Guillain-Barre Syndrome Publications (7465)
Guillain-Barre Syndrome Publications
There is an ongoing trial of a second course of IVIg in patients with severe GBS.
We retrospectively reviewed 4 patients with severe GBS who received high-dose IVIg. One patient inadvertently received a high dose of IVIg for Miller Fisher syndrome. All patients received a total of at least 2 courses of the standard dose of IVIg (total >4 g/kg). We review their clinical course and side effects.
All patients with non-O blood types developed clinically significant hemolytic anemia requiring blood transfusion.
Hemolytic anemia may limit doses of IVIg for treatment of severe GBS in patients with non-O blood types.
On 5 November 2013, we searched the Cochrane Neuromuscular Disease Group Specialized Register, CENTRAL, MEDLINE, EMBASE, CINAHL Plus, LILACS and AMED. We also searched reference lists of all studies identified for inclusion and relevant reviews, and contacted the authors of included studies and known experts in the field to identify additional published or unpublished data. We also searched trials registries for ongoing studies.
We considered for inclusion randomised controlled trials (RCTs) and quasi-RCTs comparing any form of intervention for fatigue management in adults with peripheral neuropathy with placebo, no intervention or an alternative form of intervention for fatigue. Interventions considered included drugs, pacing and grading of physical activity, general or specific exercise, compensatory strategies such as orthotics, relaxation, counselling, cognitive and educational strategies.
Two review authors independently assessed risk of bias and extracted study data. We contacted study authors for additional information. We collected information on adverse events from the included trials.
The review includes three trials, which were all at low risk of bias, involving 530 people with peripheral neuropathy. The effects of amantadine from one randomised, double-blind, placebo-controlled, cross-over trial comparing amantadine with placebo for the treatment of fatigue in 80 people with Guillain-Barré syndrome (GBS) were uncertain for the proportion of people achieving a favourable outcome six weeks post-intervention (odds ratio (OR) 0.56 (95% confidence interval (CI) 0.22 to 1.35, N = 74, P = 0.16). We assessed the quality of this evidence as low. Two parallel-group randomised double-blind, placebo-controlled trials comparing the effects of two doses of ascorbic acid with placebo for reducing fatigue in adults with Charcot-Marie-Tooth disease type 1A (CMT1A) showed that the effects of ascorbic acid at either dose are probably small (standardised mean difference (SMD) -0.12 (95% CI -0.32 to 0.08, n = 404, P = 0.25)) for change in fatigue after 12 to 24 months (moderate quality evidence). Neither ascorbic acid study measured fatigue at four to 12 weeks, which was our primary outcome measure. No serious adverse events were reported with amantadine. Serious adverse events were reported in the trials of ascorbic acid. However,risk of serious adverse events was similar with ascorbic acid and placebo.
One small imprecise study in people with GBS showed uncertain effects of amantadine on fatigue. In two studies in people with CMT1A there is moderate-quality evidence that ascorbic acid has little meaningful benefit on fatigue. Information about adverse effects was limited, although both treatments appear to be well tolerated and safe in these conditions.There was no evidence available from RCTs to evaluate the effect of other drugs or other interventions for fatigue in either GBS, CMT1A or other causes of peripheral neuropathy. The cost effectiveness of different interventions should also be considered in future randomised clinical trials.
The clinical phenotype of hepatitis E continues to emerge, and recent data show that HEV is associated with a range of neurological syndromes including Guillain-Barré syndrome and neuralgic amytrophy.
Treatment-naïve patients with CIDP initially treated with at least one full course of IVIg (2 g/kg) at one of two neuromuscular disease centres were included. Patients fulfilled the European Federation of Neurological Societies/Peripheral Nerve Society clinical criteria for CIDP. Significant improvement following IVIg was defined as an improvement (≥1 grade) on the modified Rankin scale. Difference in weakness between arms and legs was defined as ≥2 grades on the Medical Research Council scale between ankle dorsiflexion and wrist extension. Clinical predictors with a p value <0.15 in univariate analysis were analysed in multivariate logistic regression.
Of a total of 281 patients, 214 patients (76%) improved. In univariate analysis, the presence of pain, other autoimmune disease, difference in weakness between arms and legs, and a myelin-associated glycoprotein negative IgM monoclonal gammopathy of undetermined significance were associated with no response to IVIg. In multivariate analysis no pain (p=0.018) and no difference in weakness between arms and legs (p=0.048) were independently associated with IVIg response. Of IVIg non-responders, 66% improved with plasma exchange and 58% with corticosteroids.
