Guillain-Barre Syndrome Publications (7608)
Guillain-Barre Syndrome Publications
We further divided our cohort in three subgroups according to the interval between disease onset and NCS (≤4 days; 5-7 days; 8-14 days).
A great proportion of patients (37%) with an early NCS (≤4 days) showed normal neurophysiological results. The most altered parameters were F waves and their proportion increases in correspondence to number of days after onset. Conduction blocks were observed preferentially in upper limbs, in about a third of cases.
This study confirms that NCS may be normal in the early phases of GBS syndrome and suggests to perform an extensive neurophysiological evaluation in these patients.
Our results may help the clinicians in the interpretation of NCS in early-onset GBS.
They were promptly treated with intravenous immunoglobulin, and the first one with plasma exchange additionally. All the patients experienced almost complete clinical recovery. Our experience suggests that primary VZV infection constitutes a GBS triggering event.
Methods We downloaded gene chip datasets pertaining to peripheral leukocyte samples from GBS patients using the Gene Expression Omnibus (GEO) (submitted by Kuo-Hsuan Chang, et al.) and applied hierarchical cluster analysis to detect whether there was a gender difference in genome-wide gene expression levels. Then, we identified the sexually differentially expressed genes using a bioinformatic approach and applied enrichment analysis to the gene ontology (GO) and Kyoto Encyclopedia of Genes and Genomes (KEGG) terms to identify significant pathways related to these genes. Results We observed gender stratification among GBS patients. 20 genes were expressed more highly in male patients and were enriched for functions such as macrophage differentiation, leukocyte migration, bladder cancer, pathogenic E. coli infection. In female patients, 62 genes were more highly expressed and were enriched for responses to viral infection and defense, retinoic acid-inducible gene I (RIG-I)-like receptors, cytoplasmic DNA sensing. Matrix metalloproteinase 9 (MMP9) seem to play an important role in the male predominance of GBS. Conclusions This study demonstrated gender differences in the genome-wide gene expression of patients with GBS. Bioinformatic approaches offer new means for identifying candidate genes and pathways relevant to the pathophysiology of GBS.
At the association between any influenza vaccine - whether seasonal or pandemic - with GBS, the overall risk estimate was 1.41 (95%CI, 1.20-1.66); pandemic vaccines presented a slightly, though non significant, higher risk (RR=1.84; 95%CI, 1.36-2.50) compared to seasonal vaccines (RR=1.22; 95%CI, 1.01-1.48). Pandemic adjuvanted vaccines were not found to be related to a higher risk compared to non-adjuvanted vaccines. The results of the present meta-analysis point to a small but statistically significant association between influenza vaccines, particularly the pandemic ones, and GBS, which is consistent with current explanations upon possible mechanisms for this condition to appear.
Clinical use of IVIG has been supported by guidelines from American Academy of Neurology and European Federation of Neurologic Societies. IVIG is generally recommended for the treatment of Guillain-Barre syndrome and chronic inflammatory demyelinating polyneuropathy in adults, multifocal motor neuropathy and myasthenia gravis, and should be considered as a treatment option for dermatomyositis in adults and Lambert-Eaton myasthenic syndrome. Additional potential indications include stiff person syndrome, multiple sclerosis during pregnancy or while breastfeeding, refractory autoimmune epilepsy, and paraneoplastic disorders. Clinical use of IVIG is mostly safe but few adverse effects may still occur with potentially severe complications, including aseptic meningitis and thromboembolism. In addition to intravenous route (IVIG), subcutaneous immunoglobulins have been used as an alternative treatment option, especially in patients with limited intravenous access. Treatment with IVIG is effective in various autoimmune diseases, but its broader use is constrained by limited supply. This review evaluates the use of immunoglobulins in treatment of neurologic diseases.
Her cranial nerves and respiratory function remained intact. She received intravenous immunoglobulin early on for suspected Guillain-barre syndrome but remained wheelchair-bound until 6 Plasma exchange sessions were completed. After that, she continued to improve with intravenous immunoglobulin dosed every 3-4 weeks. Prominent demyelinating features were found on NCS, with cerebrospinal fluid protein of 415 mg/dL. Comprehensive infectious work-up was negative. Magnetic resonance imaging of lumbosacral and cervical spine showed tumor-like masses mistaken for neurofibromatosis (axial diameter, 7.5-10 mm). Repeated magnetic resonance imaging 6 months later showed persistent nerve root enlargement, despite the patient's improved functional status.
