[Congenital malformations of the brain misinterpreted as sequelae of poliomyelitis]. / Angeborene Fehlbildungen des Gehirns als Folgezustände der Poliomyelitis verkannt.

BACKGROUND: Poliomyelitis is an infectious disease of the peripheral motor neurons, which predominantly affects children and causes residual palsies. Because of the oral poliomyelitis vaccination started in Germany in 1960 and 1962 and the following rapid decline of the incidence of this infection, the postpolio syndrome in Germany is a disease of older people. METHODS: Since 2008, we have offered a poliomyelitis outpatient consultation at the Center of Geriatrics, Protestant Hospital Göttingen-Weende and have treated 33 patients. RESULTS: The spectrum of persistent deficits after poliomyelitis ranges from palsy of single extremities to severe disability with (temporary) ventilator dependence. Many patients suffer from scoliosis or shortening of limbs of different degrees, which promotes degenerative diseases of the spinal cord and joints with secondary myelopathy, injury of spinal nerve roots or peripheral nerves or respiratory failure. The postpolio syndrome is characterized by an increase of the functional deficits after decades of compensation. The palsies of 2 of the 33 patients were not caused by poliomyelitis but by myelomeningocele and schizencephaly, respectively. CONCLUSION: The motor deficits acquired in childhood enable the majority of the patients to successfully master their lives. Because of the limited compensatory capacities of postpolio patients, even small increases in the severity of the palsy can cause a severe decline of the functional status and an impairment of the ability to live an independent life. In a substantial proportion of patients with the diagnosis poliomyelitis the symptoms are caused by other diseases.

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

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

COVID-19 and post-poliomyelitis syndrome: coincidence?

Although severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) is a novel virus, many central and peripheral nervous system manifestations associated with coronavirus disease-19 (COVID-19) infection have been reported. Beyond the neurologic manifestations, we may still have much to learn about the neuropathologic mechanism of SARS-CoV-2 infection. Here we report a case of post-poliomyelitis syndrome (PPS) related to COVID-19 and attempt to predict the possible pathophysiologic mechanism behind this association.

Extra-motor manifestations in post-polio syndrome (PPS): fatigue, cognitive symptoms and radiological features.

BACKGROUND: There is a paucity of cerebral neuroimaging studies in post-polio syndrome (PPS), despite the severity of neurological and neuropsychological sequelae associated with the condition. Fatigue, poor concentration, limited exercise tolerance, paraesthesia and progressive weakness are frequently reported, but the radiological underpinnings of these symptoms are poorly characterised. OBJECTIVE: The aim of this study is to evaluate cortical and subcortical alterations in a cohort of adult polio survivors to explore the anatomical substrate of extra-motor manifestations. METHODS: Thirty-six patients with post-polio syndrome, a disease-control group with amyotrophic lateral sclerosis patients and a cohort of healthy individuals were included in a prospective neuroimaging study with a standardised clinical and radiological protocol. Validated clinical instruments were utilised to assess mood, cognitive and behavioural domains and specific aspects of fatigue. Cortical thickness analyses, subcortical volumetry, brainstem segmentation and region-of-interest (ROI) white matter analyses were undertaken to assess regional grey and white matter alterations. RESULTS: A high proportion of PPS patients exhibited apathy, verbal fluency deficits and reported self-perceived fatigue. On ROI analyses, cortical atrophy was limited to the cingulate gyrus, and the temporal pole and subcortical atrophy were only detected in the left nucleus accumbens. No FA reductions were noted to indicate white matter degeneration in any of the lobes. CONCLUSIONS: Despite the high incidence of extra-motor manifestations in PPS, only limited cortical, subcortical and white matter degeneration was identified. Our findings suggest that non-structural causes, such as polypharmacy and poor sleep, may contribute to the complex symptomatology of post-polio syndrome.

Intravenous immunoglobulin for postpolio syndrome: a systematic review and meta-analysis.

