Guillain-Barre Syndrome Publications (7449)
Guillain-Barre Syndrome Publications
Methods: 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. Results: 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. Conclusion: 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.
Sequentially, nerve conduction studies confirmed demyelination of both facial nerves. A diagnosis of Guillain-Barré syndrome was made according to clinical findings, laboratory results and nerve study results.
IVIg therapy was initiated (2 g/kg per 5 days) after no response to tapered intravenous corticosteroid therapy. Five days after the last IVIg dose the patient started to recover. Both eyes were treated with standard protective measures.
Guillain-Barré syndrome is an acute autoimmune polyneuropathy characterized by a course of rapid ascending muscular weakness and mild sensory symptoms appearing approximately two-four weeks after an infection. The facial nerve is of all cranial nerves most often affected but it is rarely the prominent sign of GBS. As well, bilateral facial palsy has never been described as a sole manifestation of GBS, as of our notion.
He experienced bronchitis with watery diarrhea and had laboratory evidence of recent infection with Epstein-Barr virus (EBV). He was diagnosed as having overlapping GBS and BBE associated with EBV and received treatment with a combination of immunoglobulin and methylprednisolone, as well as acyclovir, and had recovered completely after 3 months. In addition, he has not experienced any relapse over the past year. We suggest that combinations of symptoms and signs of central lesions (disturbance of consciousness) and peripheral lesions (ophthalmoplegia, facial weakness, limb weakness, and areflexia) are supportive of a diagnosis of overlapping GBS and BBE and can be helpful in achieving an early diagnosis, as well as for the administration of appropriate treatments.
We subjected all data to Rasch analyses, and calculated for each patient the magnitude of change on both scales using the minimal clinically important difference (MCID) related to the individual standard errors (SEs). A responder was defined as having an MCID-SE ≥1.96. Individual scores on both measures were correlated with the EuroQoL thermometer (heuristic responsiveness).
The I-RODS showed a significantly higher proportion of meaningful improvement compared with the INCAT-ONLS findings in GBS/CIDP. For IgM-MGUSP, the lack of responsiveness during the 1-year study did not allow a clear separation. Heuristic responsiveness was consistently higher with the I-RODS.
The I-RODS more often captures clinically meaningful changes over time, with a greater magnitude of change, compared with the INCAT-ONLS disability scale in patients with GBS and CIDP. The I-RODS offers promise for being a more sensitive measure and its use is therefore suggested in future trials involving patients with GBS and CIDP.
The main conditions that are handled and reported were acute inflammatory polyneuropathy (Guillain Barre Syndrome) and viral encephalitis, 19.7 and 18.5%, respectively. The condition showing a tendency to decrease was neurocysticercosis, and conditions most strongly associated with mortality were diseases associated to the human immunodeficiency virus and unspecified viral encephalitis. The conditions were more frequent especially in men in the age group 25 to 44 years old. It is necessary to stress the importance of timely reporting of diseases under epidemiological surveillance in Mexico, since knowledge of the behavior of action allows decisions at all levels of care.
The lumbar puncture showed protein cell separation at the fifth week after the onset of symptoms. At the same time-point, the electrophysiological examination showed demyelination changes involving the trigeminal nerve and the somatic motor nerve. Needle electromyography revealed normal results. The clinical symptoms ceased progression at the fourth week after symptom onset, and began to improve from the sixth. This case was considered to be sensory Guillain-Barré syndrome, which was characterized by its cranial nerve involvement.
We defined two groups: group 1 (group treated by IvIg: 0.4 g/kg/day for 5 days) and group 2 (group treated by PE: 4 PE during 10-14 days). We collected demographic characteristics, clinical and therapeutic aspects and outcome.
The quantitative variables are expressed on mean ± standard derivation and compared by Student test. The statistic analysis has been based on SPSS for windows. P < 0.05 is considered as significant.
Forty-one patients (21 in group 1 and 20 in group 2) were enrolled. The mean age was 37.4 ± 9.2 years, with a masculine predominance (75.4%). Electromyogram in all patients found acute inflammatory demyelinating polyradiculoneuropathy in 80.5% of patients. The mean length of hospitalization was 45.3 ± 9.2 days. The length of hospitalization of the IvIg group is less long than PE group (p = 0.03). The weaning of the MV was more precocious in IvIg group than PE group (p = 0.01). Also, the beginning of motility recuperation was precocious at IvIg group than PE group (p = 0.04).
Our work reveals a meaningful difference for the MV weaning and precocious recovery in IvIg group compared to PE group.
Logistic regression models were performed to determine risk factors of hyponatremia and poor outcome. Results: Hyponatremia was detected in 21·5% GBS patients. And 36·2% severe GBS cases were found to have hyponatremia, which was significantly higher than mild and moderate groups (11·8, 13·0%; all P value <0·001). However, no significant difference was found between mild and moderate groups (P = 0·769). Multivariable logistic regression analysis identified three conditions closely associated with hyponatremia: age > 50 years (odds ratio: 2·013, P = 0·014), facial weakness (odds ratio: 2·493, P = 0·001), and mechanical ventilation (odds ratio: 12·871, P < 0·001). Both hyponatremia (odds ratio: 12·770, P < 0·001) and bulbar weakness (odds ratio: 3·391, P = 0·023) predicted death of GBS patients. Conclusions: Hyponatremia is an important predictor of poor outcome in GBS patients.
During chemotherapy, severe infection and progressive flaccid quadriparesis appeared, which eventually developed to respiratory muscles paralysis. The clinical course and neurological examination were consistent with GBS. Following mechanical ventilation and intravenous immunoglobulin administration, the neurological symptoms were in remission after one month. Three months later, the patient achieved complete remission without any treatment during this period. We hypothesized that immune reconstruction may have a significant role in this phenomenon.
Meta-analysis was performed using random effects and heterogeneity was assessed using the I 2 value. Meta-regression was used to explore the influence of study-level variables on heterogeneity. A total of 32 studies were identified; 25 reported on ReA, five reported on RS, seven reported on IBS, two reported on IBD, two reported on GBS, one reported on MFS, and two reported on HUS. There was insufficient data in the literature to calculate a pooled estimate for RS, HUS, IBD, GBS, or MFS. The pooled estimate of the proportion of cases of NTS that developed ReA and IBS had substantive heterogeneity, limiting the applicability of a single estimate. Thus, these estimates should be interpreted with caution and reasons for the high heterogeneity should be further explored.
4 million) episodes of foodborne gastroenteritis occurred, many of which might have resulted in sequelae. We estimated the number of illnesses, hospitalizations, and deaths from Guillain-Barré syndrome, hemolytic uremic syndrome, irritable bowel syndrome, and reactive arthritis that were associated with contaminated food in Australia. Data from published studies, hospital records, and mortality reports were combined with multipliers to adjust for different transmission routes. We used Monte Carlo simulation to estimate median estimates and 90% CrIs. In Australia, circa 2010, we estimated that 35,840 (90% CrI 25,000-54,000) illnesses, 1,080 (90% CrI 700-1,600) hospitalizations, and 10 (90% CrI 5-14) deaths occurred from foodborne gastroenteritis-associated sequelae. Campylobacter spp. infection was responsible for 80% of incident cases. Reducing the incidence of campylobacteriosis and other foodborne diseases would minimize the health effects of sequelae.