Guillain-Barre Syndrome Publications (7392)
Guillain-Barre Syndrome Publications
Levels of anti-neurofascin antibodies were higher in sera from patients with Guillain-Barré syndrome and chronic inflammatory demyelinating polyneuropathy when compared with those of controls. Anti-neurofascin antibodies exacerbated and prolonged adoptive transfer experimental autoimmune neuritis and caused conduction defects when injected intraneurally.
Limited nerve conduction studies suggested possible acute motor-sensory axonal neuropathy, a rare variant of Guillain-Barré syndrome (GBS). Repeat imaging was compatible with polyradiculopathy indicating concomitance of ADEM and GBS. The patient suffered severe motor deficits and neuropathic pain. Slow but significant functional recovery was noted after intensive inpatient rehabilitation followed by continued rehabilitation via home health services.
Deglycosylated, degalactosylated, galactosylated and sialylated IgG were prepared from IVIG following treatment with glycosidases and glycosyltransferases. Sera from patients with Guillain-Barré syndrome, Miller Fisher syndrome and multifocal motor neuropathy associated with anti-ganglioside antibodies were used. Inhibition of complement deposition subsequent to IgG or IgM autoantibody binding to ganglioside, GM1 or GQ1b was assessed on microtiter plates. Sialylated and galactosylated IVIGs more effectively inhibited C3 deposition than original IVIG or enzyme-treated IVIGs (agalactosylated and deglycosylated IVIGs). Therefore, sialylated and galactosylated IVIGs may be more effective than conventional IVIG in the treatment of complement-dependent autoimmune diseases.
We conducted descriptive analyses and clinically reviewed serious reports (i.e., death, hospitalization, prolonged hospitalization, life-threatening illness, and disability) and reports of selected conditions of interest. We used empirical Bayesian data mining to identify adverse events (AEs) that were reported more frequently than expected. We calculated crude AE reporting rates to VAERS by influenza season.
During the study period, VAERS received 1207 LAIV3 reports in adults aged 18-49 years old; 107 (8.9%) were serious, including four death reports. The most commonly reported events were expired drug administered (n=207, 17%), headache (n=192, 16%), and fever (n=133, 11%). The most common diagnostic categories for non-fatal serious reports were neurological (n=40, 39%), cardiovascular (n=14, 14%), and other non-infectious conditions (n=20, 19%). We noted a higher proportion of Guillain-Barré syndrome (GBS) and cardiovascular reports in the Department of Defense (DoD) population compared to the civilian population. Data mining detected disproportional reporting of ataxia (n=15); clinical review revealed that ataxia was a component of diverse clinical entities including GBS.
Review of VAERS reports are reassuring, the only unexpected safety concern for LAIV3 identified was a higher than expected number of GBS reports in the DoD population, which is being investigated. Reports of administration of expired LAIV3 represent administration errors and indicate the need for education, training and screening regarding the approved indications.
We report a case of an 8-year-old male patient with Guillain-Barré syndrome after lung fluke infection. The child had a history of consumption of undercooked crabs. He was diagnosed with paragonimiasis. The patient experienced paralysis of and pain in the lower limbs about 3 weeks after symptom onset. Neurologic and electrophysiologic examination findings supported the diagnosis of Guillain-Barré syndrome. Parasitic infections should also be considered when determining which antecedent infection is associated with Guillain-Barré syndrome.
Persons living in Germany generally acquire hepatitis E infection within the country by consuming infected and undercooked pork; in rare cases, hepatitis E infections are imported from the tropics. HEV can be transmitted via blood products, blood transfusions, and organ transplantation. More than 99% of HEV infections are asymptomatic and self-limiting, but there are also severe cases with acute liver failure. Immunosuppressed persons can develop chronic HEV infection, potentially leading, within a few years, to liver cirrhosis with life-threatening sequelae. Moreover, HEV infection may be associated with extrahepatic manifestations such as Guillain-Barré syndrome. In two retrospectively evaluated case series, ribavirin was found to be active against HEV and can be used to treat either acute or chronic HEV infection.
Hepatitis E must be considered in the differential diagnosis of elevated hepatic enzyme levels and of systemic and neurological conditions of uncertain origin. The infection is usually self-limiting but can take a severe course in immunosuppressed persons. In such cases, ribavirin can be used as an antiviral treatment.
