Guillain-Barré Syndrome Research Articles

Reversible Conduction Failure in Guillain-Barré Syndrome

Gangliosides are the primary antigenic target in acute motor axonal neuropathy. When anti-ganglioside antibodies bind to gangliosides near the motor axon node of Ranvier, complement system activation and Wallerian degeneration occur. Nevertheless, debate persists regarding the impact of anti-ganglioside antibodies on Guillain-Barré syndrome (GBS). Certain patients with these antibodies experience a swift recovery or exhibit conduction abnormalities indicative of demyelination in nerve conduction studies. The concept of reversible conduction failure was introduced by Kuwabara et al. in 1998. They proposed that this could result from compromised physiological conduction at the node of Ranvier. Auto-antibodies that bind to GM1 or GD1a gangliosides at this node can activate the complement system and disrupt sodium channel and axo-glial junctions, causing conduction failure. In 2003, Cappaso et al. described two cases of rapidly improving flaccid paralysis following Campylobacter jejuni infection. Initial nerve conduction studies indicated motor conduction block, which resolved quickly within 2 to 5 weeks. The authors termed this phenomenon acute motor conduction block neuropathy and considered it a form of arrested or partial acute motor axonal neuropathy. Since acute motor conduction block neuropathy could be misclassified as acute inflammatory demyelinating polyneuropathy based on existing electrophysiological criteria, several suggestions were made to refine the classification of GBS subtypes.

Acute Ascending Paralysis: Time Limited Diagnostic Perplexity

Ascending paralysis is a rare neurological manifestation characterized by progressive weakness that starts in the lower extremities and ascends towards the upper body. GuillainBarré Syndrome (GBS) is a common cause of ascending paralysis and is typically preceded by an infection. We present two case reports, who presented with ascending paralysis, which was initially suspected to be GBS. However, further evaluation revealed an underlying neuropathy contributing to the clinical presentation with a vasculitic etiology. This case highlights the importance of considering alternative etiologies when evaluating patients with ascending paralysis, as over lapping clinical features between GBS and neuropathies can present diagnostic challenges. Early recognition and prompt initiation of appropriate treatment are crucial for favorable patient outcomes.

An interesting case of post dengue Guillain Barre syndrome

Neurological manifestations of dengue fever are seen in around 0.5-6% patients. Post dengue Gullain Barre Syndrome (GBS) is uncommon and only few cases of GBS have been causally linked to serologically confirmed dengue illness. We report a case of 51-year-old male with acute onset flaccid paralysis of all 4 limbs within 1 week of dengue fever, which worsened rapidly despite early initiation of IVIG. Patient became quadriplegic with bilateral LMN facial paralysis, needing intubation and ventilation. When patient did not improve even after 3 weeks of IVIG therapy, plasmapheresis was started and 5 exchanges were given over 10 days following which patient showed significant recovery and became ambulatory and independent at 6 months. In our case 2 dose of IVIG was not considered as his serum IgG levels were raised.Role of plasmapheresis in patients of GBS, responding poorly to IVIG needs further evaluation.GBS is an uncommon neurological manifestation of dengue fever. Role of plasmapheresis in patients who respond poorly to IVIG needs to be considered.

Insights into Zika Virus Pathogenesis and Potential Therapeutic Strategies.

Zika virus (ZIKV) has emerged as a significant public health threat, reaching pandemic levels in 2016. Human infection with ZIKV can manifest as either asymptomatic or as an acute illness characterized by symptoms such as fever and headache. Moreover, it has been associated with severe neurological complications in adults, including Guillain-Barre syndrome, and devastating fetal abnormalities, like microcephaly. The primary mode of transmission is through Aedes spp. mosquitoes, and with half of the world's population residing in regions where Aedes aegypti, the principal vector, thrives, the reemergence of ZIKV remains a concern. This comprehensive review provides insights into the pathogenesis of ZIKV and highlights the key cellular pathways activated upon ZIKV infection. Additionally, we explore the potential of utilizing microRNAs (miRNAs) and phytocompounds as promising strategies to combat ZIKV infection.

COVİD-19 Enfeksiyonu Sırasında Gelişen Bell Palsi

COVID-19 is an infectious disease caused by a newly discovered coronavirus (SARS-COV-2) that can cause a broad spectrum of symptoms, from asymptomatic to severe respiratory failure. Although the virus mainly affects the respiratory system, it is also known to affect many other systems. Myalgia, hyposmia, anosmia, and Guillain-Barre Syndrome are the most common manifestations due to the peripheral nervous system being affected by the virus. Bell's palsy is a unilateral and sudden facial nerve paralysis. Viral infections are most frequently blamed for etiopathogenesis. Bell palsy due to COVID-19 infection has been reported in the literature as a rare complication. In this case, we wanted to emphasize that Bell's palsy should be kept in mind among the neurological complications that may develop due to COVID-in in a patient who developed Bell's palsy after being diagnosed with COVID-19.

