Guillain-Barre Syndrome Publications (7701)
Guillain-Barre Syndrome Publications
Despite the typical clinical and radiological features, intraneural perineurioma remains largely underdiagnosed because of the lack of familiarity with this entity, but also as a result of technical limitations with conventional MRI that is typically performed as a screening test over a large field of view and without contrast sequences. The purpose of this article is to present the pitfalls and pearls learned from years of experience in the diagnosis and management of this relatively rare condition. Clinical suspicion and detailed neurological examination followed by high-quality electrophysiological studies (EPS) must lead to an adequate preimaging localization of the lesion and narrowing of the imaging area. The use of high-resolution (3-T) MRI combined with gadolinium administration will allow adequate visualization of the internal anatomy of the nerve and help in differentiating other causes of neuropathy. In cases where the lesion is not recognized but clinical suspicion is high, possible errors must be assessed, including the EPS localization, area of imaging, MRI resolution, and slice thickness.
On examination he had bilateral clawing with subtle hip flexor weakness and hyporeflexia. His nerve conduction study revealed motor axonal neuropathy. His serum lead levels and autoimmune markers were within normal limits. His cerebrospinal fluid examination revealed albuminocytological dissociation. He was diagnosed as GBS and was given intravenous immunoglobulin. He improved completely over next 8weeks.
GBS is one of the commonest causes of acquired neuropathy in the tropics. In resource limited setting, where electrophysiological facilities may not be available, identification of finger drop sign may help in correct management.
Patients with BFP do not display anti-ganglioside antibodies. Although rare, many physicians are unfamiliar with BFP as bilateral facial weakness is more commonly associated with sarcoidosis, Lyme disease or meningeal pathology. Many patients diagnosed with bilateral Bell's palsy may instead have BFP. In this review, we highlight the clinical features of BFP and outline diagnostic criteria. This article is protected by copyright. All rights reserved.
Emerging evidence has identified IL-22 involvement in neurological diseases and autoimmune disorders such as Guillain-Barré Syndrome (GBS), multiple sclerosis (MS), Alzheimer's disease (AD), encephalitis, inflammatory myopathies, myasthenia gravis (MG), systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), Sjogren's syndrome (SS), psoriasis and Crohn's disease (CD). However, the biological activity of IL-22 is variable resulting in protective or pathogenic effects in different disease states. As such, the development of therapeutic targeting strategies to modify the biological activity of IL-22 is being explored as a promising interventional approach to treat neurological and autoimmune diseases.
This patient's atypical features offer points of discussion regarding the evaluation of patients with acute neuromuscular weakness and emphasize the limitations of the botulism bioassay.
Positive sera (>100 pM) were tested/retested for the VGKC-complex Ab-positive complex proteins LGI1 and CASPR2, screened for binding to live hippocampal neurons, and 12 high-titer sera (>400 pM) tested by radioimmunoassay for binding to VGKC Kv1 subunits with or without intracellular postsynaptic density proteins.
VGKC-complex Abs were found in 39 children, including 20% of encephalopathies and 7.6% of other conditions (p = 0.001). Thirty children had inflammatory conditions and 9 had noninflammatory etiologies but titers >400 pM (n = 12) were found only in inflammatory diseases (p < 0.0001). Four sera, including from 2 children with coexisting NMDA receptor Abs and one with Guillain-Barré syndrome and Abs to both LGI1 and CASPR2, bound to hippocampal neurons. None of the sera bound detectably to VGKC Kv1 subunits on live HEK cells, but 4 of 12 >400 pM sera immunoprecipitated VGKC Kv1 subunits, with or without postsynaptic densities, extracted from transfected cells.
Positive VGKC-complex Abs cannot be taken to indicate a specific clinical syndrome in children, but appear to be a nonspecific biomarker of inflammatory neurologic diseases, particularly of encephalopathy. Some of the Abs may bind to intracellular epitopes on the VGKC subunits, or to the intracellular interacting proteins, but in many the targets remain undefined.
Tailoring the intervention for participants following physiotherapy assessment and incorporating patient preference for type and location of exercise may be important.
The current study is a pragmatic, prospective, parallel observer-blind, randomised controlled trial to evaluate the efficacy and cost-effectiveness of a twelve week tailored home exercise programme versus advice and usual care. Seventy adults with stable immune mediated inflammatory neuropathy IN will be recruited to the study from two main sources: patients attending selected specialist peripheral nerve clinics in the South East and West Midlands of England and people with who access the GAIN charity website or newsletter. Participants will be randomised to receive either advice about exercise and usual care or a 12 week tailored home exercise programme. The primary outcome of activity limitation and secondary outcomes of fatigue, quality of life, self-efficacy, illness beliefs, mood and physical activity will be assessed via self-report questionnaire at baseline, 12 weeks and 12 months post intervention. Cost effectiveness and cost utility will be assessed via interview at baseline and 12 months post intervention. Intention to treat analysis will be our primary model for efficacy analysis. Semi-structured interviews will be conducted with a selected sample of participants in order to explore the acceptability of the intervention and factors affecting adherence to the exercise programme.
