Guillain-Barre Syndrome Publications (8111)
Guillain-Barre Syndrome Publications
Therefore the efficacy of IVIg, IVIg fractions with various IgG Fc sialylation status, and the involvement of Th2 pathway were examined in one of our animal model of antibody-mediated inhibition of axonal regeneration. We demonstrate that both IVIg and sIVIg ameliorated anti-glycan antibody mediated-pathological effect, whereas, the unsialylated fractions of IVIg were not beneficial in our model. Tenfold lower doses of sIVIg compared to whole IVIg provided equivalent efficacy in our studies. Moreover, we found that whole IVIg and sIVIg significantly upregulates the gene expression of IL-33, which itself can provide protection from antibody-mediated nerve injury in our model. Our results support that the SIGN-R1-Th2 pathway is involved in the anti-inflammatory effects of IVIg on endoneurium in our model and elements of this pathway including IL-33 can provide novel therapeutics in inflammatory neuropathies.
An observational study of consecutive patients with confirmed CHIKV infection admitted to the ICU in Martinique and Guadeloupe, French West Indies, between January and November 2014, was performed. In addition, patients with CHIKV-related manifestations were compared with those whose manifestations were not specifically related to CHIKV infection.
Sixty-five patients were admitted to the ICU with CHIKV infection. Fifty-four (83%) had a previous underlying disease and 27 (41.5%) were admitted because of exacerbation of a comorbidity. Thirty-seven (57%) patients were mechanically ventilated. ICU and hospital-mortality rate were 26 and 27% respectively. CHIKV-related manifestations were observed in 28 (18%) patients and were mainly encephalitis, Guillain-Barré syndrome and severe sepsis. These patients had less frequently chronic arterial hypertension and diabetes and more frequently autoimmune diseases compared with patients without CHIKV-related manifestations.
Most patients admitted to the ICU with CHIKV infection had a pre-existing comorbidity. However, severe manifestations such as Guillain-Barré syndrome, encephalitis and severe sepsis could be specifically related to CHIKV.
Preceding Campylobacter jejuni (C. jejuni) infection and antiganglioside antibodies were also assessed. Seven (3%) recurrent GBS and one (1.4%) recurrent MFS patients were identified. In the individual patient the clinical features during episodes were usually similar varying in severity whereas the preceding infection differed. None of the patients had GBS in one episode and MFS in the recurrence or vice versa. Recurrent GBS patients, compared with monophasic GBS, did not have preceding diarrhea at the first episode and considering the electrophysiological subtypes, acute inflammatory demyelinating polyneuropathies recurred more frequently than axonal GBS (6.5% vs 0.9%, p=0.04). In conclusion in a GBS population with a balanced number of demyelinating and axonal subtypes less frequent diarrhea and demyelination at electrophysiology were associated with recurrence.
68 million, conducted GBS surveillance in Spain in 2009-2011. The network, established in 1996, carried out a retrospective and a prospective study to estimate monthly alarm thresholds in GBS incidence and tested them in 1998-1999 in a pilot study. Such incidence thresholds additionally to observation of GBS cases with immunisation antecedent in the 42 days prior to clinical onset were taken as alarm signals for 2009-2011, since November 2009 onwards. For purpose of surveillance, in 2009 we updated both the available centres and the populations served by the network. We also did a retrospective countrywide review of hospital-discharged patients having ICD-9-CM code 357.0 (acute infective polyneuritis) as their principal diagnosis from January 2009 to December 2011.
Among 141 confirmed of 148 notified cases of GBS or Miller-Fisher syndrome, Brighton 1-2 criteria in 96 %, not a single patient was identified with clinical onset during the 42-day time interval following A(H1N1)pdm09 vaccination. In contrast, seven cases were seen during a similar period after seasonal campaigns. Monthly incidence figures did not, however, exceed the upper 95 % CI limit of expected incidence. A retrospective countrywide review of the registry of hospital-discharged patients having ICD-9-CM code 357.0 (acute infective polyneuritis) as their principal diagnosis did not suggest higher admission rates in critical months across the period December 2009-February 2010.
