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At least 19 recordsLinked to original sources

Laryngeal manifestations of postpoliomyelitis syndrome.

Postpoliomyelitis syndrome (PPS) is a disease that may occur in survivors of acute poliomyelitis several decades after their initial infection. It can present as dysphonia, with vocal weakness and fatigue. Swallowing, respiratory, and other laryngopharyngeal symptoms may be manifestations of the disease or they may represent worsening of previously stable and compensated deficits. Three cases of laryngeal changes in PPS with videostroboscopic and laryngeal electromyography findings highlight the features of this disorder. We review possible etiologies of laryngeal PPS, diagnostic criteria, and treatment, as well as the current literature.

Acute Disease↗

Vocal cord paralysis in postpoliomyelitis syndrome.

Postpoliomyelitis syndrome is characterized by new neuromuscular symptoms, including weakness, developing years after recovery from acute polio. Bilateral vocal cord paralysis is presented as a new manifestation of this syndrome. Other clinical features of post-poliomyelitis syndrome in this report are discussed. The etiology of this syndrome is unknown, though attrition or immune-mediated destruction of collateral muscular innervation appears likely. The pathologic findings include scattered individual muscle fiber atrophy as well as evidence of chronic denervation and reinnervation. This form of motor deterioration is not life-threatening, but potentially may cause increasing disability in a large number of polio survivors. Proper supportive care of facial, laryngeal, and pharyngeal weakness is crucial in the management of these patients.

Biopsy↗

[Postpoliomyelitis syndrome and progressive postpoliomyelitis muscular atrophy].

New complaints of patients after poliomyelitis following after decades of a stabilized condition are described by the term postpoliomyelitic syndrome (PPS). They affect at least half the patients after poliomyelitis. The best defined picture from a broad spectrum of new complaints is so-called progressive postpoliomyelitic atrophy (PPMA). The authors review contemporary knowledge of the etiopathogenesis of PPS and PPMA and their possible therapy.

Humans↗

Postpoliomyelitis syndrome problems of knee function: a review.

Postpoliomyelitis syndrome describes a ¿new¿ weakness, unrelated to any other neurological or medical disorder. It appears 10 to 15 years or more after recovery from the initial paralysis. This new weakness interferes with activities of daily living. The management of postpoliomyelitis syndrome may include orthotics, special exercises, a change in their lifestyle, or a combination of these regimens. This article is especially concerned with knee joint stabilization in various deformities that are weakened by the postpoliomyelitis syndrome.

Age of Onset↗

Excessive muscular fatigue in the postpoliomyelitis syndrome.

To investigate anterior tibial muscle fatigability and metabolism in postpoliomyelitis syndrome patients and controls, we performed measurements of force and relaxation time, as well as 31P magnetic resonance spectroscopy, during intermittent, low-intensity, isometric, voluntary exercise. Both maximum voluntary contraction and tetanic force declined significantly more during exercise and subsequently recovered less in patients compared with controls, indicating greater fatigue in patients. However, intracellular pH and phosphocreatine were not different in the two groups at rest or during exercise or recovery, suggesting that the greater fatigue of the patients was not due to an excessive change of metabolites. Moreover, the pre-exercise half-relaxation time of the tetanus was significantly prolonged in patients compared with controls, and the decline in tetanic force during exercise was linearly related to the half-relaxation time of tetanus, suggesting impaired calcium kinetics. Taken together, the findings of increased fatigability, delayed recovery, and prolonged half-relaxation time, without differences in metabolites, suggest that the fatigue in postpoliomyelitis syndrome may be due to impaired activation beyond the muscle membrane.

Action Potentials↗

A comparison of fatigue scales in postpoliomyelitis syndrome.

OBJECTIVE: To examine the applicability and validity of traditional fatigue questionnaires in postpoliomyelitis syndrome (PPS) patients with disabling fatigue. DESIGN: Cross-sectional study. PPS and disabling fatigue were ascertained according to published criteria. Descriptiveness was determined using the McNemar test, and interscale z-score agreement was estimated with Pearson's coefficients. SETTING: PPS clinic. PARTICIPANTS: Fifty-six survivors of poliomyelitis: 39 met criteria for PPS, 25 of whom met criteria for disabling fatigue. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: The Fatigue Severity Scale (FSS), visual analog scale (VAS) for fatigue, and Fatigue Impact Scale (FIS). RESULTS: Twenty-four patients scored 50% or higher on the scale range for FSS, compared with 19 patients for VAS for fatigue (P=.042), and 7 patients for FIS (P<.001). Scores for patients with disabling fatigue averaged 81.5%, 62%, and 40.9% of the scale range for FSS, VAS for fatigue, and FIS, respectively. Agreement was moderate between the FSS and VAS for fatigue (r=.45, P=.02), but low between FSS and FIS (r=.29, P=.15), and FIS and VAS for fatigue (r=.20, P=.33). Two sample t tests showed significant differences between those with disabling fatigue and those without, based on FSS scores (t=3.8, P<.001), but not for VAS for fatigue or FIS scores. CONCLUSIONS: FSS was the most descriptive of the instruments tested. Scores generated by the scales were not interchangeable. Of the 3 scales, FFS seemed to be the most informative for the clinical assessment of fatigue in patients with PPS.

