RARE DISEASE
Postpoliomyelitis syndrome
Postpoliomyelitis syndrome
Postpoliomyelitis syndrome
Synonyms: Postpolio sequelae, Postpolio syndrome, Postpoliomyelitic syndrome, Postpoliomyelitis sequelae
Synonyms: Postpolio sequelae, Postpolio syndrome, Postpoliomyelitic syndrome, Postpoliomyelitis sequelae
Synonyms: Postpolio sequelae, Postpolio syndrome, Postpoliomyelitic syndrome, Postpoliomyelitis sequelae
Drug discovery
2
drugs
With orphan designations
Overview
Postpoliomyelitis Syndrome (PPS) is a late-onset neuromuscular disorder affecting polio survivors, characterized by new muscle weakness, atrophy, fatigue, pain, and functional decline ≥15 years after acute poliomyelitis. Pathophysiology involves chronic motor neuron stress from compensatory reinnervation and aging. Diagnosis requires exclusion of alternative neuromuscular, orthopedic, or systemic causes [1][4][6][9][16].
Burden
Progressive mobility loss, respiratory dysfunction (27-36% develop failure), and chronic pain impair quality of life. Increased fall risk, social isolation, and cognitive deficits (e.g., memory issues) contribute to disability [4][6][9].
Lifetime healthcare costs escalate due to assistive needs and recurrent complications [14][16].
Therapies
Multidisciplinary care: Energy conservation, pacing, low-impact aerobic/strengthening exercises, and assistive devices [3][8][16].
Symptomatic management: NSAIDs, antiepileptics (e.g., lamotrigine), IV immunoglobulin (limited evidence), and respiratory support for sleep apnea/ventilatory insufficiency [6][8][13].
Rehabilitative focus: Aquatic therapy, non-fatiguing regimens, and fall prevention [3][8][16].
Categories: rare neurological diseases
Research Papers
273 drug discovery papers about Postpoliomyelitis syndrome, with 1 first-in-class emerging drug candidates forecasted to outperform the average preclinical success rate. Recent publications:
273 drug discovery papers about Postpoliomyelitis syndrome, with 1 first-in-class emerging drug candidates forecasted to outperform the average preclinical success rate. Recent publications:
categories:
Small molecules
small molecules
2026-04-01 | Lumbar Medial Branch Minimally Invasive Pain and Spine Intervention for Low Back Pain in Postpolio Syndrome
Postpolio syndrome (PPS) involves a constellation of symptoms which may include back pain, slowly progressive muscle limbs paresis with muscle atrophy, joints pain, and/or paraesthesia, observed in polio survivors years after poliovirus infection. A 70-year-old male, a survivor of childhood poliomyelitis, presented with severe low back pain of gradual onset. After a thorough evaluation, the pain was identified as facetogenic in origin, and a diagnostic cum therapeutic medial branch minimally invasive pain and spine intervention was performed from T12-L, providing significant relief. Cooled radiofrequency ablation of the medial branch was performed as a definitive treatment later. This case highlights the intersection of PPS and facet joint pathology and demonstrates the effectiveness of medial branch blocks in managing facet joint-related low back pain in such patients.
2026-02-18 | Determining the presence of restless legs syndrome in patients with poliomyelitis sequelae and its effect on pain, fatigue, sleep and quality of life.
Investigation of Restless Legs Syndrome (RLS) in patients with poliomyelitis sequelae (PS) and the effect of RLS on pain, fatigue, sleep and quality of life (QoL). We investigated the presence of RLS in patients with PS who consecutively applied to the neurology outpatient clinic and did not have secondary causes of RLS. Gender, age, side affected by PS, leg muscle strength and length, International RLS Study Group criteria, Pittsburgh Sleep Quality Index questionnaire, Fatigue Severity Scale, SF-36 Form scores were recorded. After receiving a diagnosis of RLS, patients completed the RLS Severity Rating Scale (RLS-SWS) questionnaire. We identified RLS in 12 (37.5%) of the 32 patients included in the study. There were no differences in demographic characteristics. The leg lengths in the patients with RLS (PwRLS) were significantly shorter (p < 0.001). PwRLS had shorter sleep duration, longer latency, and worse sleep quality and fatigue severity scores (p < 0.001). The pain score was found to be statistically significantly higher in the PwRLS group (p = 0.007). There were no differences in other QoL areas. In the PwRLS study, the RLS-SWS score was positively correlated with sleep latency, sleep quality, and pain and fatigue scores. It was also negatively correlated with sleep duration (p < 0.001). These findings demonstrate that RLS, which has an effective treatment, is a prevalent condition among PS patients that worsens sleep quality and increases pain. Clinicians should screen PS patients who complain of leg pain and/or sleep disturbances for RLS and provide appropriate treatment.
