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International Journal of Molecular... Nov 2022Guillain-Barré syndrome (GBS) is a rare immune-mediated acute polyradiculo-neuropathy that typically develops after a previous gastrointestinal or respiratory... (Review)
Review
Guillain-Barré syndrome (GBS) is a rare immune-mediated acute polyradiculo-neuropathy that typically develops after a previous gastrointestinal or respiratory infection. This narrative overview aims to summarise and discuss current knowledge and previous evidence regarding triggers and pathophysiology of GBS. A systematic search of the literature was carried out using suitable search terms. The most common subtypes of GBS are acute inflammatory demyelinating polyneuropathy (AIDP) and acute motor axonal neuropathy (AMAN). The most common triggers of GBS, in three quarters of cases, are previous infections. The most common infectious agents that cause GBS include , , and cytomegalovirus. is responsible for about a third of GBS cases. GBS due to is usually more severe than that due to other causes. Clinical presentation of GBS is highly dependent on the structure of pathogenic lipo-oligosaccharides (LOS) that trigger the innate immune system via Toll-like-receptor (TLR)-4 signalling. AIDP is due to demyelination, whereas in AMAN, structures of the axolemma are affected in the nodal or inter-nodal space. In conclusion, GBS is a neuro-immunological disorder caused by autoantibodies against components of the myelin sheath or axolemma. Molecular mimicry between surface structures of pathogens and components of myelin or the axon is one scenario that may explain the pathophysiology of GBS.
Topics: Humans; Amantadine; Autoantibodies; Axons; Campylobacter jejuni; Guillain-Barre Syndrome
PubMed: 36430700
DOI: 10.3390/ijms232214222 -
Journal of Neurology Mar 2022Current standard treatment in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) has been proved effective, but it is poorly effective in refractory... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Current standard treatment in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) has been proved effective, but it is poorly effective in refractory patients and unclear for anti-IgG4 antibody-associated CIDP. Rituximab is a B cell-depleting monoclonal antibody. It has been applied as one of the management strategies in CIDP, but its efficacy is unknown.
OBJECTIVE
To perform a systematic review and a meta-analysis of the efficacy of rituximab treatment in CIDP patients.
METHODS
Through searches in MEDLINE, PubMed, EMBASE, BIOSOS, Web of Science, and Cochrane library on March 31st, 2021, 15 studies were identified. Patients' characteristics, treatment regime and outcome measure were extracted.
RESULTS
Ninety-six patients in 15 studies were included. The pooled estimate of responsiveness was 75% (95% CI 72-78%). The standard mean difference (SMD) of Inflammatory Neuropathy Cause and Treatment (INCAT) disability score improvement was 1.7 (95% CI 1.0-2.3, p value < 0.0001) and the Medical Research Council (MRC) score for muscle power is 1.3 (95% CI - 2.6 to - 0.1, p value 0.04). All of the anti-IgG4 antibody-positive patients showed excellent responses to rituximab treatment.
CONCLUSION
Rituximab was effective in the treatment in CIDP patients, especially in anti-IgG4 antibody-positive patients. Randomized clinical trials are needed to determine the effectiveness and safety of rituximab in CIDP patients.
Topics: Humans; Immunoglobulin G; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating; Rituximab
PubMed: 34120208
DOI: 10.1007/s00415-021-10646-y -
Autoimmunity Reviews Mar 2022Corticosteroids are the first-line treatment for several common autoimmune neurological diseases. Other therapeutic approaches, including intravenous immunoglobulin... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Corticosteroids are the first-line treatment for several common autoimmune neurological diseases. Other therapeutic approaches, including intravenous immunoglobulin (IVIg) and plasmapheresis, have shown mixed results in patient improvement.
OBJECTIVE
To compare the efficacy of IVIg administration with that of corticosteroids, plasmapheresis, and placebo in autoimmune neurological diseases like Guillain-Barré syndrome, myasthenia gravis, chronic inflammatory demyelinating polyneuropathy, optic neuritis, and multiple sclerosis.
METHODS
A systematic review was performed on the databases PubMed, MEDLINE, Embase, and Cochrane. Controlled, randomized studies comparing the efficacy of IVIg with placebo, plasmapheresis, and/or glucocorticoid administration were selected. Only studies reporting the number of patients who improved after treatment were included, irrespective of language or publication year. In total, 23 reports were included in the meta-analysis study.
RESULTS
Our meta-analysis showed a beneficial effect of IVIg administration on patient improvement over placebo (OR = 2.79, CI [95%] = 1.40-5.55, P = 0.01). Meanwhile, IVIg administration showed virtually identical effects to plasmapheresis (OR = 0.83, CI [95%] = 0.45-1.55, P < 0.01). Finally, no significant differences were found in the efficacy of IVIg and glucocorticoid administration (OR = 0.98, Cl [95%] = 0.58-1.68, P = 0.13).