This is the first randomised controlled trial to compare the efficacy and cost-effectiveness of tailored home exercise with advice about exercise and usual care for adults with inflammatory neuropathy.
Current Controlled Trials ISRCTN13311697.
Daam2 was elevated in acute presentation in GB syndrome. Reduction occurred with clinical improvement of the patients. With progressive clinical improvement, c-Jun/Krox expression significantly reduced with time. Wnt signaling likely causes immediate early gene activation and transcriptional shutdown of factors critical for formation and maintenance of myelination. Whether the findings of the present study are specific to pathophysiology of demyelination in acute infectious polyradiculopathy and multiple sclerosis or a generalized aspect of demyelinating diseases merits to be examined in future studies.
Therefore, we suspected her as having Guillain-Barré syndrome, and initiated intravenous administration of high-dose immunoglobulin. However, her symptoms progressed gradually and finally she found it difficult to walk. Her urine analysis simultaneously demonstrated albuminuria, and a kidney biopsy indicated focal segmental glomerulosclerosis. At that point, laboratory examination showed high levels of anti SS-A antibody and salivary gland biopsy revealed infiltration of a significant number of lymphocytes around the gland, which led to the diagnosis of Sjögren's syndrome. We considered the etiology of the neural and renal dysfunction as due to the inflammatory mechanism associated with Sjögren's syndrome. Therefore, we administered a second course of immunoglobulin therapy and steroid therapy, which included both pulse and oral administration. Her neurologic symptoms and albuminuria improved rapidly after steroid therapy. The present case indicates that both motor dominant neuropathy and focal segmental glomerulosclerosis can occur in patients with Sjögren's syndrome.
Here, we report four patients with Miller Fisher syndrome and delayed-onset facial palsy. We discuss the possible underlying reasons behind the delay in facial palsy.
Her symptoms completely resolved upon administration of a tailored treatment. This case suggests that puzzling neurological manifestations in patients with a history of AML should be considered as a CNS recurrence and investigated accordingly even in the context of normal imaging findings.
There is insufficient knowledge on the mechanisms of hematogenous leukocyte trafficking into the peripheral nervous system to guide the development of specific molecular therapies for immune-mediated inflammatory neuropathies compared to disorders such as psoriasis, inflammatory bowel disease, rheumatoid arthritis or multiple sclerosis. The recent isolation and characterization of human endoneurial endothelial cells that form the blood-nerve barrier provides an opportunity to elucidate leukocyte-endothelial cell interactions critical to the pathogenesis of inflammatory neuropathies at the interface between the systemic circulation and peripheral nerve endoneurium. This review discusses our current knowledge of the classic pathological features of inflammatory neuropathies, attempts at molecular classification and genetic determinants, the utilization of in vitro and in vivo animal models to determine pathogenic mechanisms at the interface between the systemic circulation and the peripheral nervous system relevant to these disorders and prospects for future potential molecular pathology biomarkers and targets for specific therapeutic intervention.
The classic signs of GBS occurring in the two patients being reported here are muscle weakness, motor and sensory impairment and ascending paralysis with respiratory involvement. The documented cases involved GBS syndrome following oral and maxillofacial surgery in which allogeneic-banked freeze-dried bone have been utilized along with autogenous grafting. There were no incidents of viral infection, vaccination or the other prodromal incidents involved in these cases. It is believed that the description of these two cases would be of interest in that it may stimulate the reporting of similar anecdotal occurrences by other surgeons. Both patients fully recovered from the GBS and are presently alive and well.
Furthermore, the dinical practice guidelinesfor the diagnosis and management of patients were developed, the new drugs were tested and included in these guidelines. The scientists of the biggest Russian neurological centre, Research Centre of Neurology (Moscow), developed a
Serial nerve ultrasound revealed significantly enlarged CSA in median, ulnar and sural nerves within the first 3weeks of disease onset. Longitudinal evaluation revealed an improvement in the nerve CSA with time, reaching significance in the ulnar and sural nerves after 12weeks. There was no significant difference between the demyelinating and axonal subtypes. There was also no significant correlation found between nerve CSA and neurophysiological parameters or changes in nerve CSA and muscle strength.
In GBS, serial studies of peripheral nerve ultrasound CSA are helpful to detect a gradual improvement in the nerve size.