2025-09-06 | Low-grade persistent poliovirus infection in long-term polio survivors diagnosed with post-polio syndrome: diagnostic and clinical implications.
Despite extensive research, the pathogenesis of Post-Polio Syndrome (PPS) remains unclear. We investigated 251 participants from Northern Italy: long-term polio survivors with PPS, long-term polio survivors with stable polio, family members of both groups, subjects with neurological disorders other than poliomyelitis, and healthy controls. This study investigated whether persistent viral activity or the existence of viral reservoirs contributes to causing PPS. Poliovirus (PV) genomes and proteins were detected in 87.2% of PPS cases versus 12.0% of stable polio cases and 3.5% of control family members, but not in pathologic and healthy controls. Among PPS patients, the highly concordant detection of PV strains in both peripheral blood leukocytes and cerebrospinal fluid (CSF) suggests the presence of an ongoing low-grade infection. Conversely, the very low detection rate in family members indicates the minimal transmissibility of these PV variants. Molecular analysis of the detected PV strains revealed mutations across most genome regions, likely leading to defects in virus replication. Furthermore, in cell cultures, PPS-derived PV strains induced the release of inflammatory mediators (IL6, IL8, MCP1) that may play a pathogenic role. These findings have several clinical implications. First, the presence of mutated PV forms in blood leukocytes and CSF could serve as a diagnostic marker for PPS. Second, the persistent virus infection suggests that antiviral treatments might help reduce PPS progression. Furthermore, advanced genome sequencing techniques hold potential for distinguishing vaccine-derived from wild-type PV strains, thereby refining our understanding of PPS and the full spectrum of polio disorders.
2024-06-22 | Prevalence and Severity of Central Sensitization in Post-Polio Syndrome: Associations with Clinical Measures and Quality of Life.
To investigate the presence and severity of central sensitization (CS) and its associations with clinical measures and quality of life (QoL) in individuals with a history of paralytic poliomyelitis with and without post-polio syndrome (PPS). In this cross-sectional study, we included 98 individuals with a history of poliomyelitis, in whom 82 (83.6%) met the criteria of PPS. We used CS Inventory (CSI) to evaluate the presence and severity of CS. We evaluated the severity of fatigue, pain, polio-related impairments, and QoL using a Numerical Rating Scale in addition to Fatigue Severity Scale, Self-reported Impairments in Persons with late effects of Polio rating scale (SIPP), and Nottingham Health Profile (NHP). CS was present in 52.4% of patients with PPS, of which 63% are classified as severe to extreme. Those with CS reported more severe symptoms, more polio-related impairments, and worse QoL than those without CS. Severity of CS showed significant positive correlations with severity of fatigue, pain, SIPP, and NHP scales in those with PPS. CSI did not indicate CS in any of those without PPS. CS was present in more than half of the individuals with PPS and correlated with more severe pain, fatigue, and more polio-related impairments, in addition to poorer QoL. These findings suggest that CS may contribute to the clinical picture in a subgroup of individuals with PPS. Thus, identification and appropriate management of CS patients may potentially help alleviate their symptoms and improve their QoL.
2023-11-15 | The Effects of Anthroposophic Medicine in Chronic Pain Conditions: A Systematic Review.