The case throws new light on the pathogenesis of this condition. Macrophage activation is proposed as fundamental; the patient was predisposed by HIV as well as the use of granulocyte colony-stimulating factor and AMAN was then precipitated by a bacterial infection.
The cerebrospinal fluid analysis revealed high cell counts with predominance of lymphocytic cells, raised protein, normal glucose level and no growth of the organisms on culture. The patient improved with supportive care such as intravenous fluids and analgesics without neurological complications. This case emphasizes the importance of recognizing IVIG-associated complications like aseptic meningitis in GBS patients.
Several treatment options exist, including plasma exchange and intravenous immunoglobulin administration. Most cases may resolve without sequelae, but those that do not may leave behind significant persistent debility.
In total, eight patients were identified between January 2007 and December 2010 as having FD accompanied by some features of GBS. These features were subjective sensory symptoms such as distal paresthesia (7/8, 88%), albumin-cytological (A/C) dissociation (7/8, 88%), antecedent infection (6/8, 75%), and minor nerve conduction study (NCS) abnormalities (5/7, 71%). One patient presented with the typical NCS feature of demyelinating neuropathy. Only two patients exhibited areflexia (2/8, 25%). None of the patients possessed any anti-ganglioside antibodies; however, the serum of two patients was positive for anti-mycoplasma antibody (2/6, 33%). FD variant of GBS occurred in less than 1% of our data set. FD can be a regional variant of GBS when it is accompanied by supporting features, such as subjective tingling, A/C dissociation, and minor NCS abnormalities.
Therefore, the mRNA of TLR2, TLR4, myeloid differentiation factor (MyD)88 and nuclear factor (NF)‑κB of peripheral blood mononuclear cells (PBMCs) in patients with GBS and healthy controls was assessed. To confirm the function of TLR2 and TLR4 in the pathogenesis of GBS, PBMCs derived from patients with GBS and healthy controls were cultured with various TLR agonists. The levels of tumor necrosis factor (TNF)‑α and interleukin (IL)‑1β were measured in the culture supernatant and fasting serum was obtained for the detection of anti‑ganglioside antibodies. The results revealed that the mRNA levels of TLR2, TLR4, MyD88 and NF‑κB were significantly increased in patients with GBS compared with those in healthy controls (P=0.003, 0.017, 0.032 and 0.015, respectively). PBMCs from patients with GBS secreted higher levels of TNF‑α and IL‑1β than those from control subjects. The positive rate of immunoglobulin (Ig)G and IgM anti‑ganglioside antibodies in patients with severe GBS was 42.86%, which was markedly higher than rates found in patients with mild GBS (9.09 and 18.18%, respectively). The results of the present study demonstrated that TLR2 and TLR4 are involved in the pathogenesis of GBS and that they and their associated signaling pathways may be targets for the treatment of GBS.
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.
All the patients underwent neurological and electrophysiologic examinations, and were followed up after surgery.
The onset of symptoms about GBS was 2-7 days following the operation. Neurological evaluation showed weaknesses of upper and lower extremities and repeal of tendon reflexes. The patients exhibited typical clinical symptoms and signs of GBS, and electromyographic findings. Lumbar puncture was performed in two cases, and cerebrospinal fluid examination showed albumino-cytological dissociation. All the four patients were diagnosed as GBS based on typical clinical, laboratory and electrophysiological findings. Intravenous immunoglobulin was instituted. At follow-up, one patient needed ventilator support; one patient could transfer from bed to chair; one walked with assistive devices; and the rest one had residual minor neurologic deficits.
These cases warn surgeons to be alert to the association of GBS and spine surgery. Based on our experience, we recommend consideration of this rare diagnosis in patients with paralysis after spine surgery.