Electroencephalographic abnormalities in children with type 1 diabetes mellitus: a prospective study.

The aim herein was to investigate epileptiform discharges on electroencephalogram (EEG), their correlation with glutamic acid decarboxylase 65 autoantibody (GAD-ab) in newly diagnosed pediatric type 1 diabetes mellitus (T1DM) patients and interpret their medium-term utility in predicting epilepsy. Children presenting with T1DM between July 2018 and December 2019 were included in this prospective longitudinal study. Patients with a history of head injury, chronic illness, neurological disorder, seizure, autism, or encephalopathy were excluded. EEGs were obtained within the first 7 days of diagnosis and later reviewed by a pediatric neurologist. All of the children were clinically followed-up in pediatric endocrinology and neurology clinics for 2 years after their diagnosis. A total of 105 children (46 male, 43.8%) were included. The mean age at the time of diagnosis was 9.6 ± 4.1 years (range: 11 months-17.5 years). At the time of admission, 24 (22.9%), 29 (27.6%), and 52 (49.5%) patients had hyperglycemia, ketosis, and diabetic ketoacidosis, respectively. GAD-ab was positive in 55 children (52.4%). No background or sleep architecture abnormalities or focal slowing were present on the EEGs. Of the patients, 3 (2.9%) had focal epileptiform discharges. The mean GAD-ab levels of the remaining 102 patients were 7.48 ± 11.97 U/mL (range: 0.01-50.54) (p = 0.2). All 3 children with EEG abnormality had higher levels of GAD-ab (3.59 U/mL, 31.3 U/mL, and 7.09 U/mL, respectively). None of the patients developed epilepsy during the follow-up, although 1 patient experienced Guillain-Barré syndrome (GBS). The prevalence of epileptiform discharges in the patients was similar to those of previous studies, in which healthy children were also included. No relationship was found between the epileptiform discharges and GAD-ab, and none of the patients manifested seizures during the first 2 years of follow-up of T1DM. These data support the findings of previous studies reporting that T1DM patients with confirmed electroencephalographic abnormalities do not have an increased risk of epilepsy. On the other hand, GBS might be considered as another autoimmune disease that may be associated with T1DM in children.

Neuromuscular diseases associated with COVID-19 vaccines: a systematic review and pooled analysis of 258 patients

BackgroundNeuromuscular diseases (NMD) emerged as one of the main side effects of the COVID-19 vaccination. We pooled and summarized the evidence on the clinical features and outcomes of NMD associated with COVID-19 vaccination.MethodsWe comprehensively searched three databases, Medline, Embase, and Scopus, using the key terms covering “Neuromuscular disease” AND “COVID-19 vaccine”, and pooled the individual patient data extracted from the included studies.ResultsA total of 258 NMD cases following COVID-19 have been reported globally, of which 171 cases were Guillain-Barré syndrome (GBS), 40 Parsonage-Turner syndrome (PTS), 22 Myasthenia Gravis (MG), 19 facial nerve palsy (FNP), 5 single fiber neuropathy, and 1 Tolosa-Hunt syndrome. All (100%) SFN patients and 58% of FNP patients were female; in the remaining NMDs, patients were predominantly male, including MG (82%), GBS (63%), and PTS (62.5%).The median time from vaccine to symptom was less than 2 weeks in all groups. Symptoms mainly appeared following the first dose of vector vaccine, but there was no specific pattern for mRNA-based.ConclusionCOVID-19 vaccines might induce some NMDs, mainly in adults. The age distribution and gender characteristics of affected patients may differ based on the NMD type. About two-thirds of the cases probably occur less than 2 weeks after vaccination.

Bickerstaff brainstem encephalitis, an uncommon presentation in a child: a case report

BackgroundThe typical clinical and radiological presentation of Bickerstaff brainstem encephalitis (BBE) has been highlighted in this case report.Case presentationBickerstaff encephalitis is a rare autoimmune inflammatory disorder and is considered a subtype of Guillain–Barré syndrome (GBS) along with Miller Fisher syndrome. The diagnosis of BBE is largely clinical, though laboratory tests and imaging can be of supportive value. We report a case of a 5-year-old child who presented with a classical clinical triad of BBE with characteristic magnetic resonance imaging (MRI) findings.ConclusionsBBE is a rare disease with very few cases being reported with typical clinical and radiological findings. Hence, we reported a typical case of BBE to make an addition to the available literature.