Despite limited power and underlying reporting bias in 2010-2011, an increase in GBS incidence over background GBS, associated with A(H1N1)pdm09 monovalent or trivalent influenza immunisations, appears unlikely.
Illustrated through an interactive tool, we evaluated how the number of Zika cases averted, the period during pregnancy in which Zika infection poses a risk of microcephaly, and probabilities of microcephaly and Guillain-Barré Syndrome (GBS) impact the cost at which an intervention is cost-effective. From Northeast Brazilian microcephaly incidence data, we estimated the probability of microcephaly in infants born to Zika-infected women (0.49% to 2.10%). We also estimated the probability of GBS arising from Zika infections in Brazil (0.02% to 0.06%) and Colombia (0.08%). We calculated that each microcephaly and GBS case incurs the loss of 29.95 DALYs and 1.25 DALYs per case, as well as direct medical costs for Latin America and the Caribbean of $91,102 and $28,818, respectively. We demonstrated the utility of our cost-effectiveness tool with examples evaluating funding commitments by Costa Rica and Brazil, the US presidential proposal, and the novel approach of genetically modified mosquitoes. Our analyses indicate that the commitments and the proposal are likely to be cost-effective, whereas the cost-effectiveness of genetically modified mosquitoes depends on the country of implementation.
Current estimates from our tool suggest that the health burden from microcephaly and GBS warrants substantial expenditures focused on Zika virus control. Our results justify the funding committed in Costa Rica and Brazil and many aspects of the budget outlined in the US president's proposal. As data continue to be collected, new parameter estimates can be customized in real-time within our user-friendly tool to provide updated estimates on cost-effectiveness of interventions and inform policy decisions in country-specific settings.
Acute motor axonal neuropathy, a subtype of GBS, was diagnosed by cerebrospinal fluid and nerve conduction velocity testing. An electroencephalogram showed that the patient had normal, appropriately reactive brain function. Transcranial Doppler (TCD) ultrasound showed appropriate blood flow to the brain. GBS rarely presents with weakness so severe as to mimic brain death. This article provides a review of similar literature. This case demonstrates the importance of performing a proper brain death examination, which includes evaluation for irreversible cerebral injury, exclusion of any confounding conditions, and performance of tests such as electroencephalography and TCDs when uncertainty exists about the reliability of the clinical exam.
Although ZIKV infection has not yet been reported in transplant recipients, it is likely that it will be reported soon because of the number of transplants performed in affected areas and global travel. We discuss the effect of ZIKV in transplantation and propose recommendations to prevent donor-derived infections.
We report a 14-year-old boy with GBS, who presented with hyperreflexia and bilateral papillitis. To the best of our knowledge, this is the first pediatric patient presenting with papillitis and hyperreflexia with acute motor and sensory axonal neuropathy form of GBS.
We applied a previously developed mathematical model on importations of dengue to estimate the number of ZIKV importations into Europe, based on the travel volume, the probability of being infected at the time of travel, the population size of Brazil, and the estimated incidence of ZIKV infections.
Our model estimated between 508 and 1,778 imported infections into Europe in 2016, of which we would expect between 116 and 355 symptomatic Zika infections; with the highest number of importations being into France, Portugal and Italy.
Our model identified high-risk countries in Europe. Such data can assist policymakers and public health professionals in estimating the extent of importations in order to prepare for the scale up of laboratory diagnostic assays and estimate the occurrence of Guillain-Barré Syndrome, potential sexual transmission, and infants with congenital ZIKV syndrome.
Amiothoplic lateral sclerosys (ALS), for which the eponym is Charcot's disease, has been replaced in the USA by Lou Gehrig's disease. Guillain-Barré syndrome (GBS) is an eponym that is still the subject of controversy, and various different names are associated with it. Finally,restless legs syndrome (RLS), which was for years known as Ekbom's syndrome, has been rechristened as RLS/Willis-Ekbom syndrome.