L5SNT causes degeneration of a small proportion of fibers that constitute sciatic nerve and its branches, but importantly breaks the blood-nerve barrier, which allows access to circulating Abs and inflammatory cells. Our studies indicate that, in this mouse model, anti-ganglioside Abs induce sequential nodal and axonal injury of intact myelinated nerve fibers, recapitulating pathologic features of human disease. Notably, our results showed that immune complex formation and the activating Fc gamma receptors (FcγRs) were involved in the anti-ganglioside Abs-mediated nodal and axonal injury in this model. These studies provide new evidence that the activating FcγRs-mediated inflammation plays a critical role in anti-ganglioside Abs-induced neuropathy (injury to intact nerve fibers) in GBS.
Severe forms of disease can have cardiac, neurologic or renal involvement. Nervous system complications are unusual and may develop in the early phase of disease or as a delayed complication. Neurological symptoms include headache and alterations of the level of consciousness, and some cases of meningoenchefalitis and Guillain-Barrè syndrome have been also reported. Peripheral nerve involvement is reported only in a limited number of case reports. We describe a case of Rickettsia conorii that was complicated with hearing loss and did not respond to specific treatment. Hearing loss is a rare event, but clinicians should be aware of this complication.
A high titer of anti-GQ1b antibodies was detected. As a result of plasmapheresis, complete recovery was achieved.
The presented case was atypical in its clinical course and treatment. It could support the theory of the continuity between MFS, Bickerstaff brainstem encephalitis (BBE), and Guillain-Barré syndrome (GBS).
For each case, a notification form providing patient and clinical information was provided.
Case definitions were derived from the French drug safety guidelines. Three types of VAE were considered: non-serious, serious and unexpected. Incidence rates were calculated by relating VAEs to the number of vaccine doses delivered.
In total, 161 VAE cases were reported. The overall VAE reporting rate was 24.6 VAEs per 100,000 doses, and the serious VAE rate was 1.3 per 100,000 doses (nine cases). The serious VAEs included two cases of Guillain-Barré syndrome, one case of optic neuritis, one case of a meningeal-like syndrome, one case of rheumatoid purpura, one case of acute asthma and three cases of fainting. The highest rates of VAE were observed with the Bacille Calmette-Guérin vaccine (BCG) (482.3 per 100,000 doses), inactivated diphtheria-tetanus-poliovirus with acellular pertussis vaccine (dTap-IPV) (106.1 per 100,000 doses) and meningococcal quadrivalent glycoconjugate vaccine (MenACWY-CRM) (39.3 per 100,000 doses).
The global rates of VAE observed in 2011 and 2012 confirm the increase that has been observed since 2009 in the French armed forces, which could reflect improved practitioner awareness about VAEs and the use of certain vaccines added to the vaccination schedule recently (dTap-IPV in 2008 and MenACWY-CRM in 2010). VAEs appear to be relatively rare, particularly serious VAEs, which indicates acceptable tolerance of vaccines.
GBS was suspected. Nerve conduction studies demonstrated acute motor-sensory axonal neuropathy including both phrenic nerves. The difficulties with the diagnosis and management of this severe and life-threatening condition are discussed. Significant morbidity is also highlighted. Axonal variants of GBS although rare cause significant morbidity in children. Diagnosis relies solely on accurate neurophysiologic testing and is important because the available treatment options for GBS are frequently ineffective in these variants.
At 3(rd) week of chemotherapy, he developed rapidly progressive ascending motor quadriparesis over 2 days. Clinical and electrophysiology revealed acute motor axonal neuropathy (AMAN) variant of GBS. He was treated with intravenous immunoglobulin (2 g/kg) without discontinuing chemotherapy. Complete recovery took 12 weeks despite immunotherapy, and it was corroborating to slow remission. We concluded that AMAN variant is usually present in B-cell type ALL, may be causal for GBS and it takes 6-16 weeks to complete recovery which may correspond to remission of ALL. However, it needs to be studied. We also present a meta-analysis of previously reported cases of GBS in ALL.