Adult↗

[A trial of cronassial use in late postpoliomyelitis syndrome].

The results are presented of treatment with gangliosides (Cronassial) in 5 patients with late postpoliomyelitis syndrome. Clinical and electrophysiological examination were done before and after three months of treatment. Only subjective improvement was found. A year-long follow-up failed to fluid progression of the signs and symptoms that, formerly, were progressing slowly. It seems that Cronassial administration (in larger doses and for a longer time) is justified in patients with peripheral motor neuron lesion.

Adult↗

Predictive factors and correlates for pain in postpoliomyelitis syndrome patients.

OBJECTIVE: To identify predictive and associated factors for muscle and joint pain in postpoliomyelitis syndrome (PPS). DESIGN: Cross-sectional study design. SETTING: Postpolio clinics. PARTICIPANTS: Baseline data on 126 PPS patients entered into a multicentered clinical trial. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: Adjusted odds ratios were computed by using logistic regression modeling. Patients with or without muscle or joint pain were compared with regard to predictors and associated factors. RESULTS: In multivariate analyses for muscle pain, significant predictive and associated factors were female gender (P=.0006), longer duration of general fatigue (P=.019), and a lower score on the general health scale (P=.009) of the Medical Outcomes Study 36-Item Short-From Health Survey (SF-36). In multivariate analyses for joint pain, significant predictive and associated factors were female gender (P<.003), longer latency (duration of stability after polio; P=.008), younger age at interview (P<.002), greater weakness at acute polio (P<.07), weaker lower-extremity muscle strength (P<.04), and a lower SF-36 general health scale score (P<.02). CONCLUSIONS: Women are more likely to report muscle and joint pain in PPS. Greater initial motor unit involvement and lower-extremity weakness may be additional important factors for determining joint pain. Both muscle and joint pain are associated with reductions in quality of life.

Aged↗

A comparison of 4 questionnaires to measure fatigue in postpoliomyelitis syndrome.

OBJECTIVE: To assess the comparability and reproducibility of 4 questionnaires used to measure fatigue in postpoliomyelitis syndrome (PPS). DESIGN: Repeated-measures at a 3-week interval. SETTING: University hospital. PARTICIPANTS: Convenience sample of 65 patients with PPS. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: The Fatigue Severity Scale (FSS), the Nottingham Health Profile (NHP) energy category, the Polio Problem List (PPL) fatigue item, and the Dutch Short Fatigue Questionnaire (SFQ). RESULTS: Correlations of scores between questionnaires were all significant (P<.01) and ranged from.43 (between the NHP energy category and the PPL fatigue item) to.68 (between the PPL fatigue item and the SFQ). Scores on the second visit, normalized to a 0 to 100 scale, were: FSS, 78+/-15; NHP energy category, 47+/-35; PPL fatigue item, 81+/-17; and SFQ, 65+/-22. Except for the difference between the FSS and the PPL fatigue item, the differences in scores between the questionnaires were significant (P<.01). Scale analysis indicated that all questionnaires measured the same unidimensional construct. The reproducibility of the FSS, the PPL fatigue item, and the SFQ was moderate. The smallest detectable change was 1.5 points for the FSS, 2.0 points for the PPL fatigue item, and 1.9 points for the SFQ. CONCLUSIONS: Although the questionnaires measure the same fatigue construct in PPS, the results are not interchangeable because the ranges of measurement differ. The NHP energy category, in particular, appeared to have a high detection threshold. The moderate reproducibility of the questionnaires indicates a lack of precision, especially when applied at the individual patient level.

Adult↗

Energy demands of walking in persons with postpoliomyelitis syndrome: relationship with muscle strength and reproducibility.