Seroprevalence of Campylobacter jejuni infection in common subtypes of Guillain–Barre syndrome in Kashmiri population

BackgroundGuillain–Barre Syndrome (GBS) is a common differential diagnosis of acute-onset flaccid quadriparesis with or without bulbar involvement. Various illnesses precede GBS, respiratory illness being the most common. Campylobacter jejuni is the single most common organism found associated with GBS. The aim of the present study was to determine the prevalence of Campylobacter jejuni positivity in different subtypes of GBS.MethodsSixty patients of GBS were tested for serological evidence of Campylobacter jejuni and compared with 60 age and sex matched controls.ResultsMean age of cases was 39.58 (± 14.76 years) and that of controls was 35 (± 12.31 years). Preceding illness was present in 38% cases. Respiratory tract illness was present in 9 (15%) cases, while as GI illness was present in 8 (13%) cases. AIDP was the most common variant accounting for 65% of cases, followed by AMAN (18.3%). Among cases, 24 (40%) tested positive for Campylobacter jejuni antibody whereas only 12 (20%) tested positive for antibody among controls. The difference was statistically significant between cases and controls (p value = 0.003). There was no statistically significant difference in antibody positivity and preceding illness among different variants of GBS (p value = 1.0).ConclusionCampylobacter jejuni infection is a frequent preceding illness in GBS patients, although it may be asymptomatic. There is however no relation between different subtypes of GBS and C. jejuni infection.

The analysis of serum lipids profile in Guillain-Barre syndrome.

Guillain-Barre syndrome (GBS) is an immune-mediated inflammatory peripheral neuropathy. This study aimed to conduct a systematic analysis of the serum lipids profile in GBS. We measured the serum lipids profile in 85 GBS patients and compared it with that of 85 healthy controls matched for age and sex. Additionally, we analyzed the correlation between lipids and the severity, subtypes, precursor infections, clinical outcomes, clinical symptoms, immunotherapy, and other laboratory markers of GBS. Compared to the healthy controls, GBS exhibited significantly elevated levels of Apolipoprotein B (APOB), Apolipoprotein C2 (APOC2), Apolipoprotein C3 (APOC3), Apolipoprotein E (APOE), triglycerides (TG), and residual cholesterol (RC). Conversely, Apolipoprotein A1 (APOA1), Apolipoprotein A2 (APOA2), and high-density lipoprotein (HDL) were substantially lower in GBS. Severe GBS displayed noticeably higher levels of APOC3 and total cholesterol (TC) compared to those with mild disease. Regarding different clinical outcomes, readmitted GBS demonstrated higher RC expression than those who were not readmitted. Moreover, GBS who tested positive for neuro-virus antibody IGG in cerebrospinal fluid (CSF) exhibited heightened expression of APOC3 in comparison to those who tested negative. GBS with cranial nerve damage showed significantly reduced expression of HDL and APOA1 than those without such damage. Additionally, GBS experiencing limb pain demonstrated markedly decreased HDL expression. Patients showed a significant reduction in TC after intravenous immunoglobulin therapy. We observed a significant positive correlation between lipids and inflammatory markers, including TNF-α, IL-1β, erythrocyte sedimentation rate (ESR), white blood cells, monocytes, and neutrophils in GBS. Notably, APOA1 exhibited a negative correlation with ESR. Furthermore, our findings suggest a potential association between lipids and the immune status of GBS. The research demonstrated a strong connection between lipids and the severity, subtypes, clinical outcomes, precursor infections, clinical symptoms, immunotherapy, inflammation, and immune status of GBS. This implies that a low-fat diet or the use of lipid-lowering medications may potentially serve as an approach for managing GBS, offering a fresh viewpoint for clinical treatment of this condition.