Background: The currently available evidence is unclear in regard to pain-related outcomes of patients with chronic pain conditions who undergo treatment with anthroposophic medicine (AM). Aim: To identify and synthesize the evidence in patients with chronic pain before and after AM therapy. Methods: The following databases and search interfaces were searched on October 21, 2021: Embase (via Embase.com), Medline (via PubMed), and the Cochrane Library. Additional references were identified via bibliographies of included studies. In at least one experimental arm that used anthroposophic therapy to treat chronic pain, AM treatments were required to be documented. Included studies reported on pain severity and physical and emotional functioning. Two authors independently assessed the studies for inclusion criteria, extracted the data, and conducted the quality evaluation of the included studies based on the critical appraisal tools provided by the Joanna Briggs Institute. Results: Seven studies (eight publications) were included in the review, of which were three randomized controlled trials (RCTs), two non-RCTs, and two pretest-post-test studies. A total number of 600 patients participated in the identified experimental studies, of whom all were adults. Three studies included patients with low back pain, one study each assessed patients with fibromyalgia, migraine, dysmenorrhea, and postpolio syndrome, respectively. The identified clinical studies reported considerable reductions in symptoms and effect sizes of pain outcomes after AM therapies being predominantly large, with no notable adverse effects. Conclusion: The findings of this systematic review of studies assessing AM therapies in patients with chronic pain problems revealed that there is a scarcity of evidence currently available, with unclear effects of AM treatments in reducing pain intensity and improving quality of life in the evaluated health conditions. Although most of the studies revealed a favorable benefit on one or more pain-related outcomes, the variability of the research did not allow for generalization across different studies, health conditions, and populations.
cell therapies
2024-02-19 | The Role of Epigenetics in Accelerated Aging: A Reconsideration of Later-Life Visual Loss After Early Optic Neuropathy.
In 2005, we reported 3 patients with bilateral optic nerve damage early in life. These patients had stable vision for decades but then experienced significant bilateral vision loss with no obvious cause. Our hypothesis, novel at that time, was that the late decline of vision was due to age-related attrition of retinal ganglion cells superimposed on a reduced neuronal population due to the earlier injury. The field of epigenetics provides a new paradigm with which to consider the normal aging process and the impact of neuronal injury, which has been shown to accelerate aging. Late-in-life decline in function after early neuronal injury occurs in multiple sclerosis due to dysregulated inflammation and postpolio syndrome. Recent studies by our group in mice have also demonstrated the possibility of partial reversal of cellular aging and the potential to mitigate anatomical damage after injury and even improve visual function. The results in mice and nonhuman primates published elsewhere have shown enhanced neuronal survival and visual function after partial epigenetic reprogramming. Injury promotes epigenetic aging , and this finding can be observed in several clinically relevant scenarios. An understanding of the epigenetic mechanisms at play opens the opportunity to restore function in the nervous system and elsewhere with cellular rejuvenation therapies. Our earlier cases exemplify how reconsideration of previously established concepts can motivate inquiry of new paradigms.
2016-07-01 | Cell-based neurorestorative therapy for postpoliomyelitis syndrome: a case report
Abstract: Postpoliomyelitis syndrome refers to the new neuromuscular symptoms that occur in patients, years after their acute poliomyelitis has stabilized. A 64-year-old Danish man presented with lower limb weakness and severe pain for 2 years and 5 months. He had a history of poliomyelitis affecting his limbs 59 years ago. Physical examination revealed atrophy of muscles of both lower limbs. Electromyography revealed that recruitment of maximal voluntary contraction of muscles was decreased in the affected muscles. He received cell-based neurorestorative therapy during admission, and then, his neurological function improved and remained stable during 4-year follow-up. This case report shows that cell therapy could be a treatment option for postpoliomyelitis syndrome. Keywords: postpoliomyelitis syndrome, neuromuscular disease, cell transplantation, cell-based neurorestorative therapy
2005-01-01 | Managing chronic pain with encapsulated cell implants releasing catecholamines and endogenous opiods
Spinal injections (intrathecal) of norepinephrine and/or opiod agonists are antinociceptive and when administered together may act in synergy. Spinal implants of adrenal chromaffin cells are an effective method for sustained delivery of the analgesic substances norepinephrine and enkephalin to the central nervous system (CNS). One method of packaging and implanting cell-loaded devices into the intrathecal space of recipients is by encapsulating the cell suspensions in a polymer membrane prior to implantation. Cells/tissue packaged within an encapsulating membrane obviate the need for immunosuppressive therapies in transplant recipients. In addition, device output can be quantified prior to implantation, and following the removal of the spinal implant. The ability to retrieve the devices with the present tubular configuration also confers an additional margin of safety over unencapsulated chromaffin cell implants. This paper reviews the research and clinical observations of cellular transplants containing adrenal chromaffin cells for relieving chronic pain. Encapsulated cell technology is discussed with an emphasis on our experiences developing pain-modulating clinical devices. The human-sized prototype devices were loaded with enzymatically isolated bovine chromaffin cells and maintained in vitro for 7 - 8 days in serum-free media. Two days prior to implantation, each device was assayed by static incubation to measure catecholamine and met-enkephalin output, and qualified devices (n = 6) were implanted into the sheep subarachnoid space for 6 weeks. Following a 6 week in life period, the retrieval forces of prototype devices were measured during removal from the subarachnoid space. Static incubation of the devices immediately following retrieval and after a 24 hour re-incubation period were used to quantify norepinephrine and met-enkephalin secretion profiles. This study demonstrated the safety, retrievability and maintenance of pharmacologically active encapsulated chromaffin cell-loaded devices with human implant dimensions.