OBJECTIVES: To describe the energy demands of walking in subjects with postpoliomyelitis syndrome (PPS) in comparison with the demands in healthy subjects, and to assess the reproducibility of walking energy measurements. DESIGN: Four repeated measurements with a 1-week interval between each measurement. SETTING: Outpatient clinic of a university hospital. PARTICIPANTS: Fourteen subjects with PPS and 14 age- and sex-matched healthy subjects. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: Walking speed and energy cost of walking. The correlation parameter was lower-extremity muscle strength sum (MSS). The reproducibility parameters were standard error (SE) of measurement and smallest detectable difference (SDD). RESULTS: Walking speed in subjects with PPS (61.8 m/min) was significantly lower (-28%) and energy cost (4.8 J.kg(-1).m(-1)) was significantly higher (40%) than in healthy subjects. MSS correlated strongly with energy cost (r=-.84, P=.000), explaining 71% of the variance. The SE of measurement of energy cost measurements ranged between 1.7% and 3.4% for PPS subjects and between 1.2% and 2.4% for healthy subjects. The SDD ranged between 4.6% and 9.4% for PPS subjects and between 3.3% and 6.6% for healthy subjects, depending on the number of repeated measurements that were considered. CONCLUSIONS: Energy cost of walking in subjects with PPS is strongly related to the extent of muscle weakness in the lower extremities. Although variability was greater for PPS subjects than for healthy subjects, reproducibility of energy cost measurements was high. Therefore, metabolic assessment of energy cost of walking is a sensitive tool that can reveal clinically relevant changes even in the condition of a person with PPS.

Adult↗

Reproducibility of maximal quadriceps strength and its relationship to maximal voluntary activation in postpoliomyelitis syndrome.

OBJECTIVES: To determine what changes in maximal isometric strength can be detected in a symptomatic quadriceps muscle in patients with postpoliomyelitis syndrome (PPS) and to investigate the association between the variability in maximal strength and maximal voluntary activation (MVA). DESIGN: Repeated-measures over a 3-week interval. SETTING: University hospital. PATIENTS: Convenience sample of 65 patients with PPS. INTERVENTION: Dynamometer testing. MAIN OUTCOME MEASURES: Maximal voluntary contraction (MVC) torque of the quadriceps was measured with a Kin-Com dynamometer and MVA was determined by twitch interpolation. RESULTS: The mean difference between the 2 consecutive measurements was -0.7+/-12.8 Nm (95% confidence interval [CI], -3.9 to 2.5). The test-retest reliability was excellent for MVC torque (intraclass correlation coefficient [ICC]=.96; 95% CI,.93-.98) and moderate for MVA (ICC=.73; 95% CI,.56-.85). The smallest detectable change in MVC torque was 25% for an individual. The variability in MVA explained 18% of the variability in maximal strength. CONCLUSIONS: Variability in maximal quadriceps strength, measured with a fixed dynamometer, was large and partly related to variability in MVA. This implies that even with optimally standardized strength testing, a follow-up of many years is required to objectify progression of quadriceps weakness in an individual patient with PPS. To demonstrate changes in strength in groups of patients in follow-up or intervention studies, feasible sample sizes are required.

Adult↗

Reproducibility of walking at self-preferred and maximal speed in patients with postpoliomyelitis syndrome.

OBJECTIVE: To assess the reproducibility of walking performance, heart rate, and perceived exertion at self-preferred speed and maximal walking speed in patients with the postpoliomyelitis syndrome (PPS). DESIGN: Repeated measurement at a 3-week interval. SETTING: University hospital. PARTICIPANTS: Convenience sample of 65 patients with PPS. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: Walking performance: the distance walked in 2 minutes at a self-preferred speed and the time needed to walk 75 m at maximal speed, heart rate, and rating of perceived exertion (RPE) on an 11-point scale. RESULTS: Test-retest reliability of walking performance was excellent for both tests (intraclass correlation coefficient [ICC] range, .94-.97). No systematic differences existed between test and retest. The smallest detectable change for an individual was 15% for both tests. Test-retest reliability for heart rate was good (ICC=.86) but moderate for RPE (Spearman rho range, .67-.70). The smallest detectable change for RPE was between 4 and 6 scale points. The variability in walking performance was significantly correlated with the variability in heart rate at self-preferred speed (r =.36, P <.01) but not with the variability in RPE (r =.20, P =.11). CONCLUSIONS: Both walking tests showed good reproducibility and may be appropriate to monitor (individual) changes in walking capacity in patients with PPS. Because of its moderate reproducibility, RPE does not seem to be suitable to monitor physical exertion. The usefulness of an objective measure such as heart rate for this purpose needs further investigation.

Female↗

Perceived health and physical functioning in postpoliomyelitis syndrome: a 6-year prospective follow-up study.

