in differentiating ALS from other motorneuron-diseases by means of the SHI, claiming a specific form of neurodegeneration in ALS, which is less marked for example in lower motor neuron disease (LMND), spinal muscle atrophy (SMA) or Hirayama disease. In our study, we aimed to compare the SHI of ALS-patients with our cohort of patients with post-polio-syndrome (PPS) to find out, whether it has a discriminative value in these patients too and to add knowledge to the proposed neuroscientific explanations of asymmetric thenar/hypothenar-atrophy. Methods We conducted a retrospective analysis of our post-polio cohort since 1997. All patients were screened whether CMAPs of APB and ADM were collected. For comparison, we screened electrodiagnostic reports of all patients with a diagnosis of ALS for collected CMAPs of APB and ADM. We excluded patients with neuropathy of the median nerve (NMN) by means of a prolonged distal motoric latency (>4,4 ms). Finally we randomly chose the same number of patients from our reports in that time period with normal results (NR), by excluding diagnosis of NMN, acute polyneuropathies and radiculo- and plexopathies of the arm. We calculated the SHI by dividing the CMAP of APB by the CMAP of ADM. Results We found a significant difference (p = 0,01) of the SHI between ALS patients (0,97 ± 0,84) and the NR-group (1,26 ± 0,72). The SHI of the PPS-group (0,91 ± 0,55) was not significantly different compared to the NR group (p = 0,08), but showed a trend. Comparing the ALS-group with the PPS-patients, we found no statistically relevant difference (p = 0,83). Discussion As expected, we could reproduce a significant decrease of the SHI in ALS patients. However our results showed no statistically relevant difference between ALS-patients and PPS-patients when comparing the SHI. There is a broader distribution of values in the PPS-group, including cases of very high grade asymmetric atrophy of APB and ADM. A low SHI therefore is of no help in differentiating PPS from ALS-patients. As a limitation of our study it is important to acknowledge the retrospective study type and a possible selection bias of patients who suffer from an already clinically visible asymmetry, possible increasing electrodiagnostic evaluation numbers of the hand.

Restless legs syndrome is highly prevalent in patients with post-polio syndrome

ObjectiveFew 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. MethodThis was a cross-sectional study conducted from April 2010 to May 2012 at the outpatient Neuromuscular Disorders clinic of Universidade Federal de São Paulo, São 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. ResultsThe 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. ConclusionIn 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.

Síndrome post-polio, quejas cognitivas y exploración neuropsicológica

ObjectiveTo examine psychometric performance in a sample of patients with postpolio syndrome (PPS) with subjective cognitive complaints. Patients and methodsThe sample consisted of 37 PPS patients (17 men) referred to our outpatient neuropsychological service. The mean age at the neuropsychological assessment was 55 years (SD: 4.58 years). A total of 59.5% was receiving pharmacological treatment for anxiety-depression. The results obtained by patients in the neuropsychological assessment were compared with normative data to identify the percentage of patients with pathological scores on the tests applied. ResultsPatients with PPS had pathological scores on all the neuropsychological tests. A particularly significant finding was the percentage of patients with scores indicative of cognitive impairment in tests that assessed cognitive processing speed, verbal learning, phonemic verbal fluency and cognitive flexibility. No statistically significant differences were observed in patients’ performance depending on the presence or absence of anxiety or depression. ConclusionsPatients with PPS and subjective cognitive complaints performed less well on neuropsychological assessment than the general population.

Quality of life in Swedish patients with post-polio syndrome with a focus on age and sex

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.

Fatigue resistance of the knee extensor muscles is not reduced in post-polio syndrome

The present study investigated whether intrinsic fatigability of the muscle fibers is reduced in patients with post-polio syndrome (PPS). This may contribute to the muscle fatigue complaints reported by patients with PPS. For this purpose, we assessed contractile properties and fatigue resistance of the knee extensor muscles using repeated isometric electrically evoked contractions in 38 patients with PPS and 19 age-matched healthy subjects. To determine whether any difference in fatigue resistance between both groups could be attributed to differences in aerobic capacity of the muscle fibers, 9 patients with PPS and 11 healthy subjects performed the same protocol under arterial occlusion. Results showed that fatigue resistance of patients with PPS was comparable to that in controls, both in the situation with intact circulation and with occluded blood flow. Together, our findings suggest that there are no differences in contractile properties and aerobic muscle capacity that may account for the increased muscle fatigue perceived in PPS.