To better understand the transmission dynamics of ZIKV, we used a mathematical model to examine the 2013-14 outbreak on the six major archipelagos of French Polynesia. Our median estimates for the basic reproduction number ranged from 2.6-4.8, with an estimated 11.5% (95% CI: 7.32-17.9%) of total infections reported. As a result, we estimated that 94% (95% CI: 91-97%) of the total population of the six archipelagos were infected during the outbreak. Based on the demography of French Polynesia, our results imply that if ZIKV infection provides complete protection against future infection, it would take 12-20 years before there are a sufficient number of susceptible individuals for ZIKV to re-emerge, which is on the same timescale as the circulation of dengue virus serotypes in the region. Our analysis suggests that ZIKV may exhibit similar dynamics to dengue virus in island populations, with transmission characterized by large, sporadic outbreaks with a high proportion of asymptomatic or unreported cases.
Recent data suggest that acute ZIKV infection in pregnant women may result in acute infection of fetal tissue and brain tissue, causing microcephaly and potentially severe debilitation of the infant or even death of the fetus. Cases of acute ZIKV are also associated with Guillain-Barré syndrome. With the increased number of cases, new complications such as ocular involvement and sexual transmission have been reported.
Zika virus is an emerging viral pathogen with significant consequences on human health throughout the world. Ongoing research into this pathogen is urgently needed to produce viable vaccine and therapeutic options.
At present, diagnosis is still a challenge in the field and there is no treatment available. Another major challenge is that one must devise therapies for pregnant women, at all stages of pregnancy. Devising adequate treatment for ZIKV infections represents a challenge that will only be met by the joint effort of the research community.
The study was conducted at tertiary training and research hospital. From January 2014 to September 2015, a total of 75 patients diagnosed with encephalitis, ataxia, Guillain Barre Syndrome (GBS), facial paralysis, acute disseminated encephalomyelitis (ADEM), pseudotumorcerebri were evaluated for inclusion to the study. Among these patients whom investigations of B. burgdorferi antibody IgM and/or IgG ELISA and Western Blot (WB) were detected to be positive, were assessed. Epidemiologic data, tick bite histories, duration of symptoms, clinical findings, radiologic findings, treatment durations and prognosis were investigated.
Totally 7 patients had been treated with the diagnosis of Lyme neuroborreliosis. The mean age was 9.14±4.91 years; duration of symptoms before admission was 8.0±4.50 days; and the duration of antibiotic use was 2.85±0.89 weeks. All patients had received ceftriaxone and intravenous immunoglobulin (IVIG); 3 patients had received plasmapheresis (42.9%) and one patient had received pulse corticosteroid therapy. While the patient with the diagnosis of encephalomyeloneuritis and atypical GBS had partially improved, the other patients were completely cured.
In this article, we report pediatric LNB patients, B. burgdorferi should also be considered in patients with atypical or severe neurologic involvement or a history of tick bite; it is known that the prognosis is good with appropriate and early treatment.
The authors of that 2003 analysis used a statistical analysis of his case by selectively choosing some of his reported symptoms. FDR's diagnosis of poliomyelitis, however, was fully supported by the findings of leading expert physicians of that time, who were very knowledgeable in the then-common disease and who periodically examined him during the period of 1921-1924. The most significant diagnostic features of polio are the absence of objective sensory findings in the presence of flaccid motor paralysis. These features are consistent with diagnostic criteria extant during the periods of major poliomyelitis epidemics as well as those of the Center for Disease Control 90 years later. Additional findings of fever, prodromal hyperesthesia, more severe residual proximal muscle weakness, and extensive lower extremity impairment requiring mobility with long leg braces or a wheelchair give further evidence for the diagnosis in FDR's case. Nonbulbar Guillain-Barré Syndrome, which shares the features of a flaccid paralysis and thus mimicking the initial presentation of poliomyelitis, has more than an 80% complete recovery with no reported cases of eventual wheelchair use. The most severe cases of Guillain-Barré Syndrome often have persistent objective sensory loss, associated with greater weakness in the feet and hands, which show no resemblance to FDR's impairment and disability. In light of the expert initial assessments by physicians completely familiar with the signs and symptoms of the then-common disease, review of his initial and subsequent disease course, and residual symptoms in comparison with those of Guillain-Barré syndrome, we find no reason to question the diagnostic accuracy of poliomyelitis and wish to put this debate to rest.