IVIg is a very effective first-line treatment. Patients with CIDP presenting with pain or a difference in weakness between arms and legs are less likely to respond to IVIg.
The remaining two who recovered well were relatively younger with a mean age of 50 years, had no prodromal diarrhea, required ventilatory support by fourth day of illness, no cardiovascular autonomic symptoms and demyelinative neuropathy.
ResultsThis report focuses on Guillain-Barre Syndrome (GBS), Rheumatoid Arthritis (RA), Systemic Lupus Erythematosus (SLE), and Idiopathic (or immune) Thrombocytopenic Purpura (ITP). Multiple curated databases and automated text mining of PubMed literature identified 667 genes associated with RA, 448 with SLE, 49 with ITP and 73 with GBS. While all data sources provided valuable and unique gene associations, text mining using natural language processing (NLP) algorithms provided the most information but required curation to remove incorrect associations. Six genes were associated with all four AIDs. Thirty-three pathways were shared by the four AIDs. Classification of genes into twelve immune system related categories identified more ¿Th17 T-cell subtype¿ genes in RA than the other AIDs, and more ¿Chemokine plus Receptors¿ genes associated with RA than SLE. Gene networks were visualized and clustered into interconnected modules with specific gene clusters for each AID, including one in RA with ten C-X-C motif chemokines. The intersection of genes associated with GBS, GBS peptide auto-antigens, influenza A infection, and influenza vaccination created a subnetwork of genes that inferred a possible role for the MAPK signaling pathway in influenza vaccine related GBS.ConclusionsResults showing unique and common gene sets, pathways, immune system categories and functional clusters of genes in four autoimmune diseases suggest it is possible to develop molecular classifications of autoimmune and inflammatory events. Combining this information with cellular and other disease responses should greatly aid in the assessment of potential immune-mediated adverse events following vaccination.
In the future, further understanding of the underlying processes may lead to more effective and targeted therapies of these disorders. This article is part of a Special Issue entitled: Neuromuscular Diseases: Pathology and Molecular Pathogenesis.
Overall, 131 AFP cases in Lombardy were reported with a mean annual incidence rate of 0.7/100 000 children <15 years of age (range: 0.3/100 000-1.1/100 000). The sensitivity of the surveillance system was optimal from 2001-2003. The monthly distribution of AFP cases was typical with peaks in November, in January, and in March. The major clinical diagnoses associated with AFP were Guillain-Barré Syndrome (GBS, 40%) and encephalomyelitis/myelitis (13%). According to the virological results, no poliomyelitis cases were caused by wild PV infections, but two Vaccine-Associated Paralytic Paralysis (VAPP) cases were reported in 1997 when the Sabin oral polio vaccine (OPV) was still being administered in Italy. Since a surveillance system is deemed sensitive if at least one case of AFP per 100,000 children <15 years of age is detected each year, our surveillance system needs some improvement and must be maintained until global poliovirus eradication will be declared.
IGOS will make it possible to better inform patients and relatives about the prognosis for GBS, to understand the mechanism of disease progression and recovery, and to conduct selective therapeutic trials to improve outcome in patients with poor prognosis.
In chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), diagnostic pitfalls can be recalled. Our knowledge of underlying pathophysiological processes has improved, and the value of monitoring with function and deficit scores has been demonstrated. IVIG can sometimes be effective longer than expected, but CIDP remains sensitive to corticosteroids, particularly with the long-term beneficial effects of megadose dexamethasone. The impact of fingolimod remains to be demonstrated in an ongoing trial. Advances concerning multifocal motor neuropathy, inflammatory plexopathy, and neuropathy with anti -MAG activity are discussed but treatments already recognized as effective should not be changed. Imaging of peripheral nerve progresses.
We estimated serious ESAVI rates by age and target group, as well as by clinical diagnosis, and completed descriptive analyses of final outcomes and classifications given in country. A total of 1000 serious ESAVI were reported by 18 of the 29 LAC countries that vaccinated against A[H1N1]pdm. The overall reporting rate in LAC was 6.91 serious ESAVI per million doses, with country reporting rates ranging from 0.77 to 64.68 per million doses. Rates were higher among pregnant women (16.25 per million doses) when compared to health care workers (13.54 per million doses) and individuals with chronic disease (4.03 per million doses). The top three most frequent diagnoses were febrile seizures (12.0%), Guillain-Barré Syndrome (10.5%) and acute pneumonia (8.0%). Almost half (49.1%) of the serious ESAVI were reported among children aged <18 years of age; within this group, the highest proportion of cases was reported among those aged <2 years (53.1%). Of all serious ESAVI reported, 37.8% were classified as coincidental, 35.3% as related to vaccine components, 26.4% as non-conclusive and 0.5% as a programmatic error. This regional overview of A[H1N1]pdm vaccine safety data in LAC estimated the rate of serious ESAVI at lower levels than other studies. However, the ESAVI diagnosis distribution is comparable to the published literature. Lessons learned can be applied in the response to future pandemics.