Test–retest reliability of the 6-min walk test in patients with postpolio syndrome

This study describes the test-retest reliability of the 6-min walk test (6MWT) and evaluates the possibility to perform the test three times on the same day in patients with postpolio syndrome (PPS). This was an intrarater test-retest reliability study. A total of 23 PPS patients (nine men, 14 women), mean age 67 years, participated in the study. The patients performed three 6MWT with a 30-min rest between each test. Borg scales were used to evaluate leg tiredness, dyspnea, and exertion before and after each test. The influence of walking aids on the outcome of the 6MWT was also evaluated. All patients (n=23) were able to complete the three 6MWT. The individual distances varied from 140-395 m. The relative reliability was high. The intraclass correlation coefficient (ICC2.1) (95% confidence interval) values ranged from 0.98 (0.95-0.99) (T1-T2) to 0.99 (0.97-0.99) (T2-T3) for the total number of participants. The SEM ranged from 12 to 9 m and the smallest real difference (SRD) ranged from 33 to 26 m for the total number of participants. Data indicate that the 6MWT is reliable for patients with PPS. For the high test-retest reliability, one test may be required. However, as a small learning effect was observed, the best result of two tests can be recommended when evaluating walking ability for research purposes.

A randomized controlled trial of IV immunoglobulin in patients with postpolio syndrome

ObjectiveTo investigate in a single-center randomized control trial whether a single IVIg course improves short-term outcome in patients with postpolio syndrome (PPS). MethodsFifty-one patients with PPS were randomly allocated to receive 2g/kg IVIg body weight or placebo infused over 5 consecutive days. The primary endpoint was health-related quality of life (HRQoL) limited to the physical component score (PCS) in the Short-Form-36 (SF-36). Secondary endpoints included the SF-36 mental component score (MCS), 6-minute walk test, visual analog scale, 101-numeric rating, and fatigue severity scale. Muscle strength was graded according to the Medical Research Council scale and by dynamometer. Primary and secondary outcome variables were tested double-blind at baseline, 2months, and 4months. ResultsAt two months, although SF-36 PCS scores were similar in both arms, the role physical (RP) domain improved significantly in the treatment arm (p=0.05) and so did the composite MCS (p=0.015), and role emotional (RE) subscale (p=0.02). No differences were found in the remaining outcome measures. At 4months, none of the outcome variables differed significantly between groups. ConclusionsAlthough the study did not reach the primary endpoint, we showed that a single IVIg course improves HRQoL related to mental activity, as measured by the SF-36 composite MCS, and role limitations including RP and RE SF-36 subscales at 2months, in patients with PPS. A single IVIg course leaves, gait, muscle strength, fatigue and bodily pain unchanged in patients with PPS. Classification of evidenceClass I evidence indicates that IVIg did not change SF-36 PCS, and Class II evidence indicates that IVIg improved scores on the SF-36 MCS, RP, and RE.

Case report: Effect of immunoglobulin on pain in Post-Polio Syndrome—Three case reports

Study design: Case reports. Setting: University hospital setting. Objective: To analyze the effect of intravenous immunoglobulin on neuropathic and nociceptive pain in three patients with Post-Polio Syndrome (PPS). Materials and Methods: Three patients with PPS and pain who received treatment with 90 g IvIg are described. Results: Before treatment one of the patients had pure neuropathic pain and the other two had a combination of neuropathic and nociceptive pain. There was no effect on pain in the patient with pure neuropathic pain and only effect on the nociceptive pain in the patients with a combination of neuropathic and nociceptive pain. Discussion: Pain is one of the most common symptoms in PPS. Previous studies have shown an effect on pain in PPS patients receiving IvIg. The results of the present study point to that the effect on pain is limited to nociceptive pain and that there is no effect on neuropathic pain which leads to increased knowledge of characterization of responders of IvIg treatment. Conclusion: IvIg treatment treatmentreduces nociceptive but not neuropathic pain in PPS patients.

IVIG treatment in post-polio patients: evaluation of responders

The aim of this work is to evaluate the outcome of IVIG treatment in patients with post-polio syndrome (PPS) and to identify responders. The study included 113 PPS patients who had received one IVIG treatment in an open trial, prospective follow-up study. Clinical examination was performed and clinical data were retrieved from medical records. The short form 36 (SF-36), physical activity scale for the elderly (PASE), and the visual analogue scale (VAS) were used as measurements of quality of life, physical activity, and the intensity of pain. Data before treatment and at 6-month follow-up were collected. Analysis was performed in subgroups based on demographic and medical parameters. A statistically significant increase of the SF-36 sub domains bodily pain, vitality, social function, role emotional, and the mental compound score (MCS) was found at the 6-month follow-up. A significant decrease of pain was found in patients who reported pain intensity over VAS of 20 mm, in patients younger than 65 years of age and in patients who had paresis in the lower extremities. A trend was found in patients with PPS as the only diagnosis. IVIG leads to increase of quality of life at 6-month follow-up for SF-36 regarding sub domains of bodily pain, vitality, social function, role emotional, as well as for pain. Age below 65 years, paresis in the lower extremities, and lack of concomitant disorders may be the main indicators for a future identification of responders.