In 2007, however, the first major outbreak of ZIKV occurred in Yap Island (Micronesia), and so it was reported for the first time outside of Africa and Asia. Between the years of 2007 and 2014, ZIKV spreaded to island groups located in Southeast Asia and the Pacific Ocean, and in 2015-2016, it has spread to South and Central America and the Caribbean. Today, travel-related imported cases is still been reported in Europe, North America, and other countries in the Far East. According to the data from the World Health Organization and the Centers for Disease Control and Prevention, as of March 2016, ZIKV infections have already spread locally in more than 30 countries, and travel alerts have been issued for the countries where the virus is present. Zika virus infections are generally asymptomatic or may present with a moderate clinical picture (e.g. acute onset of fever, maculopapular rash, arthralgia, and nonpurulent conjunctivitis). Although no deaths were attributed to ZIKV infection over the past 60 years, as of November 2015, it has been suggested that three deaths in Brazil, including the death of a newborn with microcephaly, may be attributed to ZIKV infection. In addition, concurrent with outbreaks in 2013 in French Polynesia and in 2015 in Brazil, there have been significant rises reported in the incidence of some autoimmune and neurodevelopmental disorders, including Guillain-Barre syndrome and microcephaly; these reports have caused considerable international concern. There are many points that are still unclear about ZIKV, including: (1) intrauterine transmission risk, frequency, and effects of the infection on fetal development; (2) the probability of perinatal transmission and if so the possible risks; (3) association with autoimmune and neurological diseases, and presence of long-term sequelae risks after infection; (4) possible routes of transmission other than mosquito bites, such as sexual contact, blood transfusion, and other body fluids (saliva, semen, or urine); (5) presence of reservoir(s) and different mosquito vectors; (6) diagnostic difficulties including cross reactivity in serological tests and standardization of testing procedures; (7) severity of the infection in immunocompromised patients; and (8) the potential effectiveness of antiviral therapy or preventive vaccines. In this review, updated information and recommendations regarding ZIKV outbreaks and risks, and the epidemiology, diagnosis and characteristics of ZIKV infections, are summarized in light of the most recent literature.
Pig herds worldwide are infected with HEV genotype 3 and HEV has been found in the human food chain in a number of developed countries. However, the route of transmission is not fully understood, since most cases are not obviously associated with pigs/pig products. HEV can be transmitted by blood transfusion and surprisingly high numbers of asymptomatic blood donors are viremic at the time of donation: Germany 1:1,200, Netherlands 1:2,671, England 1:2,848. Our understanding of the clinical phenotype of HEV infection in humans has undergone a sea-change in recent years. Previously, HEV was thought to cause only acute self-limiting hepatitis. However, HEV may cause persistent disease in the immunocompromised. Patients with chronic HEV infection have no symptoms, but some develop rapidly progressive liver cirrhosis. The full clinical spectrum of HEV is still emerging. HEV has important extra-hepatic manifestations, which deserve further investigation. For example, HEV can cause a wide range of neurological illness. In particular, very recent data suggest that Guillain-Barré syndrome and neuralgic amyotrophy are associated with locally acquired HEV in approximately 5 and 10% of the cases, respectively.
The patient had persistent weakness and progressed to areflexia, at which point urgent lumbar puncture supported a diagnosis of GBS.
The patient was promptly started on intravenous immunoglobulin and made significant clinical improvement with near-complete resolution of symptoms by 3-month follow-up visit. By the sixth month, he was able to function and ambulate without a cane. GBS is a rare and potentially critical cause of diminished reflexes and weakness in the post-operative elective orthopedic patient. We propose that morbid obesity may have contributed to the patient's susceptibility of developing GBS following surgery.