As understanding continues to increase regarding the physiology of these conditions and the best treatment, rapid identification, triage, and treatment of these patients in the emergency department is paramount.
We retrospectively evaluated the medical records and electrodiagnostic study (EDS) of 121 GBS patients who were managed in our hospital during 11 years. After regarding the exclusion criteria, patients classified as three groups: acute inflammatory demyelinating polyneuropathy (AIDP), acute motor axonal neuropathy (AMAN), and acute motor sensory axonal neuropathy (AMSAN). The most frequent subtype and then electrophysiological characteristic based on the time of EDS and their cerebrospinal fluid (CSF) profile were assessed.
Among 70 patients finally included in the study, 67% were men. About 63%, 23%, and 14% had AIDP, AMAN, and AMSAN, respectively. AIDP patients represented a wider range of ages compared with other groups. Higher levels of CSF protein, abnormal late responses and sural sparing were more frequent in AIDP subtype. Five AMSAN patients also revealed sural sparing. Conduction block (CB) was observed in one AMAN patient. Prolonged F-wave latency was observed only in AIDP cases. CB and inexcitable sensory nerves were more frequent after 2 weeks, but reduced F-wave persistency was more prominent in the early phase.
AIDP was the most frequent subtype. Although the electrophysiology and CSF are important diagnostic tools, classification should not be made based on a distinct finding.
We analyzed several variables associated with this treatment, including patient characteristics, time from liver transplantation to conversion to everolimus, immunosuppression regimens before and after conversion, treatment efficacy, adverse events, and discontinuation after conversion. Median follow-up after conversion to everolimus was 27 months (range, 1-63 mo). Neurotoxic events were: akinetic mutism in 4 patients, repeated convulsions in 3, cerebrovascular accident in 1, Guillain-Barré syndrome in 1, and disabling tremor in 1. Treatment with calcineurin inhibitors was discontinued in all patients. Post-conversion regimens consisted of everolimus plus mycophenolate mofetil (MMF) plus steroids in 7 patients, everolimus plus MMF in 1, everolimus plus steroids in 1, and everolimus alone in 1. Liver function was maintained for ≥1 month in all patients except 1, who presented a severe rejection that was treated with steroid bolus and Neoral cyclosporine. Neurologic function was fully recovered in 8 patients. In 1 patient with akinetic mutism and another with convulsions, tacrolimus was reintroduced at 2 months and 1 month, respectively, after resolution of the neurotoxic event. Everolimus is feasible and effective as the main immunosuppressant in patients suffering severe neurotoxicity during the 1st 3 months after transplantation. It allows neurologic function to be recovered while maintaining adequate liver function.
Nerve conduction studies of corresponding nerves were undertaken. Consequently, significant differences between the groups were found in compound muscle action potential amplitudes, distal motor latency, F-wave latency and persistency. Ultrasonic cross-sectional areas showed significant enlargement in all nerves except of the ulnar nerve (upper arm) and the sural nerve compared to healthy controls, most prominent in proximal and middle median nerve (p < 0.01). The vagal nerve also showed enlargement compared to controls (p < 0.05), which was most pronounced in patients with autonomic dysfunction compared to patients without (p < 0.05). C6 root diameter showed a significant correlation to the amount of CSF-protein (Pearson correlation, p <0.05). Ultrasonography shows nerve enlargement in several peripheral nerves including vagal nerve and C6 root in acute phase of GBS and could be an additional diagnostic tool, e.g., in GBS of atypical onset and autonomic dysfunction.
Observational studies reporting the number of Campylobacter cases that developed reactive arthritis (ReA), Reiter's syndrome (RS), haemolytic uraemic syndrome (HUS), irritable bowel syndrome (IBS), inflammatory bowel disease (IBD) ,Guillain Barre syndrome (GBS) or Miller Fisher syndrome (MFS) were included. Data extraction through independent extraction of articles by four reviewers (two per article). Random effects meta-analysis was performed and heterogeneity was assessed using the I2 value. Meta-regression was used to explore the influence of study level variables on heterogeneity.