BACKGROUND: Postpolio syndrome (PPS) is characterized by progressive disabilities that develop decades after prior paralytic poliomyelitis. Because chronic inflammation may be the process underlying the development of PPS, immunomodulatory management, such as intravenous immunoglobulin (IVIg) administration, may be beneficial. METHODS: We performed a systematic review and meta-analysis of published randomized controlled trials (RCTs) and prospective studies that evaluated the efficacy of IVIg in managing PPS. Electronic databases, including PubMed, EMBASE, CINAHL, and the Cochrane Central Register of Controlled Trials, were searched for articles on PPS published before December 2014. The primary outcomes were pain severity, fatigue scores, and muscle strength. The secondary outcomes were physical performance, quality of life (QoL), and cytokine expression levels. RESULTS: We identified 3 RCTs involving 241 patients and 5 prospective studies involving 267 patients. The meta-analysis of pain severity (weighted mean difference [WMD] = -1.02, 95% confidence interval [CI] = -2.51 to 0.47), fatigue scores (WMD = 0.28, 95% CI -0.56 to 1.12), and muscle strength revealed no significant differences between the IVIg and the placebo group. Regarding QoL, the RCTs yielded controversial outcomes, with improvement in only certain domains of the Short Form 36 (SF-36). Moreover, one prospective study reported significant improvement on SF-36, particularly in patients aged younger than 65 years, those with paresis of the lower limbs, and high pain intensity. CONCLUSION: The present review indicated that IVIg is unlikely to produce significant improvements in pain, fatigue, or muscle strength. Thus, routinely administering IVIg to patients with PPS is not recommended based on RCTs. However, a potential effect in younger patients with lower limbs weakness and intense pain requires confirmation from further well-structured trials.

Post poliomyelitis syndrome: A rare sequel of acute poliomyelitis.

Post poliomyelitis syndrome (PPS) is a rare sequel of acute poliomyelitis, usually seen 30-40 years after an acute episode. It is characterized by new muscle weakness seen in survivors of acute poliomyelitis. We describe a rare case of a 50 year old man; with a previous history of poliomyelitis in right lower limb who now presented with complaints of progressive left lower limb weakness for past two years. The diagnosis was made on the basis of clinical suspicion and EMG findings. PPS is not a well recognized disease in Pakistan and due to the lack of documentation; its true prevalence is not known. Though, over the years, cases of Poliomyelitis have decreased worldwide, however, PPS still remains a constant challenge for the physicians. This report highlights the impact of the disease on the quality of life of patients suffering from PPS and emphasis on the need for new therapeutic approach.

Post-polio syndrome - somatosensory dysfunction and its relation to pain: a pilot study with quantitative sensory testing.

OBJECTIVE: To explore and characterize somatosensory dysfunction in patients with post-polio syndrome and chronic pain, by conducting examinations with Quantitative Sensory Testing. DESIGN: A cross-sectional, descriptive, pilot study conducted during 1 month. SUBJECTS/PATIENTS: Six patients with previously established post-polio syndrome and related chronic pain. METHODS: All subjects underwent a neurological examination including neuromuscular function, bedside sensory testing, a thorough pain anamnesis, and pain drawing. Screening for neuropathic pain was done with 2 questionnaires. A comprehensive Quantitative Sensory Testing battery was conducted with z-score transformation of obtained data, enabling comparison with published reference values and the creation of sensory profiles, as well as comparison between the study site (more polio affected extremity) and internal control site (less affected extremity) for each patient. RESULTS: Derived sensory profiles showed signs of increased prevalence of sensory aberrations compared with reference values, especially Mechanical Pain Thresholds, with significant deviation from reference data in 5 out of 6 patients. No obvious differences in sensory functions were seen between study sites and internal control sites. CONCLUSION: Post-polio syndrome may be correlated with a mechanical hyperalgesia/allodynia and might be correlated to a somatosensory dysfunction. With lack of evident side-to-side differences, the possibility of a generalized dysfunction in the somatosensory system might be considered.

[General anesthesia for a patient with post-polio syndrome].

We gave anesthesia for a patient with post-polio syndrome undergoing laparoscopic sigmoid colectomy. She is a 68-year-old woman and had been diagnosed as post-polio syndrome since she was 55 years of age. Before the operation, paralysis was observed in her right leg. After inducing anesthesia using propofol, muscle relaxation was obtained by rocuronium bromide for intubation. Remifentanil was used during the operation, and good pain relief was obtained by iv-PCA (fentanyl) after operation.