CONCLUSION
IVIg can be regarded as a viable therapeutic approach, either as a first- or second-line therapy, and as an adjuvant therapy for autoimmune neurological diseases.
Topics: Guillain-Barre Syndrome; Humans; Immunoglobulins, Intravenous; Myasthenia Gravis; Plasma Exchange; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating
PubMed: 34920107
DOI: 10.1016/j.autrev.2021.103019 -
Autoimmunity Reviews Jun 2021To compare the efficacy and compliance of up-to-date disease modifying therapies (DMTs) in patients with remitting-relapsing MS (RRMS). (Meta-Analysis)
Meta-Analysis Review
OBJECTIVE
To compare the efficacy and compliance of up-to-date disease modifying therapies (DMTs) in patients with remitting-relapsing MS (RRMS).
METHODS
We searched PubMed, EMBASE and Cochrane Library for eligible studies. Annualized relapse rate, discontinuation due to adverse events (AEs) were assessed as primary outcomes. Sensitivity analysis and inconsistency detection were performed to evaluated whether exclusion of high-risk studies affected the validity. Risk of bias was assessed using Cochrane's Risk-of-Bias Tool 2. Surface under the cumulative ranking curve (SUCRA) was used to estimate the rankings among different DMTs.
RESULTS
21 studies were included for main report. Seven studies were evaluated as "high risk" and were therefore excluded. Exclusion of high-risk studies did not affect the validity of evidence. The risk of relapses for most DMTs except Betaseron 50 μg was significantly lower comparing to placebo. Incompliance in patients treated with DMTs was not significantly increased comparing to placebo. Dimethyl fumarate and ocrelizumab had superiority in improving MRI outcomes. Ocrelizumab and ofatumumab had the largest reduction of risk in disability progression at 3 months. Referring to SUCRA, ofatumumab, alemtuzumab and natalizumab showed the best efficacy and compliance.
CONCLUSION
The present study demonstrated the hierarchy of DMTs treating RRMS. Ofatumumab, alemtuzumab and natalizumab have superiority with respect to effectiveness and compliance. More studies are required to explore the long-term effect of DMTs. Our findings could provide helpful information and contribute to clinical treatment decision-making.
Topics: Humans; Immunologic Factors; Immunosuppressive Agents; Multiple Sclerosis; Multiple Sclerosis, Relapsing-Remitting; Natalizumab; Network Meta-Analysis
PubMed: 33878488
DOI: 10.1016/j.autrev.2021.102826 -
Journal of Neurology Oct 2021Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare neurological disorder characterised by muscle weakness and impaired sensory function. The present... (Review)
Review
BACKGROUND
Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare neurological disorder characterised by muscle weakness and impaired sensory function. The present study provides a comprehensive literature review of the burden of illness of CIDP.
METHODS
Systematic literature search of PubMed, Embase, and key conferences in May 2019. Search terms identified studies on the epidemiology, humanistic burden, current treatment, and economic burden of CIDP published since 2009 in English.
RESULTS
Forty-five full texts and nineteen conference proceedings were identified on the epidemiology (n = 9), humanistic burden (n = 7), current treatment (n = 40), and economic burden (n = 8) of CIDP. Epidemiological studies showed incidence and prevalence of 0.2-1.6 and 0.8-8.9 per 100,000, respectively, depending on geography and diagnostic criteria. Humanistic burden studies revealed that patients experienced physical and psychosocial burden, including impaired physical function, pain and depression. Publications on current treatments reported on six main types of therapy: intravenous immunoglobulins, subcutaneous immunoglobulins, corticosteroids, plasma exchange, immunosuppressants, and immunomodulators. Treatments may be burdensome, due to adverse events and reduced independence caused by treatment administration setting. In Germany, UK, France, and the US, CIDP economic burden was driven by direct costs of treatment and hospitalisation. CIDP was associated with indirect costs driven by impaired productivity.
CONCLUSIONS
This first systematic review of CIDP burden of illness demonstrates the high physical and psychosocial burden of this rare disease. Future research is required to fully characterise the burden of CIDP, and to understand how appropriate treatment can mitigate burden for patients and healthcare systems.
Topics: Adrenal Cortex Hormones; Cost of Illness; Humans; Immunoglobulins, Intravenous; Plasma Exchange; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating
PubMed: 32583051
DOI: 10.1007/s00415-020-09998-8 -
Neurocritical Care Jun 2023Guillain-Barré syndrome (GBS) often carries a favorable prognosis. Of adult patients with GBS, 10-30% require mechanical ventilation during the acute phase of the...