He also presented with raised intracranial pressure with papilledema; then bilateral optic neuritis developed during the later course of illness. Based on the temporal association and exclusion of alternative etiologies, diagnosis of the association between ADEM and GBS was made. Electro-diagnosis (electromyography-nerve conduction velocity) and magnetic resonance imaging study supported our diagnosis. He improved remarkably after treatment with intravenous immunoglobulin and intravenous methylprednisolone.
We herein describe the treatment of a 68-year-old woman who developed progressive quadriplegia 10 days after receiving multiple honeybee venom sting acupuncture treatments. The electrophysiological findings were consistent with Guillain-Barré syndrome (GBS). The temporal relationship between the development of GBS and honeybee venom sting acupuncture is suggestive of a cause-and-effect relationship, although the precise pathophysiology and causative components in honeybee venom need to be verified.
Several biological properties of Cjejuni, e. g. motility and chemotaxis, contribute to the biological fitness of the pathogen. For this, deficiencies in the function of these features clearly reduce the pathogenicity of C. jejuni without being a virulence factor per se. Opposing to this, there are two essential requirements to determine the virulence of C. jejuni which represent the adherence to, and the invasion of host cells. Thereby, adherence, as a virulence factor, is mediated by many different bacterial-derived components like proteins but also by several oligo- and polysaccharide structures that are linked to surface proteins but also to the flagella. In addition, several invasion-relevant features of C. jejuni have been detected so far. Whereas some of them are described functionally to modulate cytoskeleton arrangement of the host cell, others are only described as invasion relevant. Indeed, investigations with respect to the pathogenic potential of some adherence- and invasion-relevant components did not give identical results indicating that their relevance might depend on the interplay of the respective C. jejuni strains used in these studies with the corresponding host cells. This review summarizes the C. jejuni components for adherence to and invasion of host cells together with their particular mode of action if known.
In the evaluable immunogenicity population (N = 216; mean age, 37.8 years), geometric mean fold rises (GMFRs) of immunoglobulin G geometric mean concentrations from baseline to postdose 3 showed significant increases in antibody levels across all PCV13 serotypes (GMFR range, 2.99-23.85; 95% confidence interval lower limit, >1); there were significant declines over the next 6 months, significant increases from predose 4 to postdose 4 (GMFR range, 3.00-6.97), and little change after PPSV23 (GMFR range, 0.86-1.12). Local and systemic reactions were more frequent after dose 4. Six patients experienced serious adverse events possibly related to PCV13 (facial diplegia, injection-site erythema and pyrexia, autoimmune hemolytic anemia, and suspected lack of vaccine efficacy after dose 3 leading to pneumococcal infection), PCV13 and PPSV23 (Guillain-Barré syndrome), or PPSV23 (cellulitis). There were 14 deaths, none related to study vaccines.
A 3-dose PCV13 regimen followed by a booster dose may be required to protect against pneumococcal disease in HSCT recipients. Dose 4 was associated with increased local and systemic reactions, but the overall safety profile of a 4-dose regimen was considered acceptable.
jejuni induces the development of anti-GM1b antibodies in Guillain-Barré syndrome patients.
Mass spectrometry analysis determined the LOS outer core structures, with which mice were immunized. IgG antibodies to single gangliosides and complex of gangliosides were tested in sera from Guillain-Barré syndrome patients from whom C. jejuni LOS had been isolated.
Two isolates from GBS patients who had anti-GM1b antibodies, but neither anti-GM1 nor -GD1a antibodies, expressed both GM1-like and GD1a-like LOSs, but not GM1b-like LOS. Anti-GM1b antibodies were induced in one of the mice immunized with the C. jejuni bearing GM1-like and GD1a-like LOS. Sera from 20 patients had antibodies to the complex of GM1 and GD1a, all of which carried anti-GM1b reactivity. Five of these sera harbored neither anti-GM1 nor anti-GD1a antibodies. IgG antibodies to the complex were absorbed by GM1b, but by neither GM1 nor GD1a.
GM1-like and GD1a-like LOSs form a GM1b epitope, inducing the development of anti-GM1b antibodies in patients with Guillain-Barré syndrome subsequent to C. jejuni enteritis. Here, we present a new paradigm that the complex of two different structures forms a new molecular mimicry, inducing the production of autoantibodies.