Serial nerve ultrasound studies could serve as a useful tool in demonstrating nerve recovery in GBS.
Both genetic (e.g. HLA-DQB1*0602) and environmental risk factors (e.g. Pandemrix) contribute to the disease development, but the underlying and the mediating immunological mechanisms are largely unknown. Influenza virus hemagglutinin is known to bind gangliosides, which serve as host cell virus receptors. Anti-ganglioside antibodies have previously been linked to various neurological disorders, like the Guillain-Barré syndrome which may develop after infection or vaccination. Because of these links we screened sera of NC patients and controls for IgG anti-ganglioside antibodies against 11 human brain gangliosides (GM1, GM2, GM3, GM4, GD1a, GD1b, GD2, GD3, GT1a, GT1b, GQ1b) and a sulfatide by using a line blot assay. Samples from 173 children and adolescents were analyzed: 48 with Pandemrix-associated NC, 20 with NC without Pandemrix association, 57 Pandemrix-vaccinated and 48 unvaccinated healthy children. We found that patients with Pandemrix-associated NC had more frequently (14.6%) anti-GM3 antibodies than vaccinated healthy controls (3.5%) (P = 0.047). Anti-GM3 antibodies were significantly associated with HLA-DQB1*0602 (P = 0.016) both in vaccinated NC patients and controls. In general, anti-ganglioside antibodies were more frequent in vaccinated (18.1%) than in unvaccinated (7.3%) individuals (P = 0.035). Our data suggest that autoimmunity against GM3 is a feature of Pandemrix-associated NC and that autoantibodies against gangliosides were induced by Pandemrix vaccination.
This study involved 21 GBS patients and an equal number of healthy controls (HCs). The results indicated that the average concentration of IL-35 in the plasma of GBS patients was lower than that of healthy controls (HCs). Increased levels of STAT1, STAT3 and STAT4 proteins and T-bet, ROR γt, IFN-γ and IL-17A mRNA were observed in CD4+ T cells from GBS patients. In contrast, the levels of STAT5 and STAT6 proteins and GATA3, Foxp3, IL-4 and TGF-β1 mRNAs were decreased in GBS patients in comparison with those of HCs. In addition, treatment of CD4+ T cells from GBS patients with IL-35 upregulated IL-35, STAT5 and STAT6 protein and T-bet, GATA3, Foxp3, IFN-γ, IL-4, IL-17A and TGF-β1 mRNA while inhibited levels of STAT3 and STAT4 protein and RORγt and IL-17A mRNA. These results indicate that IL-35 might play a potential role in GBS pathogenesis. Further studies are required in order to evaluate its role in GBS.
Using NUS significant enlargement was found in all measured nerves (P<0.001), except the sural nerve (P=0.086) compared to the controls at onset. The vagus (median 3.0mm(2) vs. 2.0mm(2), P<0.0001) and the cervical spinal nerves were significantly enlarged (median 3.5/4.0mm vs. 2.6/3.2mm, p<0.0001), the vagus most obviously in patients with autonomic dysregulation (AD, 4.0mm(2)). Six months later, NCS showed persisting pathology in CMAP-amplitudes with amelioration of F-wave pathology. NUS showed restitution in the spinal nerves (median 2.6/3.2mm) and the vagus (median 2.0mm(2)) in all patients excluding the vagus in those with persistent AD (median 4.0mm(2)). The peripheral nerves did not change significantly (P>0.05).
Ultrasonographic detection of cervical spinal nerve enlargement supports the diagnosis of GBS in the early phase. Its regression may be a good parameter for the clinical restitution over time. Vagus enlargement may be a risk marker for development of AD.
Ultrasound is a reliable diagnostic follow-up tool in early GBS.
The purpose of this paper is to consider the safety of these two vaccines based on the data from the U.S. Vaccine Adverse Event Reporting System (VAERS) and case reports.
The current HPVv are both effective and generally safe. However, it should be noted that autoimmune side effects have been reported in several studies. Further research should be done to understand the relationship between HPVv and autoimmunity.
PRES in GBS might be an underestimated condition. It should be suspected in GBS patients in the presence of even mild CNS symptoms. A timely PRES diagnosis along with early correction of autonomic system dysfunction in GBS patients is recommended to prevent possible dangerous CNS complications.
In our center, since 2010, we have identified five cases of neuralgic amyotrophy associated with acute hepatitis E in immunocompetent patients. For all these patients, neuralgic amyotrophy was diagnosed with electromyogram and positive IgM for hepatitis E, and detectable HEV RNA in 4 of the cases. Including our patients, we count 26 cases in literature. The mean age of the patients was 44 years old, with a large predominance of males (88%). The disorder is bilateral and asymmetric in 69% of cases. Peripheral nerves other than the brachial plexus were affected in 6 patients (23%). In industrialized countries, any neuralgic amyotrophy, particularly if there is bilateral, asymmetric associated with involvement of nerves outside the brachial plexus, should lead physicians to consider a diagnosis of acute hepatitis E.