BACKGROUND
Guillain-Barré syndrome (GBS) often carries a favorable prognosis. Of adult patients with GBS, 10-30% require mechanical ventilation during the acute phase of the disease. After the acute phase, the focus shifts to restoration of motor strength, ambulation, and neurological function, with variable speed and degree of recovery. The objective of these guidelines is to provide recommendations on the reliability of select clinical predictors that serve as the basis of neuroprognostication and provide guidance to clinicians counseling adult patients with GBS and/or their surrogates.
METHODS
A narrative systematic review was completed using Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology. Candidate predictors, including clinical variables and prediction models, were selected based on clinical relevance and presence of appropriate body of evidence. The Population/Intervention/Comparator/Outcome/Time frame/Setting (PICOTS) question was framed as follows: "When counseling patients or surrogates of critically ill patients with Guillain-Barré syndrome, should [predictor, with time of assessment if appropriate] be considered a reliable predictor of [outcome, with time frame of assessment]?" Additional full-text screening criteria were used to exclude small and lower quality studies. Following construction of an evidence profile and summary of findings, recommendations were based on four GRADE criteria: quality of evidence, balance of desirable and undesirable consequences, values and preferences, and resource use. In addition, good practice recommendations addressed essential principles of neuroprognostication that could not be framed in PICOTS format.
RESULTS
Eight candidate clinical variables and six prediction models were selected. A total of 45 articles met our eligibility criteria to guide recommendations. We recommend bulbar weakness (the degree of motor weakness at disease nadir) and the Erasmus GBS Respiratory Insufficiency Score as moderately reliable for prediction of the need for mechanical ventilation. The Erasmus GBS Outcome Score (EGOS) and modified EGOS were identified as moderately reliable predictors of independent ambulation at 3 months and beyond. Good practice recommendations include consideration of both acute and recovery phases of the disease during prognostication, discussion of the possible need for mechanical ventilation and enteral nutrition during counseling, and consideration of the complete clinical condition as opposed to a single variable during prognostication.
CONCLUSIONS
These guidelines provide recommendations on the reliability of predictors of the need for mechanical ventilation, poor functional outcome, and independent ambulation following GBS in the context of counseling patients and/or surrogates and suggest broad principles of neuroprognostication. Few predictors were considered moderately reliable based on the available body of evidence, and higher quality data are needed.
Topics: Adult; Humans; Guillain-Barre Syndrome; Prognosis; Reproducibility of Results; Respiration, Artificial; Respiratory Insufficiency
PubMed: 36964442
DOI: 10.1007/s12028-023-01707-3 -
Multiple Sclerosis and Related Disorders Aug 2017There is evidence for the benefits of exercise training in persons with multiple sclerosis (MS). However, these benefits have primarily been established in individuals... (Review)
Review
INTRODUCTION
There is evidence for the benefits of exercise training in persons with multiple sclerosis (MS). However, these benefits have primarily been established in individuals with mild-to-moderate disability (i.e., Expanded Disability Status Scale [EDSS] scores 1.0-5.5), rather than among those with significant mobility impairment. Further, the approaches to exercise training that have been effective in persons with mild-to-moderate MS disability may not be physically accessible for individuals with mobility limitations. Therefore, there is a demand for an evidence-base on the benefits of physically accessible exercise training approaches for managing disability in people with MS with mobility impairment.
OBJECTIVE
To conduct a systematic review of the current literature pertaining to exercise training in individuals with multiple sclerosis (MS) with severe mobility disability.
METHODS
Four electronic databases (PubMed, EMBASE, OvidMEDLINE, and PsychINFO) were searched for relevant articles published up until October 2016. The review focused on English-language studies that examined the effect of exercise training in people with MS with severe mobility disability, characterized as the need for assistance in ambulation or EDSS score ≥ 6.0. The inclusion criteria involved full-text articles that: (i) included participants with a diagnosis of MS; (ii) included primarily participants with a reported EDSS score ≥ 6.0 and/or definitively described disability consistent with this level of neurological impairment; and (iii) implemented a prospective, structured exercise intervention. Data were analyzed using a descriptive approach and summarized by exercise training modality (conventional or adapted exercise training), and by outcome (disability, physical fitness, physical function, and symptoms and participation).
RESULTS
Initially, 1164 articles were identified and after removal of duplicates, 530 articles remained. In total, 512 articles did not meet the inclusion criteria. 19 articles were included in the final review. Five studies examined conventional exercise training (aerobic and resistance training), and thirteen studies examined adapted exercise modalities including body-weight support treadmill training (BWSTT), total-body recumbent stepper training (TBRST), and electrical stimulation cycling (ESAC). Outcomes related to mobility, fatigue, and quality of life (QOL) were most frequently reported. Two of five studies examining conventional resistance exercise training reported significant improvements in physical fitness, physical function, and/or symptomatic and participatory outcomes. Nine of 13 studies examining adapted exercise training reported significant improvements in disability, physical fitness, physical function, and/or symptomatic and participatory outcomes.