Coexistence of fibromyalgia and post-polio syndrome in persons with prior poliomyelitis in Turkey: the relations with symptoms, polio-related impairments, and quality of life.

PURPOSE: To investigate the prevalence of fibromyalgia(FM) and to show its relations with symptoms, polio-related impairments (PRI), and quality of life (QoL) in persons with prior paralytic poliomyelitis (PsPP) with and without post-polio syndrome (PPS). MATERIALS AND METHODS: The study included 74 PsPP under 60 years of age, 60 of whom met the criteria for PPS. Presence and severity of FM were assessed by the American College of Rheumatology (ACR) 1990, 2010, and 2016 criteria, and Fibromyalgia Severity Score. PPS symptoms, PRI, and QoL were evaluated using the Self-Reported Impairments in Persons with Late Effects of Polio Rating Scale, Fatigue Severity Scale, and Nottingham Health Profile. Frequency, comparison, and correlation analyses were performed. RESULTS: While 15% of PsPP with PPS met the criteria of ACR 1990, 32% of ACR 2010, and 35% of ACR 2016, none of those without PPS met any of the criteria for FM. Severity of PPS symptoms and PRI were significantly higher, and QoL was significantly lower in those with co-existing FM. FM severity was found to be significantly associated with severity of PPS symptoms, PRI and reduced QoL. CONCLUSIONS: FM frequently coexists in PsPP with PPS and may increase the burden of PPS.Implications for RehabilitationFibromyalgia (FM) is commonly seen in patients with post-polio syndrome (PPS).Co-existing FM may increase the burden of PPS, as it is associated with more severe symptoms, more polio-related impairments, and worse quality of life.Recognition, appropriate referral, and successful management of co-existing FM may allow for reduced symptoms or symptom severity and improved quality of life in persons with PPS.

Primary total knee arthroplasty in patients with post-polio syndrome.

Aims: Knowledge on total knee arthroplasties (TKAs) in patients with a history of poliomyelitis is limited. This study compared implant survivorship and clinical outcomes among affected and unaffected limbs in patients with sequelae of poliomyelitis undergoing TKAs. Methods: A retrospective review of our total joint registry identified 94 patients with post-polio syndrome undergoing 116 primary TKAs between January 2000 and December 2019. The mean age was 70 years (33 to 86) with 56% males (n = 65) and a mean BMI of 31 kg/m2 (18 to 49). Rotating hinge TKAs were used in 14 of 63 affected limbs (22%), but not in any of the 53 unaffected limbs. Kaplan-Meier survivorship analyses were completed. The mean follow-up was eight years (2 to 19). Results: The ten-year survivorship free from revision was 91% (95% confidence interval (CI) 81 to 100) in affected and 84% (95% CI 68 to 100) in unaffected limbs. There were six revisions in affected limbs: three for periprosthetic femoral fractures and one each for periprosthetic joint infection (PJI), patellar clunk syndrome, and instability. Unaffected limbs were revised in four cases: two for instability and one each for PJI and tibial component loosening. The ten-year survivorship free from any reoperation was 86% (95% CI 75 to 97) and 80% (95% CI 64 to 99) in affected and unaffected limbs, respectively. There were three additional reoperations among affected and two in unaffected limbs. There were 12 nonoperative complications, including four periprosthetic fractures. Arthrofibrosis occurred in five affected (8%) and two unaffected limbs (4%). Postoperative range of motion decreased with 31% achieving less than 90° knee flexion by five years. Conclusion: TKAs in post-polio patients are complex cases associated with instability, and one in four require constraint on the affected side. Periprosthetic fracture was the main mode of failure. Arthrofibrosis rates were high and twice as frequent in affected limbs.

Renal failure in a patient with postpolio syndrome and a normal creatinine level.