A total of 31 studies were identified; 20 reported on ReA, 2 reported on RS, 9 reported on IBS, 3 studies reported on IBD, 8 reported on GBS, 1 reported on MFS and 3 reported on HUS. The proportion of Campylobacter cases that developed ReA was 2.86% (95% CI 1.40% - 5.61%, I2 = 97.7%), irritable bowel syndrome was 4.01% (95% CI 1.41% - 10.88%, I2 = 99.2%). Guillain Barre syndrome was 0.07% (95% CI 0.03% - 0.15%, I2 = 72.7%).
A significant number of Campylobacter cases develop a chronic sequela. However, results should be interpreted with caution due to the high heterogeneity.
Neurological examination revealed distal weakness and loss of deep tendon reflexes in lower extremities. Nerve conduction studies displayed severe demyelinating sensorimotor polyneuropathy, and plasmapheresis was therefore applied with mild improvement. Few weeks after, dysphagia occurred and electrophysiologic tests showed progressive axonal involvement with spread of demyelination to the cranial nerves. The patient underwent a new plasmapheresis course and slowly reached stable clinical improvement of neurological status, which allowed him to be safely discharged. This case showed a critical onset with respiratory failure and kidney functional impairment due to L. pneumophila, subsequently disclosing Guillain-Barré syndrome.
Both patients made a full clinical recovery. The tissue distribution and function of CASPR2 make it a biologically plausible autoimmune target in GBS and its clincial relevance in GBS should be determined in further studies.
Due to the rarity of the rheumatic diseases, the evidence for IVIG use has been shown to be lacking in some areas and promising in others. Conditions in which IVIG has been shown to have benefit include ITP, Guillain-Barré syndrome and chronic inflammatory demyelinating polyneuropathy occurring in the context of rheumatic disease, as well as in SLE, idiopathic inflammatory myopathies and ANCA-associated vasculitides. This review looks at current IVIG use and is designed to be an aid for rheumatologists when considering the use of IVIG in clinical practice.
It is considered safe and effective, conferring protection in more than 90% in 10 years. Adverse effects are known as mild reactions (allergies, transaminases transient elevation, fever, headache) and severe (visceral and neurotropic disease related to vaccine). However, little is known about its potential to induce autoimmune responses. This systematic review aims to identify the occurrence of autoinflammatory diseases related to 17D vaccine administration. Six studies were identified describing 13 possible cases. The diseases were Guillain-Barré syndrome, multiple sclerosis, multiple points evanescent syndrome, acute disseminated encephalomyelitis, autoimmune hepatitis, and Kawasaki disease. The data suggest that 17D vaccination may play a role in the mechanism of loss of self-tolerance.
We assessed GBS patients who had mechanical ventilation for more than two months. PTSD was assessed using Horowitz Impact of Event Scale (IES), IESRevisited (IES-R) and the Post-traumatic CheckList Scale (PCLS); functional outcome using Rankin and Barthel scales; QoL using Nottingham Health Profile (NHP) and 36-Item Short Form Health Survey (SF-36) and depression using Hospital Anxiety and Depression Scale (HAD) and Beck questionnaire. Thirteen patients could be identified and analyzed. They had only mild disability. They were neither anxious nor depressed with an anxiety HAD at 5 [4-11.5], a depression HAD at 1 [0 - 3.5] and a Beck at 1 [0-5]. QoL was mildly decreased in our population with a NHP at 78.5 [12.8-178.8] and mild decreased SF-36. Compared to the French population, the SF-36 sub-categories were, however, not statistically different. 22% of our 13 patients had PTSD and post-traumatic stress symptoms with a Horowitz IES at 12 [2-29], and an IES-R at 16 [2-34.5]. Although severe GBS patients requiring prolonged mechanical ventilation had good functional recovery and no difference in QoL, they had a high incidence of post-traumatic stress symptoms.
1%) or critical illness neuropathy (n = 32 (17.2%)).
Cohort analysis of data from the UK Rehabilitation Outcomes Collaborative national clinical dataset. Outcome measures include the UK Functional Assessment Measure, Northwick Park Dependency Score (NPDS) and Care Needs Assessment (NPCNA). Patients were analysed in three groups of dependency based on their admission NPDS score: 'low' (NPDS<10), 'medium' (NPDS 10-24) and 'high' (NPDS ≥25). Cost-efficiency was measured as the time taken to offset the cost of rehabilitation by savings in NPCNA-estimated costs of on-going care in the community.