small molecules
2026-04-01 | Lumbar Medial Branch Minimally Invasive Pain and Spine Intervention for Low Back Pain in Postpolio Syndrome
Postpolio syndrome (PPS) involves a constellation of symptoms which may include back pain, slowly progressive muscle limbs paresis with muscle atrophy, joints pain, and/or paraesthesia, observed in polio survivors years after poliovirus infection. A 70-year-old male, a survivor of childhood poliomyelitis, presented with severe low back pain of gradual onset. After a thorough evaluation, the pain was identified as facetogenic in origin, and a diagnostic cum therapeutic medial branch minimally invasive pain and spine intervention was performed from T12-L, providing significant relief. Cooled radiofrequency ablation of the medial branch was performed as a definitive treatment later. This case highlights the intersection of PPS and facet joint pathology and demonstrates the effectiveness of medial branch blocks in managing facet joint-related low back pain in such patients.
2026-02-18 | Determining the presence of restless legs syndrome in patients with poliomyelitis sequelae and its effect on pain, fatigue, sleep and quality of life.
Investigation of Restless Legs Syndrome (RLS) in patients with poliomyelitis sequelae (PS) and the effect of RLS on pain, fatigue, sleep and quality of life (QoL). We investigated the presence of RLS in patients with PS who consecutively applied to the neurology outpatient clinic and did not have secondary causes of RLS. Gender, age, side affected by PS, leg muscle strength and length, International RLS Study Group criteria, Pittsburgh Sleep Quality Index questionnaire, Fatigue Severity Scale, SF-36 Form scores were recorded. After receiving a diagnosis of RLS, patients completed the RLS Severity Rating Scale (RLS-SWS) questionnaire. We identified RLS in 12 (37.5%) of the 32 patients included in the study. There were no differences in demographic characteristics. The leg lengths in the patients with RLS (PwRLS) were significantly shorter (p < 0.001). PwRLS had shorter sleep duration, longer latency, and worse sleep quality and fatigue severity scores (p < 0.001). The pain score was found to be statistically significantly higher in the PwRLS group (p = 0.007). There were no differences in other QoL areas. In the PwRLS study, the RLS-SWS score was positively correlated with sleep latency, sleep quality, and pain and fatigue scores. It was also negatively correlated with sleep duration (p < 0.001). These findings demonstrate that RLS, which has an effective treatment, is a prevalent condition among PS patients that worsens sleep quality and increases pain. Clinicians should screen PS patients who complain of leg pain and/or sleep disturbances for RLS and provide appropriate treatment.
2025-09-06 | Low-grade persistent poliovirus infection in long-term polio survivors diagnosed with post-polio syndrome: diagnostic and clinical implications.
Despite extensive research, the pathogenesis of Post-Polio Syndrome (PPS) remains unclear. We investigated 251 participants from Northern Italy: long-term polio survivors with PPS, long-term polio survivors with stable polio, family members of both groups, subjects with neurological disorders other than poliomyelitis, and healthy controls. This study investigated whether persistent viral activity or the existence of viral reservoirs contributes to causing PPS. Poliovirus (PV) genomes and proteins were detected in 87.2% of PPS cases versus 12.0% of stable polio cases and 3.5% of control family members, but not in pathologic and healthy controls. Among PPS patients, the highly concordant detection of PV strains in both peripheral blood leukocytes and cerebrospinal fluid (CSF) suggests the presence of an ongoing low-grade infection. Conversely, the very low detection rate in family members indicates the minimal transmissibility of these PV variants. Molecular analysis of the detected PV strains revealed mutations across most genome regions, likely leading to defects in virus replication. Furthermore, in cell cultures, PPS-derived PV strains induced the release of inflammatory mediators (IL6, IL8, MCP1) that may play a pathogenic role. These findings have several clinical implications. First, the presence of mutated PV forms in blood leukocytes and CSF could serve as a diagnostic marker for PPS. Second, the persistent virus infection suggests that antiviral treatments might help reduce PPS progression. Furthermore, advanced genome sequencing techniques hold potential for distinguishing vaccine-derived from wild-type PV strains, thereby refining our understanding of PPS and the full spectrum of polio disorders.