CONCLUSIONS
There is limited, but promising evidence for the benefits of exercise training in persons with MS with severe mobility disability. Considering the lack of effective therapeutic strategies for managing long-term disability accumulation, exercise training could be considered as an alternative approach. Further research is necessary to optimize the prescription and efficacy of exercise training for adults with MS with severe mobility disability.
Topics: Disability Evaluation; Exercise Therapy; Humans; Movement Disorders; Multiple Sclerosis
PubMed: 28755682
DOI: 10.1016/j.msard.2017.06.003 -
Emerging Infectious Diseases Dec 2021We conducted a multi-institutional study in Taiwan and a systematic review of the literature for reports of Guillain-Barré syndrome after coronavirus disease...
We conducted a multi-institutional study in Taiwan and a systematic review of the literature for reports of Guillain-Barré syndrome after coronavirus disease vaccination. This condition, mostly the classic form and the acute inflammatory demyelinating polyneuropathy subtype, has been reported in 39 cases and has occurred within 2 weeks of vaccine administration.
Topics: COVID-19; COVID-19 Vaccines; Guillain-Barre Syndrome; Humans; SARS-CoV-2; Vaccination
PubMed: 34648420
DOI: 10.3201/eid2712.211634 -
Journal of Rehabilitation Medicine Oct 2012To determine the effects of physiotherapy interventions on balance in people with multiple sclerosis. (Meta-Analysis)
Meta-Analysis Review
OBJECTIVE
To determine the effects of physiotherapy interventions on balance in people with multiple sclerosis.
DATA SOURCES
A systematic literature search was conducted in Medline, Cinahl, Embase, PEDro, both electronically and by manual search up to March 2011.
STUDY SELECTION
Randomized controlled trials of physiotherapy interventions in people with multiple sclerosis, with an outcome measure linked to the International Classification of Functioning, Disability and Health (ICF) category of "Changing and maintaining body position", were included.
DATA EXTRACTION
The quality of studies was determined by the van Tulder criteria. Meta-analyses were performed in subgroups according to the intervention.
DATA SYNTHESIS
After screening 233 full-text papers, 11 studies were included in a qualitative analysis and 7 in a meta-analysis. The methodological quality of the studies ranged from poor to moderate. Low evidence was found for the efficacy of specific balance exercises, physical therapy based on an individualized problem-solving approach, and resistance and aerobic exercises on improving balance among ambulatory people with multiple sclerosis.
CONCLUSION
These findings indicate small, but significant, effects of physiotherapy on balance in people with multiple sclerosis who have a mild to moderate level of disability. However, evidence for severely disabled people is lacking, and further research is needed.
Topics: Exercise; Humans; Multiple Sclerosis; Physical Therapy Modalities; Postural Balance; Randomized Controlled Trials as Topic; Resistance Training; Sensation Disorders; Treatment Outcome
PubMed: 22990349
DOI: 10.2340/16501977-1047 -
Journal of Neurology Feb 2022Since the declaration of COVID-19 pandemic, several case reports of demyelination of both peripheral and central nervous systems have been published. The association... (Review)
Review
BACKGROUND
Since the declaration of COVID-19 pandemic, several case reports of demyelination of both peripheral and central nervous systems have been published. The association between CNS demyelination and viral infection has long been documented, and this link was recently reported following SARS-CoV-2 infection as well.
OBJECTIVES
In this systematic review, we aim to investigate the existing literature on CNS demyelination associated with SARS-CoV-2, and the proposed pathophysiological mechanisms.
METHODS
We conducted a systematic review of articles in PubMed, SCOPUS, EMBASE, Cochrane, Google Scholar and Ovid databases, from 1 January 2020 until June 15, 2021. The following keywords were used: "COVID-19", "SARS-CoV-2", "demyelination", "demyelinating disease", "multiple sclerosis", "neuromyelitis optica", and "transverse myelitis".
RESULTS
A total of 60 articles were included in the final analysis of this systematic review and included 102 patients: 52 (51%) men and 50 (49%) women, with a median age of 46.5 years. The demyelination mimicked a variety of conditions with a picture of encephalitis/encephalomyelitis being the most common. At the same time other patterns were less frequently reported such as MS, NMOSD and even MOGAD. Longitudinally extensive transverse myelitis (LETM) was the most frequently reported pattern of spinal cord involvement.
CONCLUSION
A growing body of literature has shown an association between SARS-CoV-2 infection and the development of different types of CNS demyelination. Although causality cannot readily be inferred, this review may suggest a probable causal relationship, through a para-infectious or post-infectious immune-mediated etiology in COVID-19 patients. This relationship needs to be clarified in future research.
Topics: COVID-19; Female; Humans; Male; Middle Aged; Myelitis, Transverse; Neuromyelitis Optica; Pandemics; SARS-CoV-2
PubMed: 34386902
DOI: 10.1007/s00415-021-10752-x