The presence of cerebrospinal fluid albuminocytological dissociation (raised protein, normal cell count), antiganglioside antibodies and neurophysiological evidence of axonal or demyelinating neuropathy also support a diagnosis in many cases, but should not be relied upon. Mimics of GBS and MFS can broadly be divided into those presenting with symmetrical limb weakness and those presenting with brainstem signs. MFS and the pharyngeal-cervical-brachial variant of GBS are frequently mistaken for brainstem stroke, botulism or myasthenia gravis, whereas Bickerstaff's brainstem encephalitis is often diagnosed as Wernicke's encephalopathy. Chameleons or atypical presentations of GBS-related disorders include: paraparetic GBS, bifacial weakness with paraesthesias, acute ataxic neuropathy, acute ophthalmoparesis, acute ptosis and acute mydriasis. Many neurologists may also not be aware that deep tendon reflexes remain present or may even appear brisk in up to 10% of patients with GBS. Correct diagnosis of GBS-related disorders helps to avoid unnecessary investigations and allows early immunotherapy if appropriate.
Other Cochrane systematic reviews have shown that plasma exchange (PE) significantly hastens recovery in GBS compared with supportive treatment alone, and that corticosteroids alone are ineffective.
We had the following four objectives.1. To examine the efficacy of intravenous immunoglobulin (IVIg) in hastening recovery and reducing the long-term morbidity from Guillain-Barré syndrome (GBS).2. To determine the most efficacious dose of IVIg in hastening recovery and reducing the long-term morbidity from GBS.3. To compare the efficacy of IVIg and plasma exchange (PE) or immunoabsorption in hastening recovery and reducing the long-term morbidity from GBS.4. To compare the efficacy of IVIg added to PE with PE alone in hastening recovery and reducing the long-term morbidity from GBS.
We searched the Cochrane Neuromuscular Disease Group Specialized Register (2 December 2013), CENTRAL (2013, Issue 12 in The Cochrane Library), MEDLINE (January 1966 to November 2013) and EMBASE (January 1980 to November 2013). We checked the bibliographies in reports of the randomised trials and contacted the authors and other experts in the field to identify additional published or unpublished data.
Randomised and quasi-randomised trials of IVIg compared with no treatment, placebo treatment, PE, or other immunomodulatory treatments in children and adults with GBS of all degrees of severity. We also included trials in which IVIg was added to another treatment.
Two authors independently selected papers, extracted data and assessed quality. We collected data about adverse events from the included trials.
Twelve trials were found to be eligible for inclusion in this review. Seven trials with a variable risk of bias compared IVIg with PE in 623 severely affected participants. In five trials with 536 participants for whom the outcome was available, the mean difference (MD) of change in a seven-grade disability scale after four weeks was not significantly different between the two treatments: MD of 0.02 of a grade more improvement in the intravenous immunoglobulin than the plasma exchange group; 95% confidence interval (CI) 0.25 to -0.20. There were also no statistically significant differences in the other measures considered. Three studies including a total of 75 children suggested that IVIg significantly hastens recovery compared with supportive care. The primary outcome for this review, available for only one trial with 21 mildly affected children, showed significantly more improvement in disability grade after four weeks with IVIg than supportive treatment alone, MD 1.42, 95% CI 2.57 to 0.27.In one trial involving 249 participants comparing PE followed by IVIg with PE alone, the mean grade improvement was 0.2 (95% CI -0.14 to 0.54) more in the combined treatment group than in the PE alone group; not clinically significantly different, but not excluding the possibility of significant extra benefit. Another trial with 34 participants comparing immunoabsorption followed by IVIg with immunoabsorption alone did not reveal significant extra benefit from the combined treatment.Adverse events were not significantly more frequent with either treatment, but IVIg is significantly much more likely to be completed than PE.Small trials in children showed a trend towards more improvement with high-dose compared with low-dose IVIg, and no significant difference when the standard dose was given over two days rather than five days.
A previous Cochrane review has shown that PE hastens recovery compared with supportive treatment alone. There are no adequate comparisons of IVIg with placebo in adults, but this review provides moderate quality evidence that, in severe disease, IVIg started within two weeks from onset hastens recovery as much as PE. Adverse events were not significantly more frequent with either treatment but IVIg is significantly much more likely to be completed than PE. Also, according to moderate quality evidence, giving IVIg after PE did not confer significant extra benefit. In children, according to low quality evidence, IVIg probably hastens recovery compared with supportive care alone. More research is needed in mild disease and in patients whose treatment starts more than two weeks after onset. Dose-ranging studies are also needed and one is in progress.