The mean rehabilitation length of stay was 72.2 (sd = 66.6) days. Significant differences were seen between the diagnostic groups on admission, but all showed significant improvements between admission and discharge, in both motor and cognitive function (p<0.0001). Patients who were highly dependent on admission had the longest lengths of stay (mean 97.0 (SD 79.0) days), but also showed the greatest reduction in on-going care costs (£1049 per week (SD £994)), so that overall they were the most cost-efficient to treat.
Patients with polyneuropathies have both physical and cognitive disabilities that are amenable to change with rehabilitation, resulting in significant reduction in on-going care-costs, especially for highly dependent patients.
pylori usually persists throughout life, resulting in a chronic inflammatory response with local secretion of numerous inflammatory mediators including chemokines [interleukin (IL)-8, macrophage chemotactic protein (MCP)-1, growth-regulated oncogene (GRO)-α] and cytokines [IL-1β, tumor necrosis factor (TNF)-α, IL-6, IL-12, interferon (IFN)-γ], which can pass into the circulation and have a systemic effect. The persistence of detectable systemic and local concentrations of inflammatory mediators is likely to alter the outcome of neurological diseases. These proinflammatory factors can induce brain inflammation and the death of neurons and could eventually be associated to Parkinson's disease and also may be involved in the development of Alzheimer's disease. However, most neurological diseases are the result of a combination of multiple factors, but the systemic inflammatory response is a common component and determinant in the onset, evolution, and outcome of diseases. However, more studies are needed to allow understanding of the effects and mechanisms by which the inflammatory response generated by H. pylori infection affects neurological diseases.
Second, the female predominance of autoimmune diseases such as systemic sclerosis is well recognized but enigmatic, and a unifying hypothesis remains elusive. Third, the management of conditions triggered by infectious agents as in Guillain-Barre syndrome or mixed cryoglobulinemia is challenging, and clinical guidelines are needed in the setting of infections and autoimmunity. Fourth, gamma-delta T cells represent major players in innate immunity and are the subject of extensive studies in autoimmune diseases to provide new therapeutic targets for disease prevention or modulation in the near future. Ultimately, we acknowledge the major developments in the broad fields of rheumatology and immunology and expect that microbiota definition, epigenetics studies, and microRNA analysis will provide new exciting avenues toward the understanding and treatment of chronic and acute inflammation.
In the present study, we observed that GBS patients have increased frequencies of Th1 and Th17 cells, but reduced number of Foxp3(+) regulatory T cells (Treg cells) with defective functions. We show that IVIg treatment in GBS patients results in a marked reduction in the frequency of Th1 and Th17 cells with a concomitant expansion of Treg cells. Importantly, IVIg-expanded Treg cells exhibited an increased T cell suppressive function. Together our results demonstrate that therapeutic benefit of IVIg in GBS patients implicates the reciprocal regulation of Th1/Th17 and Treg cells.
Forty-eight patients with GBS were studies. Mean age of onset was 5 years. Male and female ratio was 1.4:1. History of antecedent infection was 73%. Clinical presentations included limb weakness 100%, respiratory distress 27%, facial palsy 27%, autonomic nervous dysfunction 22% and ataxia 17%. Nerve conduction study revealed demyelinating process in 57%, axonopathy in 26% and mixed type in 17%. Clinical outcomes were satisfactory in most of the patients except three patients who still had disability eighteen months after onset. Autonomic nervous dysfunction was a significant factor to determine the severity.
The demographic and clinical features of GBS were similar to other published studies. The autonomic nervous dysfunction was a significant predictor for adverse clinical course. All but three patients had complete, full recovery.
Studies comparing germ--free animals and animals exposed to pathogenic bacterial infections, probiotics or antibiotics suggest the participation of the microbiota in this communication and a role in host defense, regulation of immunity and autoimmune disease appearance. The GIT could represent a vulnerable area through which pathogens influence all aspects of physiology and even induce CNS neuro--inflammation. All those concepts may suggest the modulation of the gut microbiota as an achievable strategy for innovative therapies in complex disorders. Moving from this background, the present review discusses the relationship between intestinal microbiota and CNS and the effects in health and disease. We particularly look at how the commensal gut microbiota influences systemic immune response in some neurological disorders, highlighting its impact on pain and cognition in multiple sclerosis, Guillain--Barrè Syndrome, neurodevelopment and behavioral disorders and Alzheimer's disease. In this review we discuss recent studies showing that the potential microbiota--gut--brain dialogue is implicated in neurodegenerative diseases. Gaining a better understanding of the relationship between microbiota and CNS could provide an insight on the pathogenesis and therapeutic strategies of these disorders.