We searched PubMed for case-control genetic association studies for HLA-DQB1 polymorphisms (*020x, *030x, *040x, *050x, and *060x) and the risk for GBS. Fixed-effect meta-analytical methods were used for the outcome measure and subgroup analyses. Estimated odds ratios (ORs) and 95% confidence intervals (CIs) were used to investigate the associations between HLA-DQB1 polymorphisms and the risk for GBS. Nine case-control studies involving 780 cases of GBS and 1353 controls were identified in the current study. The meta-analysis demonstrated no significant associations between HLA-DQB1 polymorphisms and the risk for GBS in Asian and Caucasian populations. There were two associations that approached significance: HLA-DQB1*030x in Asian patients (P = 0.07; OR: 0.76, 95% CI: 0.57-1.03) and HLA-DQB1*060x in all patients (P = 0.08; OR: 1.48, 95% CI: 0.96-2.29). Additional studies with larger sample sizes are required to establish a definitive assessment of the contribution of HLA-DQB1 polymorphisms to GBS risk.
Patients were classified into acute inflammatory demyelinating polyneuropathy (AIDP) group, acute motor axonal neuropathy (AMAN) group, Miller-Fisher syndrome (MFS) group, cranial nerve variants(CNV), Bickerstaff's brainstem encephalitis overlaps with GBS (BBE-GBS) group and unclassifiable group based on clinical features and electrophysiological findings. Hughes function grade score (HFGS) was used to assess the prognosis at 3 and 6 months. The prognosis of different subtypes and outcome predictors were analyzed. The most common subtype of GBS was AIDP (57%), followed by AMAN (22%) and MFS (7%). The prognosis of AMAN and BBE-GBS is similar at 3 month(P = 0.0704)and 6 month (P = 0.1614) follow-up. The prognosis of AMAN group was poorer than that of AIDP group at 3 month and 6 month follow-up (P<0.001). Outcome of MFS group and that of CNV group at 6 months were both good(Hughes≤1). Hughes≥3(P<0.0001,OR = 6.650,95%CI = 2.865 to 15.023))and dysautonomia (P = 0.043,OR = 2.820,95%CI = 1.031 to 7.715)) were associated with poor outcome at 6 month follow-up. AIDP is the most common subtype of GBS. Prognosis of AMAN group and BBE-GBS group is poorer than that of AIDP group at 3 month and 6 month follow-up. Hughes≥3 at nadir and dysautonomia are predictors of poor prognosis at 6 month follow-up.
Her blood work revealed marked eosinophilia (>50%), she had purpuric lesions in her legs, and a head magnetic resonance image showed evidence of pansinusitis. Coupled with a history of asthma we suspected EGPA-associated neuropathy and started steroid treatment. The patient showed rapid and significant improvement. ANCAs were later reported positive. ANCA-associated vasculitides present most often as mononeuritis multiplex, but they can mimic GBS and should always be considered in the differential diagnosis, since the treatment strategies for these conditions are radically different.
The gene frequencies for HLA-B Bw4-I were significantly greater in CIDP than HC, but did not differ from GBS. The frequency of the combination of 3DL1/HLA-B Bw4I was greater in CIDP than HC, but did not differ from that of GBS. These data raise the possibility of NK cell function being an important factor in the pathogenesis of CIDP.
jejuni strains from a patient with GBS (ICDCCJ07001), a patient with gastroenteritis (ICDCCJ07002) and a healthy carrier (ICDCCJ07004), which were all associated with the outbreak. The ganglioside-like structures of the lipo-oligosaccharides of these strains were determined by mass spectrometry. Seventeen (53%) of the GBS patients had anti-GT1a IgG antibodies. GT1a mimicry was found in the lipo-oligosaccharides of strain ICDCCJ07002 and ICDCCJ07004; but a combination of GM3/GD3 mimics was observed in ICDCCJ07001, although this patient had anti-GT1a IgG antibodies. A single-base deletion in a glycosyltransferase gene caused the absence of GT1a mimicry in ICDCCJ07001. The phylogenetic tree showed that ICDCCJ07002 and ICDCCJ07004 were genetically closer to each other than to ICDCCJ07001. C. jejuni, bearing a GT1a-like lipo-oligosaccharide, might have caused the GBS outbreak and the loss of GT1a mimicry may have helped ICDCCJ07001 to survive in the host.