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.
Surgery and anesthesia may be triggers for relapse in association with an overall increase in pro-inflammatory cytokines in the postpartum period. We report a unique case of GBS complicating pregnancy in the third trimester followed by a relapse in the postpartum period. She made a good recovery with supportive measures and a repeat course of IVIG during the relapse.
Assessment of fatigue was done by Fatigue Severity Scale (FSS), disability-status by Hughe's Disability Scale (HDS), functional-status by Barthel Index, anxiety/depression by Hospital Anxiety Depression Scale, sleep disturbances by Pittsburgh Sleep Quality Index and muscle weakness by Medical Research Council sum scores.
A total of 90 patients (62 men) with mean age 34 years (95% CI 32.2, 37.7) were included. Median duration of, stay at neurological rehabilitation ward was 30 days, while that of symptoms was 18.5 days. Presence of fatigue at admission (FSS ≥ 4 in 39% patients) was associated with ventilator requirement (P = 0.021) and neuropathic pain (P = 0.03). Presence of fatigue at discharge (FSS ≥ 4 in 12% patients) was associated with disability- HDS (≥3) (P = 0.008), presence of anxiety (P = 0.042) and duration of stay at rehabilitation ward (P = 0.02). Fatigue did not correlate with age, gender, antecedent illness, muscle weakness, depression and sleep disturbances.
Fatigue is prevalent in GBS during early recovery phase of illness. Despite motor recovery fatigue may persist. Knowledge about fatigue as burden of disease in these patients will improve patient care.
MethodsWe investigated the origin of plasminogen in CSF using Alexa Fluor 488¿labelled rat plasminogen injected into rats with systemic inflammation and blood¿CSF barrier dysfunction provoked by lipopolysaccharide (LPS). Near-infrared fluorescence imaging and immunohistochemistry fluorescence microscopy were used to identify plasminogen in brain structures, its concentration and functionality were determined by Western blotting and a chromogenic substrate assay, respectively. In parallel, plasminogen was investigated in CSF from patients with Guillain-Barré syndrome (n¿=¿15), multiple sclerosis (n¿=¿19) and noninflammatory neurological diseases (n¿=¿8).ResultsEndogenous rat plasminogen was detected in higher amounts in the CSF and urine of LPS-treated animals as compared to controls. In LPS-primed rats, circulating Alexa Fluor 488¿labelled rat plasminogen was abundantly localized in the choroid plexus, CSF and urine. Plasminogen in human CSF was higher in Guillain-Barré syndrome (median¿=¿1.28 ng/¿l (interquartile range (IQR)¿=¿0.66 to 1.59)) as compared to multiple sclerosis (median¿=¿0.3 ng/¿l (IQR¿=¿0.16 to 0.61)) and to noninflammatory neurological diseases (median¿=¿0.27 ng/¿l (IQR¿=¿0.18 to 0.35)).ConclusionsOur findings demonstrate that plasminogen is transported from circulating blood into the CSF of rats via the choroid plexus during inflammation. Our data suggest that a similar mechanism may explain the high CSF concentrations of plasminogen detected in patients with inflammation-derived CSF barrier impairment.
Encephalopathy is the most common reported complication. In endemic regions, dengue infection should be considered as one of the aetiologies of encephalitis. Even for other neurological syndromes like myelitis, myositis, GBS etc., dengue infection should be kept in differential diagnosis and should be ruled out especially so in endemic countries during dengue outbreaks and in cases where the aetiology is uncertain. A high degree of suspicion in endemic areas can help in picking up more cases thereby helping in understanding the true extent of neurological complications in dengue fever. Also knowledge regarding the various neurological complications helps in looking for the warning signs and early diagnosis thereby improving patient outcome.
Five patients required mechanical ventilation, two of them having died 9 and 28days after onset. Upper- and lower-limb nerve US showed abnormal findings in just 8.8% of scanned peripheral nerves. In comparison with 46 aged-matched control subjects, US of the fifth to seventh cervical nerves showed changes in four cases, which consisted of significant nerve enlargement, blurred boundaries of the corresponding ventral rami, or both. Autopsy study in one case demonstrated that pathology, consisting of demyelination and endoneurial inflammatory oedema, mainly involved cervical and lumbar nerves.