OBJECTIVES: To study prospectively the health status, and especially the physical functioning, of polio survivors with and without postpoliomyelitis syndrome (PPS), and to identify prognostic determinants of change in physical functioning. DESIGN: Prospective cohort study; measurements at baseline and after 1, 2, and 6 years. SETTING: University hospital in the Netherlands. PARTICIPANTS: Seventy-six subjects with PPS and 27 without PPS. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURE: The Nottingham Health Profile (NHP) physical mobility category. RESULTS: Subjects with PPS had significantly poorer health status than subjects without PPS. No significant differences in mean NHP physical mobility scores between baseline and 6 years were found; both groups had improved after 1 year, after which there was a slow decline over the next 5 years. During the first 2 years, strength measurements showed little decline that was not related to changes in NHP physical mobility score. A physical performance test revealed no mean change in the first 2 years, but the subgroup with a decline above the 75th percentile eventually deteriorated 10.5+/-16.3 points on the NHP physical mobility category (P=.01) at 6 years from baseline. This subgroup had more extensive paresis than the other subjects, although it was not significant (P=.07). The extent of paresis at baseline was the only prognostic determinant for an increase in NHP physical mobility problems after 6 years. CONCLUSIONS: Subjects with and without PPS did not differ with regard to changes in health status in a 6-year period. The fact that the extent of paresis was a prognostic factor for a decline in physical functioning is in accord with a (slow) decline in muscle mass, as a late effect of polio, that may lead to a decline in physical functioning as the reduced muscle capacity becomes less able to meet the demands of daily physical activities.

Activities of Daily Living↗

Neurologically normal patients with suspected postpoliomyelitis syndrome: electromyographic assessment of past denervation.

Patients with neurologic signs consistent with previous poliomyelitis have been shown to have electrodiagnostic evidence of widespread denervation, often in clinically normal muscles thought to have been uninvolved by poliomyelitis. Eight percent of patients seen in our postpoliomyelitis clinic had no abnormalities on neurologic examination or with conventional electrodiagnostic studies to corroborate history of poliomyelitis. Seventeen patients were studied, subtle electrodiagnostic evidence of denervation was sought. Measurements of fiber density and analysis of turns and amplitude showed no abnormalities in nine patients. Five patients had borderline elevated fiber density. Analysis of turns and amplitude showed mild neurogenic changes in two patients and borderline changes in three patients. These findings were disparate, with isolated abnormalities in individual muscles. It was concluded that (1) there is a group of patients whose histories of poliomyelitis are uncertain, and (2) the lack of clear evidence for previous denervation after extensive electrodiagnostic testing is a valid means for excluding the diagnosis of postpoliomyelitis syndrome.

Adult↗

Motor unit characteristics in healthy subjects and those with postpoliomyelitis syndrome: a high-density surface EMG study.

The purpose of this study was to identify optimal ways to detect neurogenic changes with high-density surface electromyography (HD-sEMG). For this purpose, we searched for the variables that most clearly discriminated between postpoliomyelitis and healthy subjects. We obtained HD-sEMG from the quadriceps muscle at different force levels in nine subjects with postpoliomyelitis syndrome and in matched healthy controls. Single motor unit action potentials (MUAPs), extracted from the HD-sEMG signal and the raw signal itself, were analyzed. Areas under the curve of the extracted MUAP waveform, indicating motor unit size, perfectly separated both groups. Raw signal analysis showed significant differences between groups for the monopolarly recorded amplitude up to 60% of maximal force and for the level of interference at higher force levels (40-100% force). We conclude that with HD-sEMG it is possible to detect neurogenic motor unit changes noninvasively, both by analysis of the raw signal itself and by analysis of extracted single MUAPs. The diagnostic yield of the single MUAP analysis is clearly higher. These findings point toward applications for clinical practice and invite further studies exploring the diagnostic value of HD-sEMG.

Action Potentials↗

[Post-polyiomyelitis syndrome].

Postpoliomyelitis syndrome is a clinical syndrome characterized by late progression of symptoms, neuromuscular weakness, fatigue and pain, several (more than 20) Years after acute anterior poliomyelitis. In the United States, where it has been mainly described, frequency is estimated between 20 and 30p.100 in patients with sequelae of poliomyelitis. Although the cause is still unknown, postpoliomyelitis syndrome is likely due to degeneration and dysfunction of terminal axons of enlarged post-polio units, with a possible role of inflammatory reaction driven by persistence of the polio virus. Due to lack of specific therapy, rational therapeutic approaches are symptomatic, including exercise, reassurance and life-strategies for fatigue.

Cerebrospinal Fluid↗