Patients with renal failure who are taking trimethoprim have an increased risk of developing hyperkalemia, which can cause muscle weakness. In patients with postpolio syndrome, a normal creatinine level could be abnormally high, renal failure is possible because of lack of creatinine production, and the muscle weakness from resultant hyperkalemia could be more severe because of their underlying condition. This abnormally high creatinine level has been termed from this point relative renal failure. The objective of the study was to review a case in which relative renal failure and hyperkalemia caused muscle weakness that manifested as shortness of breath and confusion with electrocardiographic changes. A dehydrated patient with relative renal failure and postpolio syndrome had taken trimethoprim-sulfamethoxazole that caused symptomatic hyperkalemia. The patient presented with muscle weakness, shortness of breath, and confusion, with her postpolio syndrome compounding the situation and likely making the muscle weakness more severe. A patient on trimethoprim with renal failure is at an increased risk of developing hyperkalemia. Patients with postpolio syndrome could have severe muscle weakness from the hyperkalemia and could have renal failure even with a normal creatinine level. This case report will remind treating physicians to evaluate such patients for hyperkalemia if they present with muscle weakness, especially if the patient has renal failure and is on trimethoprim.

[Post-polio syndrome. Part I. The "legacy" of forgotten disease, challenges for professionals and polio survivors]. / Zespól post-polio. Czesc I. "Dziedzictwo" zapomnianej choroby, wyzwanie dla lekarzy i pacjentów

The outcome of paralytic polio was believed to be a stable neurological state. Now, it is established that polio has an additional, slowly progressive phase, called post-polio syndrome (PPS) that develops 30-40 years after the acute poliomyelitis in 25-80% of paralytic and about 40% of nonparalytic polio survivors. The clinical symptoms are nonspecific and usually include muscle weakness, fatigue and muscle or joint pain. Some patients suffer from muscular atrophy, respiratory insufficiency, dysphagia, sleep disturbances or cold intolerance. The etiopathogenesis of PPS is unclear and many factors, such as dysfunction of the surviving motor units, aging, defects of neuromuscular transmission, persistence of viral infection and immunological mechanisms, are considered.

Postpolio Syndrome from Non-paralytic Poliovirus Infection.

A 73-year-old man presented with muscle weakness and atrophy of his right arm. Atrophy of his left brachia and left calf had occurred 13 years before without any improvement or deterioration. His sister and cousin had a history of paralytic poliomyelitis. Serum poliovirus type 2 neutralizing antibody was elevated to 128×. Electromyography revealed chronic denervation potentials not only in the muscles affected previously but also in the unaffected muscles. Acute and chronic denervation potentials were found in the newly affected muscle. Postpolio syndrome should be considered in patients with unilateral muscular atrophy even when they have no history of paralytic poliomyelitis.

Describing post-polio syndrome.

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

Pain and fatigue in persons with postpolio syndrome: independent effects on functioning.

OBJECTIVES: To better understand the importance of pain and fatigue in relation to functioning, and to investigate the role that age plays in these relationships in individuals with postpolio syndrome (PPS). DESIGN: Cross-sectional survey. SETTING: Community-based survey. PARTICIPANTS: Convenience sample of 446 individuals with PPS. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: Physical functioning (Patient Reported Outcomes Measurement Information System Physical Functioning item bank items), psychological functioning (Patient Health Questionnaire-9), pain intensity (0-10 numerical rating scale [NRS]), and fatigue (0-10 NRS). RESULTS: Pain and fatigue make independent contributions to the prediction of physical and psychological functioning. Depression was more severe in the middle-aged (≤64y) group than in the young-old (65-74y) or middle-old to oldest (≥75y) groups, although the associations between pain and fatigue and both physical and psychological functioning are similar across all age cohorts. CONCLUSIONS: Complaints of pain or fatigue in patients with PPS who are older or elderly should not be attributed "merely" to the process of aging. The findings also support the need for clinical trials to develop and evaluate interventions that may help patients with PPS function better by treating pain and fatigue, as well as the negative effects that these symptoms can have on functioning.

Fatigue and aging with a disability.

