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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 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 -
BMC Neurology Mar 2020Despite many benefits of the physical activity on physical and mental health of patients with Multiple Sclerosis (MS), the activity level in these patients is still very... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Despite many benefits of the physical activity on physical and mental health of patients with Multiple Sclerosis (MS), the activity level in these patients is still very limited, and they continue to suffer from impairment in functioning ability. The main aim of this study is thus to closely examine exercise's effect on fatigue of patients with MS worldwide, with particular interest on Iran based on a comprehensive systematic review and meta-analysis.
METHODS
The studies used in this systematic review were selected from the articles published from 1996 to 2019, in national and international databases including SID, Magiran, Iranmedex, Irandoc, Google Scholar, Cochrane, Embase, ScienceDirect, Scopus, PubMed and Web of Science (ISI). These databases were thoroughly searched, and the relevant ones were selected based on some plausible keywords to the aim of this study. Heterogeneity index between studies was determined using Cochran's test and I. Due to heterogeneity in studies, the random effects model was used to estimate standardized mean difference.
RESULTS
From the systematic review, a meta-analysis was performed on 31 articles which were fulfilled the inclusion criteria. The sample including of 714 subjects was selected from the intervention group, and almost the same sample size of 720 individuals were selected in the control group. Based on the results derived from this meta-analysis, the standardized mean difference between the intervention group before and after the intervention was respectively estimated to be 23.8 ± 6.2 and 16.9 ± 3.2, which indicates that the physical exercise reduces fatigue in patients with MS.
CONCLUSION
The results of this study extracted from a detailed meta-analysis reveal and confirm that physical exercise significantly reduces fatigue in patients with MS. As a results, a regular exercise program is strongly recommended to be part of a rehabilitation program for these patients.
Topics: Exercise; Exercise Therapy; Fatigue; Humans; Iran; Multiple Sclerosis
PubMed: 32169035
DOI: 10.1186/s12883-020-01654-y -
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 Comparative Effectiveness... Jul 2023To assess the relative efficacy of disease-modifying therapies (DMTs) for relapsing multiple sclerosis (RMS) including newer therapies (ozanimod, ponesimod,... (Meta-Analysis)
Meta-Analysis
To assess the relative efficacy of disease-modifying therapies (DMTs) for relapsing multiple sclerosis (RMS) including newer therapies (ozanimod, ponesimod, ublituximab) using network meta-analysis (NMA). Bayesian NMAs for annualised relapse rate (ARR) and time to 3-month and 6-month confirmed disability progression (3mCDP and 6mCDP) were conducted. For each outcome, the three most efficacious treatments versus placebo were monoclonal antibody (mAb) therapies: alemtuzumab, ofatumumab, and ublituximab for ARR; alemtuzumab, ocrelizumab, and ofatumumab for 3mCDP; and alemtuzumab, natalizumab, and either ocrelizumab or ofatumumab (depending on the CDP definition used for included ofatumumab trials) for 6mCDP. The most efficacious DMTs for RMS were mAb therapies. Of the newer therapies, only ublituximab ranked among the three most efficacious treatments (for ARR).
Topics: Humans; Multiple Sclerosis; Multiple Sclerosis, Relapsing-Remitting; Alemtuzumab; Network Meta-Analysis; Bayes Theorem; Recurrence
PubMed: 37265062
DOI: 10.57264/cer-2023-0016 -
Neurology Jan 2021Since the last epidemiologic review of neuromyelitis optica/neuromyelitis optica spectrum disorder (NMO/NMOSD), 22 additional studies have been conducted. We...
OBJECTIVE
Since the last epidemiologic review of neuromyelitis optica/neuromyelitis optica spectrum disorder (NMO/NMOSD), 22 additional studies have been conducted. We systematically review the worldwide prevalence, incidence, and basic demographic characteristics of NMOSD and provide a critical overview of studies.
METHODS
PubMed, Ovid MEDLINE, and Embase using Medical Subject Headings and keyword search terms and reference lists of retrieved articles were searched from 1999 until August 2019. We collected data on the country; region; methods of case assessment and aquaporin-4 antibody (AQP4-Ab) test; study period; limitations; incidence (per 100,000 person-years); prevalence (per 100,000 persons); and age-, sex-, and ethnic group-specific incidence or prevalence.
