Post-polio syndrome: clinical manifestations and cerebrospinal fluid markers

Fiorini, Michele; Zanusso, Gianluigi; Baj, Andreina; Bertolasi, Laura; Toniolo, Antonio; Monaco, Salvatore

doi:10.2217/14796708.2.4.451

Cited by 6 publications

(7 citation statements)

References 78 publications

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“…We previously showed that 14-3-3 was positive in the CSF of neuroleptic-treated patients [13], as well as other neurologic and inflammatory diseases, such as Guillain Barrè syndrome [14], post-polio syndrome [15], acute myelitis [16], and multiple sclerosis [17]. Most of these CSF samples had normal tau levels, likely because the degree of neuronal or axonal damage was not severe enough to determine an increase in released tau protein in the CSF [13].…”

Section: Discussionmentioning

confidence: 98%

High Diagnostic Accuracy of RT-QuIC Assay in a Prospective Study of Patients with Suspected sCJD

Fiorini

Iselle

Perra

et al. 2020

IJMS

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The early and accurate in vivo diagnosis of sporadic Creutzfeldt–Jakob disease (sCJD) is essential in order to differentiate CJD from treatable rapidly progressive dementias. Diagnostic investigations supportive of clinical CJD diagnosis include magnetic resonance imaging (MRI), electroencephalogram (EEG), 14-3-3 protein detection, and/or real-time quaking-induced conversion (RT-QuIC) assay positivity in the cerebrospinal fluid (CSF) or in other tissues. The total CSF tau protein concentration has also been used in a clinical setting for improving the CJD diagnostic sensitivity and specificity. We analyzed 182 CSF samples and 42 olfactory mucosa (OM) brushings from patients suspected of having sCJD with rapidly progressive dementia (RPD), in order to determine the diagnostic accuracy of 14-3-3, the total tau protein, and the RT-QuIC assay. A probable and definite sCJD diagnosis was assessed in 102 patients. The RT-QuIC assay on the CSF samples showed a 100% specificity and a 96% sensitivity, significantly higher compared with 14-3-3 (84% sensitivity and 46% specificity) and tau (85% sensitivity and 70% specificity); however, the combination of RT-QuIC testing of the CSF and OM samples resulted in 100% sensitivity and specificity, proving a significantly higher accuracy of RT-QuIC compared with the surrogate biomarkers in the diagnostic setting of patients with RPD. Moreover, we showed that CSF blood contamination or high protein levels might interfere with RT-QuIC seeding. In conclusion, we provided further evidence that the inclusion of an RT-QuIC assay of the CSF and OM in the diagnostic criteria for sCJD has radically changed the clinical approach towards the diagnosis.

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Section: Discussionmentioning

confidence: 98%

High Diagnostic Accuracy of RT-QuIC Assay in a Prospective Study of Patients with Suspected sCJD

Fiorini

Iselle

Perra

et al. 2020

IJMS

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“…There are no diagnostic tests or specific biomarkers for PPS. 22,66,67 The early diagnostic criteria of Halstead represent the basis of the criteria presented by Trojan and Cashman 68 have been integrated into current diagnostic guidelines: 22 (1) Prior paralytic poliomyelitis with evidence of motor neuron loss, as confirmed by history of the acute paralytic illness, signs of residual weakness, and atrophy of muscles on neurological examination, and signs of denervation on electromyography. (2) A period of partial or complete functional recovery after acute paralytic poliomyelitis, followed by an interval (usually 15 years of more) of stable neurological function.…”