OBJECTIVE: To compare self-reported fatigue in 4 disability populations with age-matched, U.S. population norms. We assessed fatigue and age in a sample of individuals with spinal cord injury (SCI), postpolio syndrome (PPS), multiple sclerosis (MS), and muscular dystrophy (MD). DESIGN: This study used survey responses and published age cohort means for fatigue to test the hypothesis that fatigue would be higher in each of 4 clinical samples than the U.S. population norm. We also hypothesized that, for clinical samples, the mean fatigue reported within age cohorts would be higher than the general U.S. population norms for those age ranges derived in the Patient-Reported Outcomes Measurement Information System (PROMIS). SETTING: Survey responses were collected from participants in the Washington state area. PARTICIPANTS: Participants (N=1836) were persons with MD (n=337), MS (n=580), Post-polio (n=441), and SCI (n=478). INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURE: PROMIS Depression Short Form. RESULTS: Individuals with disabilities reported higher levels of fatigue than the normative PROMIS population. In the normative population, self-reported fatigue was substantially lower in age cohorts from middle age to retirement age. However, individuals with disabilities did not demonstrate this age cohort effect. CONCLUSIONS: Individuals with disabilities are not only at greater risk to experience fatigue, but this risk, relative to normative values, increases with age. More research is needed to determine the specific negative impact of fatigue symptoms on functioning in individuals with disabilities as they age.

Concomitant CNS pathology in a patient with amyotropic lateral sclerosis following poliomyelitis in childhood.

Post-polio syndrome (PPS) develops in approximately 30% of polio survivors several decades after the acute attack of paralytic poliomyelitis. Some of these patients develop post-poliomyelitis muscular atrophy (PPMA) which is characterized by a slowly progressive muscle weakness. Due to its clinicopathological features, investigators have often studied PPS and PPMA in association with amyotrophic lateral sclerosis (ALS), the underlying hypothesis being an increased risk of developing ALS from a prior acute paralytic poliomyelitis. Various studies, however, have indicated that de novo ALS cases in patients with prior acute paralytic poliomyelitis are rare. Herein, we describe a rare case of a 75-year-old woman who at post-mortem examination presented a combination of a PPS with proven histopathological sporadic ALS features. Furthermore, neuropathology of this case also revealed several other histopathological findings reminiscent of a tauopathy, synucleinopathy and amyloid angiopathy and a large pituitary cyst. To our knowledge, this is the first reported case of PPS with clear pathological hallmarks of sporadic ALS, including ubiquitin-, TDP-43, phosphorylated TDP-43- and p62-positive inclusions, with accompanying features compatible with Alzheimer's and Parkinson's disease.

Medical Comorbidities and Complications Associated with Poliomyelitis and Its Sequelae.

Survivors of poliomyelitis may experience long-term sequelae that put them at increased risk for injury, pain, cardiovascular deconditioning, and functional decline. Osteoporotic fractures and entrapment neuropathies, in particular, may result in greater impairments in one's mobility and ability to perform activities of daily living. Dysphagia may necessitate the use of compensatory swallow strategies to minimize aspiration risk. Comorbid conditions, including hypertension, dyslipidemia, obesity, and stroke, are also very prevalent in this population. Risk factor modification, including diet, exercise, and medication compliance, is essential to achieve optimal health and function among survivors of poliomyelitis.

Imaging as an alternate diagnostic modality in a presumptive case of abdominopelvic TB in a COVID-19 patient.

A 30-year-old, multiparous widow, with postpolio residual paralysis, presented with complaints of dull aching abdominal pain for 15 days. Ultrasound showed a mixed echogenic right adnexal mass with free fluid in the pelvis and abdomen. CT abdomen and pelvis revealed partially defined peripherally enhancing collection in lower abdomen and right adnexa suggestive of tubo-ovarian abscess. There was mild ileal wall thickening and few enlarged mesenteric lymph nodes. Ascitic fluid did not show acid fast bacilli and cultures were sterile. Extensive diagnostic laboratory work was done which was inconclusive. Diagnostic laparoscopy could not be performed due to non-availability of elective operation theatre in the COVID-19 pandemic. Presumptive extrapulmonary tuberculosis was clinically and radiologically diagnosed. She was started on daily anti tuberculosis treatment. This case shows us the importance of imaging as a diagnostic tool and as an alternative for laparoscopy in COVID-19 pandemic to diagnose abdomino-pelvic tuberculosis.