Neurologic symptoms of this autoimmune condition may also mimic the clinical picture of an elective spine patient, thus confounding diagnosis. If imaging cannot explain exam findings or new neurologic symptoms post-operatively, rare disease processes should be considered in the differential diagnosis.
To provide physicians with an updated clinical review of the epidemiology, pathogenesis, diagnosis, management, and mimics of zika virus.
This flavivirus is spread by the bite of the Aedes mosquito during daylight. The ease of worldwide travel has allowed the virus to spread to Mexico and the U.S. Main transmission route is via blood contact or sexual activity involving mucous membranes. Incubation ranges from 2 to 12 days, but only 20% of patients experience symptoms. Fever is low grade with conjunctivitis, arthralgias, myalgias, and a maculopapular rash. Chikungunya and Dengue Fever differ in that patients experience higher fever and no conjunctivitits. The dreaded complication of Zika virus is microcephaly in infants born to infected mothers. Guillain-Barre Syndrome is also linked to the virus. Historical factors including travel history are paramount, and diagnosis includes PCR or serology. No current treatment regimen exists beyond symptom control. The emergency physician must seek to rule out other similar diseases such as malaria, chikungunya, and dengue fever.
Zika virus has created public fear due to complications, and this flavivirus spread by the Aedes mosquito presents similarly to Chikungunya and Dengue Fever. The dreaded complication of Zika virus is microcephaly in infants born to infected mothers. This review provides key information concerning the disease and management.
Progress in assessing interventions will require the development of animal models to test efficacies; however, there are only limited reports on in vivo studies. The only susceptible murine models have involved intracerebral inoculations or juvenile animals, which do not replicate natural infection. Our report has studied the effect of ZIKV infection in type-I interferon receptor deficient (A129) mice and the parent strain (129Sv/Ev) after subcutaneous challenge in the lower leg to mimic a mosquito bite. A129 mice developed severe symptoms with widespread viral RNA detection in the blood, brain, spleen, liver and ovaries. Histological changes were also striking in these animals. 129Sv/Ev mice developed no clinical symptoms or histological changes, despite viral RNA being detectable in the blood, spleen and ovaries, albeit at lower levels than those seen in A129 mice. Our results identify A129 mice as being highly susceptible to ZIKV and thus A129 mice represent a suitable, and urgently required, small animal model for the testing of vaccines and antivirals.
In December 2015, Puerto Rico became the first U.S. jurisdiction to report local transmission of Zika virus, with the index patient reporting symptom onset on November 23, 2015 (4). This report provides an update to the epidemiology of and public health response to ongoing Zika virus transmission in Puerto Rico. During November 1, 2015-April 14, 2016, a total of 6,157 specimens from suspected Zika virus-infected patients were evaluated by the Puerto Rico Department of Health (PRDH) and CDC Dengue Branch (which is located in San Juan, Puerto Rico), and 683 (11%) had laboratory evidence of current or recent Zika virus infection by one or more tests: reverse transcription-polymerase chain reaction (RT-PCR) or immunoglobulin M (IgM) enzyme-linked immunosorbent assay (ELISA). Zika virus-infected patients resided in 50 (64%) of 78 municipalities in Puerto Rico. Median age was 34 years (range = 35 days-89 years). The most frequently reported signs and symptoms were rash (74%), myalgia (68%), headache (63%), fever (63%), and arthralgia (63%). There were 65 (10%) symptomatic pregnant women who tested positive by RT-PCR or IgM ELISA. A total of 17 (2%) patients required hospitalization, including 5 (1%) patients with suspected Guillain-Barré syndrome. One (<1%) patient died after developing severe thrombocytopenia. The public health response to the outbreak has included increased laboratory capacity to test for Zika virus infection (including blood donor screening), implementation of enhanced surveillance systems, and prevention activities focused on pregnant women. Vector control activities include indoor and outdoor residual spraying and reduction of mosquito breeding environments focused around pregnant women's homes. Residents of and travelers to Puerto Rico should continue to employ mosquito bite avoidance behaviors, take precautions to reduce the risk for sexual transmission (5), and seek medical care for any acute illness with rash or fever.