2024-06-22 | Prevalence and Severity of Central Sensitization in Post-Polio Syndrome: Associations with Clinical Measures and Quality of Life.
To investigate the presence and severity of central sensitization (CS) and its associations with clinical measures and quality of life (QoL) in individuals with a history of paralytic poliomyelitis with and without post-polio syndrome (PPS). In this cross-sectional study, we included 98 individuals with a history of poliomyelitis, in whom 82 (83.6%) met the criteria of PPS. We used CS Inventory (CSI) to evaluate the presence and severity of CS. We evaluated the severity of fatigue, pain, polio-related impairments, and QoL using a Numerical Rating Scale in addition to Fatigue Severity Scale, Self-reported Impairments in Persons with late effects of Polio rating scale (SIPP), and Nottingham Health Profile (NHP). CS was present in 52.4% of patients with PPS, of which 63% are classified as severe to extreme. Those with CS reported more severe symptoms, more polio-related impairments, and worse QoL than those without CS. Severity of CS showed significant positive correlations with severity of fatigue, pain, SIPP, and NHP scales in those with PPS. CSI did not indicate CS in any of those without PPS. CS was present in more than half of the individuals with PPS and correlated with more severe pain, fatigue, and more polio-related impairments, in addition to poorer QoL. These findings suggest that CS may contribute to the clinical picture in a subgroup of individuals with PPS. Thus, identification and appropriate management of CS patients may potentially help alleviate their symptoms and improve their QoL.
2023-11-15 | The Effects of Anthroposophic Medicine in Chronic Pain Conditions: A Systematic Review.
Background: The currently available evidence is unclear in regard to pain-related outcomes of patients with chronic pain conditions who undergo treatment with anthroposophic medicine (AM). Aim: To identify and synthesize the evidence in patients with chronic pain before and after AM therapy. Methods: The following databases and search interfaces were searched on October 21, 2021: Embase (via Embase.com), Medline (via PubMed), and the Cochrane Library. Additional references were identified via bibliographies of included studies. In at least one experimental arm that used anthroposophic therapy to treat chronic pain, AM treatments were required to be documented. Included studies reported on pain severity and physical and emotional functioning. Two authors independently assessed the studies for inclusion criteria, extracted the data, and conducted the quality evaluation of the included studies based on the critical appraisal tools provided by the Joanna Briggs Institute. Results: Seven studies (eight publications) were included in the review, of which were three randomized controlled trials (RCTs), two non-RCTs, and two pretest-post-test studies. A total number of 600 patients participated in the identified experimental studies, of whom all were adults. Three studies included patients with low back pain, one study each assessed patients with fibromyalgia, migraine, dysmenorrhea, and postpolio syndrome, respectively. The identified clinical studies reported considerable reductions in symptoms and effect sizes of pain outcomes after AM therapies being predominantly large, with no notable adverse effects. Conclusion: The findings of this systematic review of studies assessing AM therapies in patients with chronic pain problems revealed that there is a scarcity of evidence currently available, with unclear effects of AM treatments in reducing pain intensity and improving quality of life in the evaluated health conditions. Although most of the studies revealed a favorable benefit on one or more pain-related outcomes, the variability of the research did not allow for generalization across different studies, health conditions, and populations.
cell therapies
2024-02-19 | The Role of Epigenetics in Accelerated Aging: A Reconsideration of Later-Life Visual Loss After Early Optic Neuropathy.