Section: Diagnostic Criteria For Ppsmentioning

confidence: 99%

Post-poliomyelitis syndrome as a possible viral disease

Baj

Colombo

Headley³

et al. 2015

International Journal of Infectious Diseases

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This review summarizes current concepts on post-polio syndrome (PPS), a condition that may arise in polio survivors after partial or complete functional recovery followed by a prolonged interval of stable neurological function. PPS affects 15-20 million people worldwide. Epidemiological data are reported, together with the pathogenic pathways that possibly lead to the progressive degeneration and loss of neuromuscular motor units. As a consequence of PPS, polio survivors experience new weakness, generalized fatigue, atrophy of previously unaffected muscles, and a physical decline that may culminate in the loss of independent life. Emphasis is given to the possible pathogenic role of persistent poliovirus infection and chronic inflammation. These factors could contribute to the neurological and physical decline in polio survivors. A perspective is then given on novel anti-poliovirus compounds and monoclonal antibodies that have been developed to contribute to the final phases of polio eradication. These agents could also be useful for the treatment or prevention of PPS. Some of these compounds/antibodies are in early clinical development. Finally, current clinical trials for PPS are reported. In this area, the intravenous infusion of normal human immunoglobulins appears both feasible and promising.

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“…About two to three decades after the acute episode, there is a tendency to overload this system, that can be accelerated depending on individual factors especially the activities and exercises carried out that promote overuse of the affected muscles. In this case of overuse and super training , an intense metabolic demand in the residual motor units occurs, which then triggers a process of secondary neuronal death (Orsini et al, 2015), and active inflammatory process is present in the spinal cord with increased level of cytokines in the cerebrospinal fluid but without any convincing evidence of viral reactivation (Fiorini et al, 2007).…”

Section: Introductionmentioning

confidence: 99%

A twelve‐week, four‐arm, randomized, double‐blind, placebo‐controlled, phase 2 prospective clinical trial to evaluate the efficacy and safety of an anthroposophic multimodal treatment on chronic pain in outpatients with postpolio syndrome

et al. 2020

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Introduction: Chronic pain and fatigue are the main symptoms of postpoliomyelitis syndrome (PPS). This study aimed to evaluate the efficacy and safety of an anthroposophic multimodal treatment for chronic pain in PPS outpatients. Methods: A twelve-week, four-arm, randomized, double-blind, placebo-controlled, phase 2 prospective clinical trial was designed to compare four groups (n = 48): groups A and B received daily active experimental transdermal gel (ETG) or placebo gel (PTG), respectively; groups C and D received weekly external therapies, art therapies, and neurofunctional reorganization, plus either daily ETG or PTG, respectively. The pain symptoms were evaluated through a visual analogue scale (VAS), the McGill questionnaire, and thermography. Quality of life and resilience were evaluated by the WHOQOL-BREF and Antonovsky sense of coherence questionnaires applied at baseline and after the interventions.Results: No related adverse events occurred, and 10% of the patients reports dysphagia improvement. In the groups C and D, pain reduction was statistically significant in both the placebo group (p = .02, d = 1.315) and in the ETG (p = .005, d = 2.035).However, following the week-to-week evolution of pain with the concomitant use of the ETG, this significant pain reduction occurred earlier from the 4th week and continued to decrease (p = .016, d = 1.369). In the group that received the complete multimodal treatment, the greatest significant benefit in increasing quality of life occurred in the physical domain and elevation in resilience with an emphasis on meaning and comprehension domains. Conclusions:The anthroposophic multimodal treatment group presented both safety and efficacy as an analgesic in the groups that received the nonpharmacological therapies, much earlier when associated with the ETG. The multimodal approach

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Post-polio syndrome: clinical manifestations and cerebrospinal fluid markers

Cited by 6 publications

References 78 publications

High Diagnostic Accuracy of RT-QuIC Assay in a Prospective Study of Patients with Suspected sCJD

High Diagnostic Accuracy of RT-QuIC Assay in a Prospective Study of Patients with Suspected sCJD

Post-poliomyelitis syndrome as a possible viral disease

A twelve‐week, four‐arm, randomized, double‐blind, placebo‐controlled, phase 2 prospective clinical trial to evaluate the efficacy and safety of an anthroposophic multimodal treatment on chronic pain in outpatients with postpolio syndrome

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