RESULTS
We identified 33 relevant articles. The results indicated the highest estimates of incidence and prevalence of NMOSD in Afro-Caribbean region (0.73/100 000 person-years [95% CI: 0.45-1.01] and 10/100 000 persons [95% CI: 6.8-13.2]). The lowest incidence and prevalence of NMOSD were found in Australia and New Zealand (0.037/100 000 person-years [95% CI: 0.036-0.038] and 0.7/100,000 persons [95% CI: 0.66-0.74]). There was prominent female predominance in adults and the AQP4-Ab-seropositive subpopulation. The incidence and prevalence peaked in middle-aged adults. African ethnicity had the highest incidence and prevalence of NMOSD, whereas White ethnicity had the lowest. No remarkable trend of incidence was described over time.
CONCLUSION
NMOSD is a rare disease worldwide. Variations in prevalence and incidence have been described among different geographic areas and ethnicities. These are only partially explained by different study methods and NMO/NMOSD definitions, highlighting the need for specifically designed epidemiologic studies to identify genetic effects and etiologic factors.
Topics: Global Health; Humans; Incidence; Neuromyelitis Optica; Prevalence
PubMed: 33310876
DOI: 10.1212/WNL.0000000000011153 -
Journal of Neurology Jan 2021We conducted a systematic review and wide-angled Mendelian randomization (MR) study to examine the association between possible risk factors and multiple sclerosis (MS).
OBJECTIVES
We conducted a systematic review and wide-angled Mendelian randomization (MR) study to examine the association between possible risk factors and multiple sclerosis (MS).
METHODS
We used MR analysis to assess the associations between 65 possible risk factors and MS using data from a genome-wide association study including 14 498 cases and 24 091 controls of European ancestry. For 18 exposures not suitable for MR analysis, we conducted a systematic review to obtain the latest meta-analyses evidence on their associations with MS.
RESULTS
Childhood and adulthood body mass index were positively associated with MS, whereas physical activity and serum 25-hydroxyvitamin D were inversely associated with MS. There was evidence of possible associations of type 2 diabetes, waist circumference, body fat percentage, age of puberty and high-density lipoprotein cholesterol. Data of systematic review showed that exposure to organic solvents, Epstein Barr virus and cytomegalovirus virus infection, and diphtheria and tetanus vaccination were associated with MS risk.
CONCLUSIONS
This study identified several modifiable risk factors for primary prevention of MS that should inform public health policy.
Topics: Adult; Child; Diabetes Mellitus, Type 2; Epstein-Barr Virus Infections; Genome-Wide Association Study; Herpesvirus 4, Human; Humans; Mendelian Randomization Analysis; Multiple Sclerosis; Risk Factors
PubMed: 32728946
DOI: 10.1007/s00415-020-10119-8 -
Multiple Sclerosis (Houndmills,... Feb 2023Intrathecal immunoglobulin-G synthesis is a hallmark of multiple sclerosis (MS), which can be detected by oligoclonal IgG bands (OCB) or by κ-free light chains (κ-FLC)... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Intrathecal immunoglobulin-G synthesis is a hallmark of multiple sclerosis (MS), which can be detected by oligoclonal IgG bands (OCB) or by κ-free light chains (κ-FLC) in cerebrospinal fluid.
OBJECTIVE
To perform a systematic review and meta-analysis to evaluate whether κ-FLC index has similar diagnostic value to identify patients with clinically isolated syndrome (CIS) or MS compared to OCB, and to determine κ-FLC index cut-off.
METHODS
PubMed was searched for studies that assessed diagnostic sensitivity and specificity of κ-FLC index and OCB to discriminate CIS/MS patients from control subjects. Two reviewers following preferred reporting items for systematic reviews and meta-analyses (PRISMA) guidelines performed study eligibility assessment and data extraction. Findings from studies were analyzed with bivariate mixed models.