In early GBS inflammatory oedema of spinal nerves is a pathogenically relevant feature to understanding the mechanism of ascending paralysis, particularly when conventional electrophysiological studies are normal or not diagnostic.
Findings advocate the use of cervical nerve US in early GBS.
5 year-old boy diagnosed early with positive GD1a and GD1b gangliosides of Guillan-Barre syndrome pharyngeal cervical-Brachial variant, who improved and recovered fully in a short period. This is in contrast to those whose recovery period prolongs in spite of early diagnosis and appropriate treatment and/or those who experience incomplete recovery.
In summary, diagnosis of PCB variant of GBS should be considered in infants with sudden onset bulbar symptoms and muscle weakness, and it should be kept in mind that early diagnosis and appropriate treatment can give successful outcomes.
Neurologic complications including Guillain-Barre syndrome or facial paralysis are reported in the literature as a side effect after rabies immunisation.
Sudden hearing loss was detected in an 11 year-old male patient who had taken the medication for rabies immunisation.
This study presents a case report of sudden hearing loss developing after rabies immunisation - no other aetiological factors were detected and clinical management is discussed in light of the literature.
Many patients continue to suffer from decreased exercise capacity and compromised quality of life for months to years after the acute event. Substantial progress has been made lately in the understanding of the pathophysiology of critical illness polyneuropathy and myopathy. Clinical and ancillary test results should be carefully interpreted to differentiate critical illness polyneuropathy/myopathy from similar weaknesses in this patient population. The present review is aimed at providing the latest knowledge concerning the pathophysiology of critical illness polyneuropathy/myopathy along with relevant clinical, diagnostic, differentiating, and treatment information for this debilitating neurological disease.
However, the most widely accepted hypothesis is that the syndrome is the result of an immune-mediated process. Cardiac surgery may be a trigger for immune-mediated response.
The interviews were transcribed verbatim and analysed using content analysis.
The analysis revealed four categories and an overall theme: 'Striving for balance in everyday life'. The participants described persistent lived body restrictions that affected their arms, legs, and face. Bodily symptoms and loss of energy limited or restricted many everyday activities. In connection with healthcare, both satisfaction and feeling vulnerable in a critical situation were described. Experiences of the recovery process varied. The participants described acceptance and reappraisal of a new life situation despite their limitations, and having gained the knowledge that life can change suddenly. However, they also expressed disappointment following an overly positive prognosis in the early stages, and over a continuous wait for recovery. For some participants life had returned to as before.
The participants experienced limitations in everyday life and decreased functioning in several parts of the body. The recovery process may still be ongoing two years after onset. Rehabilitation intervention with an extended focus on supporting individualized coping processes could facilitate ways to live with persistent disability.
This strain, whose genome is described here, was isolated from the fecal flora of a 53-year-old French Caucasoid woman who had been admitted to intensive care unit for Guillain-Barré syndrome. Collinsella massiliensis is a Gram-positive, obligate anaerobic, non motile and non sporulating bacillus. Here, we describe the features of this organism, together with the complete genome sequence and annotation. The genome is 2,319,586 bp long (1 chromosome, no plasmid), exhibits a G+C content of 65.8% and contains 2,003 protein-coding and 54 RNA genes, including 1 rRNA operon.
A cohort of 69 adult patients with GBS (63.8% men) was identified giving an annual incidence of 1.82/100 000. Of these, four (5.8%) had experienced a surgical procedure during the preceding 6 weeks with a relative risk of 6.28 (95% confidence interval 4.15-9.47, P < 0.001) compared with the general study population or a risk of 1.25/100 000 operations. No difference between genders was found. Only two (2.9%) patients had received a vaccination [one against seasonal influenza (P = 0.888) and one against pandemic influenza (Pandemrix(®) , GlaxoSmithKline Biologicals, Rixensart, Belgium, relative risk 2.85, 95% confidence interval 1.27-6.38, P = 0.011)] during the preceding 6 weeks. The most common GBS triggers identified were respiratory tract infections in 30 cases (43.5%) and gastroenteritis in 16 cases (23.2%) whilst two patients (2.9%) had had both.
The overall incidence of GBS in the adult population of southwestern Finland was similar to previous studies worldwide and the most common triggers were respiratory tract infections and gastroenteritis. Surgery was a rare risk factor and of vaccinations only the one against pandemic influenza raised the risk of GBS.