In 2005, we reported 3 patients with bilateral optic nerve damage early in life. These patients had stable vision for decades but then experienced significant bilateral vision loss with no obvious cause. Our hypothesis, novel at that time, was that the late decline of vision was due to age-related attrition of retinal ganglion cells superimposed on a reduced neuronal population due to the earlier injury. The field of epigenetics provides a new paradigm with which to consider the normal aging process and the impact of neuronal injury, which has been shown to accelerate aging. Late-in-life decline in function after early neuronal injury occurs in multiple sclerosis due to dysregulated inflammation and postpolio syndrome. Recent studies by our group in mice have also demonstrated the possibility of partial reversal of cellular aging and the potential to mitigate anatomical damage after injury and even improve visual function. The results in mice and nonhuman primates published elsewhere have shown enhanced neuronal survival and visual function after partial epigenetic reprogramming. Injury promotes epigenetic aging , and this finding can be observed in several clinically relevant scenarios. An understanding of the epigenetic mechanisms at play opens the opportunity to restore function in the nervous system and elsewhere with cellular rejuvenation therapies. Our earlier cases exemplify how reconsideration of previously established concepts can motivate inquiry of new paradigms.
2016-07-01 | Cell-based neurorestorative therapy for postpoliomyelitis syndrome: a case report
Abstract: Postpoliomyelitis syndrome refers to the new neuromuscular symptoms that occur in patients, years after their acute poliomyelitis has stabilized. A 64-year-old Danish man presented with lower limb weakness and severe pain for 2 years and 5 months. He had a history of poliomyelitis affecting his limbs 59 years ago. Physical examination revealed atrophy of muscles of both lower limbs. Electromyography revealed that recruitment of maximal voluntary contraction of muscles was decreased in the affected muscles. He received cell-based neurorestorative therapy during admission, and then, his neurological function improved and remained stable during 4-year follow-up. This case report shows that cell therapy could be a treatment option for postpoliomyelitis syndrome. Keywords: postpoliomyelitis syndrome, neuromuscular disease, cell transplantation, cell-based neurorestorative therapy
2005-01-01 | Managing chronic pain with encapsulated cell implants releasing catecholamines and endogenous opiods
Spinal injections (intrathecal) of norepinephrine and/or opiod agonists are antinociceptive and when administered together may act in synergy. Spinal implants of adrenal chromaffin cells are an effective method for sustained delivery of the analgesic substances norepinephrine and enkephalin to the central nervous system (CNS). One method of packaging and implanting cell-loaded devices into the intrathecal space of recipients is by encapsulating the cell suspensions in a polymer membrane prior to implantation. Cells/tissue packaged within an encapsulating membrane obviate the need for immunosuppressive therapies in transplant recipients. In addition, device output can be quantified prior to implantation, and following the removal of the spinal implant. The ability to retrieve the devices with the present tubular configuration also confers an additional margin of safety over unencapsulated chromaffin cell implants. This paper reviews the research and clinical observations of cellular transplants containing adrenal chromaffin cells for relieving chronic pain. Encapsulated cell technology is discussed with an emphasis on our experiences developing pain-modulating clinical devices. The human-sized prototype devices were loaded with enzymatically isolated bovine chromaffin cells and maintained in vitro for 7 - 8 days in serum-free media. Two days prior to implantation, each device was assayed by static incubation to measure catecholamine and met-enkephalin output, and qualified devices (n = 6) were implanted into the sheep subarachnoid space for 6 weeks. Following a 6 week in life period, the retrieval forces of prototype devices were measured during removal from the subarachnoid space. Static incubation of the devices immediately following retrieval and after a 24 hour re-incubation period were used to quantify norepinephrine and met-enkephalin secretion profiles. This study demonstrated the safety, retrievability and maintenance of pharmacologically active encapsulated chromaffin cell-loaded devices with human implant dimensions.
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Drug Discovery Landscape
2 orphan drug designations for Postpoliomyelitis syndrome.
2 orphan drug designations for Postpoliomyelitis syndrome.
Drug | Therapy type | Regulator | Orphan designation | Approval | Sponsor |
|---|---|---|---|---|---|
Human immune globulin | antibodies | FDA | 2006-03-29 | — | Instituto Grifols, S.A. |
Recombinant human insulin-like growth factor-I | proteins | FDA | 1995-10-13 | — | Teva Branded Pharmaceutical Product R&D, Inc. |
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