RESULTS
A total of 32 studies were included in the meta-analysis to evaluate diagnostic value of κ-FLC index. Sensitivity and specificity ranged from 52% to 100% (weighted average: 88%) and 69% to 100% (89%) for κ-FLC index and from 37% to 100% (85%) and 74% to 100% (92%) for OCB. Mean difference of sensitivity and specificity between κ-FLC index and OCB was 2 and -4 percentage points. Diagnostic accuracy determined by mixed models revealed no significant difference between κ-FLC index and OCB. A discriminatory cut-off for κ-FLC index was determined at 6.1.
CONCLUSION
The findings indicate that κ-FLC index has similar diagnostic accuracy in MS as OCB.
Topics: Humans; Multiple Sclerosis; Immunoglobulin kappa-Chains; Oligoclonal Bands; Immunoglobulin G; Demyelinating Diseases; Biomarkers
PubMed: 36453167
DOI: 10.1177/13524585221134213 -
Multiple Sclerosis and Related Disorders May 2021Multiple sclerosis (MS) commonly affects young adults at the ages 20 to 40 years old, but it can onset at each age. Late-onset multiple sclerosis (LOMS) is defined as... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Multiple sclerosis (MS) commonly affects young adults at the ages 20 to 40 years old, but it can onset at each age. Late-onset multiple sclerosis (LOMS) is defined as symptoms initiating after the age of 50. Misdiagnosis and a remarkable gap in diagnosis of LOMS is a challenge of the elderly population so in this article we described the proportion of LOMS and the clinical features and phenotype of the disease in this age group.
METHODS
After registration of the study protocol, an electronic search was performed in 3 databases and for full coverage of the published studies, we also checked the references of each related article. Two independent researchers screened the records in title/abstract and full-text stages and extracted the data using a data extraction table. The risk of bias was assessed using Joanna Briggs Institute checklist and meta-analysis was conducted by CMA 2. Only the studies with 50 years old cut-off and using McDonald or Poser diagnostic criteria were included in the meta-analysis.
RESULTS
After removing duplicated studies, out of 733 results of electronic and hand searching, 31 studies met our inclusion criteria for the systematic review, and 11 of them were included in the quantitative synthesis. With different cut-offs and diagnostic methods 1.1% to 21.3% proportion of LOMS, has been reported in the studies. Meta-analysis reached a 5.01% (95% CI: 3.78% to 6.57%), proportion of LOMS in total MS cases. The female cases were more than males (range between 57.7% to 70.2%) and 64.46% (95% CI: 61.94% to 66.91%) proportion of females has been found in this study. 65.00% (95% CI: 44.71% to 81.02%) proportion of spinal cord involvements and 49.80% (95% CI: 39.28% to 60.24%) proportion of relapsing-remitting multiple sclerosis (RRMS) was also observed in LOMS cases. In 4 of included studies, the progressive form was the predominant phenotype. The most prevalent first disease presentation of LOMS was motor dysfunction (ranges between 100% to 35.4%) followed by sensory problems (ranges between 94% to 5%), visual symptoms (ranges between 22.9% to 5%), and brainstem dysfunction (ranges between 25% to 12.3%). The proportion of positive oligoclonal band (OCB), was varied from 46% to 98% in different studies and positive immunoglobulin G (IgG) index also was seen in 45.04% and 66% of the patients. 2.2% - 12.5% of the LOMS cases had a positive family history.
CONCLUSION
In about 5% of cases, MS can be diagnosed at ages above 50 years old. There is an increasing concern of a more progressive form of MS in LOMS cases. Unlike the adult-onset MS, the first presentation of LOMS is usually motor dysfunction. Understanding the proportion and clinical features of LOMS will help clinicians with the diagnosis of MS in this age group and prevention of wrong management plans and complications in these patients. Although the proportion of females is still more than males in LOMS cases; but there is a trend to increase in male cases with aging.
Topics: Adult; Age of Onset; Aged; Disease Progression; Female; Humans; Male; Middle Aged; Multiple Sclerosis; Multiple Sclerosis, Relapsing-Remitting; Oligoclonal Bands; Young Adult
PubMed: 33571792
DOI: 10.1016/j.msard.2021.102816