7%, though incidence in other transplants is not well known. We present the first description of tacrolimus associated GBS in lung transplant recipients in the English language literature. The pathophysiology of tacrolimus-induced polyneuropathy is not known, but some have hypothesized that tacrolimus induces an inflammatory phenomenon by differential effects on T cell subsets. Diagnosis of association may be challenging and requires high index of suspicion. The optimal treatment of GBS-associated with tacrolimus after lung transplantation is unknown, although drug discontinuation may result in improvement in some patients, while some reports suggest that the use of IVIG and/or plasmapheresis may be helpful and safe in organ transplant recipients with severe symptoms.
The neurological manifestations seen in dengue are encephalitis, meningitis, encephalopathy, stroke and Guillain-Barré syndrome. Dengue encephalitis is a rare disease. We report an interesting case of dengue encephalitis from Southern India. A 49-year-old gentleman presented with fever, altered sensorium and seizures. Dengue NS-1 antigen test was reactive. Dengue IgM was also positive. CSF PCR was negative for herpes simplex 1 & 2. Dengue encephalitis should be considered in the differential diagnosis of fever with altered sensorium, especially in countries like India where dengue is rampant.
Raynaud's phenomenon, although had been described in relation to many disease conditions, has not been described in association with Guillain-Barré syndrome up to date.
We report the first case of Guillain-Barré syndrome presenting with Raynaud's phenomenon in a 21-year-old previously well boy. New onset Raynaud's phenomenon was experienced followed by acute ascending flaccid paralysis of lower limbs and upper limbs together with palpitations and postural giddiness. Nerve conduction studies showed acute inflammatory demyelinating polyneuropathy with cerebrospinal fluid cyto-protein dissociation. He was treated with intravenous immunoglobulin and showed a satisfactory clinical recovery of muscle weakness, Raynaud's phenomenon and autonomic disturbances.
Guillain-Barré syndrome presenting with Raynaud's phenomenon is not being reported in literature previously. Although the underlying mechanism is not fully understood, Raynaud's phenomenon should prompt the physician to consider Guillain-Barré syndrome with a complimentary clinical picture.
Moreover, we listed up other potential antigens, which may become helpful biomarkers for acquired, dysimmune demyelinating neuropathies based on their critical functions during myelination and their implications in hereditary demyelinating neuropathies.
These glycolipids were GM1, GM2, GD1a, GD1b, GQ1b, crude gangliosides, and 3-O-sulfo-β-d-galactosylceramide C24:1 (designated as C24:1). A direct interaction between ligand and receptor was examined using an ELISA-based binding assay. C-type lectin (CLEC5a, SIGNR3) and immunoglobulin-like receptors (TREM2, TREM3, LMIR2, LMIR5, LMIR7, LMIR8) interacted with C24:1. In addition, TREM3 did bind to GQ1b. LMIR5 interacted with GD1a, GQ1b, and crude gangliosides. Binding with highest affinity was observed for the LMIR5-C24:1 interaction, which was selected for further verification. C24:1 was found to induce MCP-1 production, but not proinflammatory cytokines, in basophils. C24:1-induced MCP-1 production was significantly reduced in DAP12(-/-) basophils. Importantly, LMIR5 ligation by C24:1 resulted in NFAT activation through DAP12 in LMIR5-expressing reporter cells. Structural analysis showed that LMIR5 recognized the 3-O-sulfo-β-d-galactose moiety of C24:1. The findings indicated that C24:1 is a potential ligand for DAP12-coupled LMIR5.
We report here that C. jejuni targets C-type lectin (SIGNR1, SIGNR3) and immunoglobulin-like receptors (TREM2, TREM3, LMIR5, LMIR8). Among these, C. jejuni interacted preferentially with LMIR5, which was selected for further verification using reporter cells. LMIR5 ligation by C. jejuni activated transcriptional factor NFAT through adaptor protein DAP12. Furthermore, LMIR5 activators were identified as protein components, RNA-associated proteins, and 150-kDa high-molecular-weight glycoconjugates. This finding discloses potential receptors that might link C. jejuni to immunopathology.
Female patient, 30 years old, 38 weeks' pregnant, diagnosed with fetal death that occurred about a day, and with SGB. Cesarean section was performed under general anesthesia, progressing without complications perioperatively.
Although it is uncommon, GBS can affect pregnant women and the anesthesiologist may encounter such patients in his (her) daily practice. It is important to understand the peculiarities of GBS to adequately address the patient in the perioperative period, contributing to its better evolution.