ZIKV infection was characterized by causing a mild disease presented with fever, headache, rash, arthralgia, and conjunctivitis, with exceptional reports of an association with Guillain-Barre syndrome (GBS) and microcephaly. However, since the end of 2015, an increase in the number of GBS associated cases and an astonishing number of microcephaly in fetus and new-borns in Brazil have been related to ZIKV infection, raising serious worldwide public health concerns. Clarifying such worrisome relationships is, thus, a current unavoidable goal. Here, we extensively review what is currently known about ZIKV, from molecular biology, transmission routes, ecology, and epidemiology, to clinical manifestations, pathogenesis, diagnosis, prophylaxis, and public health.
Number of GBS cases peaked after a lag of 5-9 weeks from the AEI peak. Number of suspected cases of microcephaly peaked after a lag of 30-33 weeks from the AEI peak, which corresponded to time of potential infections of pregnant mothers during the first trimester. These findings support the association of GBS and microcephaly with Zika virus infection and provide evidence for a temporal relationship between timing of arboviral infection of pregnant women during the first trimester and birth outcome.
A total protein concentration increase was shown to be due to even changes in all proteins rather than some specific response, supporting the hypothesis of protein leakage from blood through the blood-nerve barrier. The elevated CSF protein level in AIDP was complemented by activization of protein degradation and much higher peptidome diversity. Due to the studies of the acute motor axonal form, GBS as a whole is thought to be associated with autoimmune response against neurospecific molecules. Thus, in AIDP, autoantibodies against cell adhesion (CAM) proteins localized at Ranvier's nodes were suggested as possible targets in AIDP. Indeed, AIDP CSF peptidome analysis revealed CAM proteins degradation, however no reliable dependence on the corresponding autoantibodies levels was found. Proteome analysis revealed overrepresentation of Gene Ontology groups related to responses to bacteria and virus infections, which were earlier suggested as possible AIDP triggers. Immunoglobulin blood serum analysis against most common neuronal viruses did not reveal any specific pathogen; however AIDP patients were more immunopositive in average and often had polyinfections. Cytokine analysis of both AIDP CSF and blood did not show a systemic adaptive immune response or general inflammation, while innate immunity cytokines were upregulated. To supplement the widely-accepted though still unproven autoimmunity-based AIDP mechanism we propose a hypothesis of the primary PNS damaging initiated as an innate immunity-associated local inflammation following neurotropic viruses egress, while the autoantibody production might be an optional complementary secondary process.
Using EAN, an established GBS model, we investigated the effect of DMF by assessing clinical score, histological staining and electrophysiological studies. Then, we further explored the potential mechanism by Western blot analysis, flow cytometry, fluorescence immunohistochemistry, PCR, and ELISA analysis. The Mann-Whitney U test was used to compare differences between control group and treatment groups where appropriate.
DMF treatment reduced the neurological deficits by ameliorating inflammatory cell infiltration and demyelination of sciatic nerves. In addition, DMF treatment decreased the level of pro-inflammatory M1 macrophages while increasing the number of anti-inflammatory M2 macrophages in the spleens and sciatic nerves of EAN rats. In RAW 264.7, a shift in macrophage polarization from M1 to M2 phenotype was demonstrated to be depended on DMF application. In sciatic nerves, DMF treatment elevated the level of the antioxidant transcription factor nuclear factor erythroid-derived 2-related factor 2 (Nrf2) and its target gene hemoxygenase-1 (HO-1) which could facilitate macrophage polarization toward M2 type. Moreover, DMF improved the inflammatory milieu in spleens of EAN rats, characterized by downregulation of messenger RNA (mRNA) of IFN-γ, TNF-α, IL-6, and IL-17 and upregulation of mRNA level of IL-4 and IL-10.
Taken together, our data demonstrate that DMF can effectively suppress EAN, and the mechanism involves altering the balance of M1/M2 macrophages and attenuating inflammation.