Analysis of long-standing nociceptive and neuropathic pain in patients with post-polio syndrome

The purpose of this study was to analyze pain, both nociceptive and neuropathic, in patients with post-polio syndrome (PPS) and relate the pain to age at the initial polio infection, age at examination, to gender and disability. The study was conducted in a university hospital department. Patients with PPS were interviewed at their regular visits about pain, its character, intensity and localization. A clinical examination, including a thorough neurological examination, was performed. Data included age at time of polio infection, age at time of examination and gender. Pain intensity was measured with the VAS-scale and walking capability by the WISCI-scale. One hundred sixty-three (88 women, 75 men) patients were included in the study. Pain was present in 109 (67%). Pain was more frequently reported by women (82%) than by men (49%). 96 patients experienced nociceptive pain, 10 patients both neuropathic and nociceptive pain and three experienced pure neuropathic pain. Half of the patients with pain experienced pain in more than one body region. When neuropathic pain was present, another additional neurological disorder was diagnosed. Pain was more often found in younger patients (around 70%) than in older patients (around 50%). In summary pain is common in patients with PPS and most patients experienced nociceptive pain. Women have pain more often than men. Older patients experience pain more seldom than younger patients. Age at time of primary polio infection is important for the development of pain. When neuropathic pain is present, it is important to proceed with neurological examination to find an adequate diagnosis.

The challenge of diagnosing ALS in patients with prior poliomyelitis

Four patients with postpolio syndrome (PPS) developed ALS. Weakness and atrophy started from previously unaffected extremities but, contrary to PPS, spread to all muscles leading to death within 0.4 to 8 (mean 3.9) years. Upper motor neuron signs were absent in the atrophic limbs. Abundant spontaneous activity and group atrophy in newly affected muscles were prominent. ALS can rarely occur in the postpolio population starting de novo rather than as evolution of PPS.

Varied clinical patterns, physical activities, muscle enzymes, electromyographic and histologic findings in patients with post-polio syndrome in Taiwan.

A study of the clinical features, physical activity, muscle enzyme, electromyography and histopathological alternations of muscles in patients with post-polio syndrome (PPS). To assess the varied patterns of PPS in Taiwan. Taiwan. Thirty-one patients who fulfill the inclusion criteria of PPS were selected for study. Clinical features, physical activity scale, serum concentrations of creatine kinase, electromyography and histopathological alterations of muscles were assessed and correlated to the causes of PPS patients. Patients with PPS in Taiwan are relatively young, with a mean age of 39.3 years. Elevated concentration of creatine kinase was found predominantly in male patients with higher physical activities. Electromyographic examinations as well as histological tests of affected muscles revealed prominent evidence of chronic and active denervation with reinnervation in PPS patients. Patients with PPS in Taiwan are young. Thus, PPS should not be attributed to aging. Physical attrition with degradation of nerve terminals is considered the main cause of this disease. This study was supported by the National Science Council, Republic of China under grant no. NSC-83-0412-B002-302.

Illness narratives, dependence level and life satisfaction in post-polio syndrome

Objective. The purpose of this study was to investigate the lifetime illness experience of four patients with Post-Polio Syndrome (PPS). Method. The empirical contents were submitted to a categorization process containing questions referring to: (1) the acute phase of paralytic poliomyelitis and recollection of past clinical pictures and syndromes; (2) physical rehabilitation, guidance as well as care at services for the disabled; (3) adaptation to a new lifestyle; (4) living with PPS and related prejudices and misconceptions; (5) apprehensions towards the future. The level of functional independence (autonomy) was measured using the Barthel Index, a questionnaire regarding several practical motor skills. Results. We have found that the symptoms in post-polio patients increased their day-to-day level of motor disability and sometimes decreased their overall level of life satisfaction. Conclusion. Rehabilitation processes as well as counseling have a positive influence on (PPS) patients.

Antineurofilament antibodies in postpolio syndrome.

We determined the levels of antineurofilament antibodies in 29 patients with postpolio syndrome (PPS), 26 stable postpolio (PP) patients, 22 patients with ALS, and 20 normal controls (NCs). Patients with PPS had higher antibody levels to cholinergic neurofilaments than did all other groups. PP patients and those with ALS had antibody levels similar to those of NCs. The antibody binding level showed no relation to the age of the patients, duration of disease, or motor score.

Sleep apnea in patients with postpolio syndrome.

We studied sleep architecture and sleep apnea pattern in patients with postpolio syndrome (PPS). Ten patients with clinical signs of PPS underwent polysomnographic recording for two consecutive nights. Although sleep efficiency and proportions of sleep stages were within the normal range, sleep architecture was disrupted owing to sleep apnea. Patients with bulbar involvement had more frequent sleep apnea (mean sleep apnea index, 11.09) than patients without (apnea index, 5.88). The former also had significantly more central apnea, which occurred more commonly during non-rapid-eye-movement (NREM) than rapid-eye-movement (REM) sleep, than those without bulbar signs. This finding suggests reduction in forebrain control of compromised bulbar respiratory centers during NREM sleep in PPS.