CLINICAL OUTCOME OF GUILLAIN BARRE SYNDROME IN THE INTENSIVE CARE UNIT OF A PEDIATRIC TERTIARY CARE HOSPITAL

Since poliomyelitis was eradicated, Guillain-Barre syndrome has been the leading cause of acute flaccid paralysis globally. Critical care and mechanical breathing may be necessary in extreme circumstances. Among paediatric patients hospitalized in the intensive care unit of a Karachi tertiary hospital, we aimed to quantify the incidence of Guillain-Barré Syndrome clinical outcomes. Cardiology Intensive Care Unit (NICH) in Karachi, Pakistan. January 9, 2020, to July 10, 2020—six months. Children admitted to the intensive care unit at NICH with GBS, as defined operationally, ranging in age from six months to twelve years, were included in the study. They stimulated the posterior tibial nerve (PTN) and common peroneal nerve (CPN), allowing for studying motor nerve conduction and using the median and ulnar nerves to measure compound muscle action potentials' onset delay, amplitude and conduction velocity (CMAPs). Research on the conduction of sensory nerves was carried out to quantify SNAPs in the sural, median, and ulnar regions. All patients were given intravenous immunoglobulin for five days at a 400 mg/kg/day dosage, as per the protocol. Clinical outcomes were evaluated using the HFG scale at discharge and four weeks. The average patient age was 5.81 ± 3.26 years out of 30. Thirteen men (43.3%) and seventeen females (56.7%) were present. Fifteen patients (53.3%) had a history of upper respiratory illness, while seven (23.3%) had a history of gastrointestinal infection. A mean HFG score of 3.03 ±1.90 was recorded. Outcomes were good for 21 patients (56.7%) and deficient for 9 (43.3%). A tertiary care hospital in Karachi reported that 56.7% of children admitted with Guillain-Barre Syndrome had good clinical results in the intensive care unit.

Biological analysis of the potential pathogenic mechanisms of Infectious COVID-19 and Guillain-Barré syndrome.

Guillain-Barré syndrome (GBS) is a medical condition characterized by the immune system of the body attacking the peripheral nerves, including those in the spinal nerve roots, peripheral nerves, and cranial nerves. It can cause limb weakness, abnormal sensations, and facial nerve paralysis. Some studies have reported clinical cases associated with the severe coronavirus disease 2019 (COVID-19) and GBS, but how COVID-19 affects GBS is unclear. We utilized bioinformatics techniques to explore the potential genetic connection between COVID-19 and GBS. Differential expression of genes (DEGs) related to COVID-19 and GBS was collected from the Gene Expression Omnibus (GEO) database. By taking the intersection, we obtained shared DEGs for COVID-19 and GBS. Subsequently, we utilized bioinformatics analysis tools to analyze common DEGs, conducting functional enrichment analysis and constructing Protein-protein interaction networks (PPI), Transcription factors (TF) -gene networks, and TF-miRNA networks. Finally, we validated our findings by constructing the Receiver Operating Characteristic (ROC) curves. This study utilizes bioinformatics tools for the first time to investigate the close genetic relationship between COVID-19 and GBS. CAMP, LTF, DEFA1B, SAMD9, GBP1, DDX60, DEFA4, and OAS3 are identified as the most significant interacting genes between COVID-19 and GBS. In addition, the signaling pathway of NOD-like receptors is believed to be essential in the link between COVID-19 and GBS.

Synergistic effects of immune checkpoints and checkpoint inhibitors in inflammatory neuropathies: Implications and mechanisms.

Immune checkpoint molecules play pivotal roles in the regulation of immune homeostasis. Disruption of the immune checkpoints causes autoimmune/inflammatory as well as malignant disorders. Over the past few years, the immune checkpoint molecules with inhibitory function emerged as potential therapeutic targets in oncological conditions. The inhibition of the function of these molecules by using immune checkpoint inhibitors (ICIs) has brought paradigmatic changes in cancer therapy due to their remarkable clinical benefits, not only in improving the quality of life but also in prolonging the survival time of cancer patients. Unfortunately, the ICIs soon turned out to be a "double-edged sword" as the use of ICIs caused multiple immune-related adverse effects (irAEs). The development of inflammatory neuropathies such as Guillain-Barré syndrome (GBS) and Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) as the secondary effects of immunotherapy appeared very challenging as these conditions result in significant and often permanent disability. The underlying mechanism(s) through which ICIs trigger inflammatory neuropathies are currently not known. Compelling evidence suggests autoimmune reaction and/or inflammation as the independent risk mechanism of inflammatory neuropathies. There is a lack of understanding as to whether prior exposure to the risk factors of inflammatory neuropathies, the presence of germline genetic variants in immune function-related genes, genetic variations within immune checkpoint molecules, the existence of autoantibodies, and activated/memory T cells act as determining factors for ICI-induced inflammatory neuropathies. Herein, we highlight the available pieces of evidence, discuss the mechanistic basis, and propose a few testable hypotheses on inflammatory neuropathies as irAEs of immunotherapy.

Immune thrombocytopenic purpura and Guillain-Barré syndrome after 23-valent pneumococcal polysaccharide vaccination in Japan: The vaccine effectiveness, networking, and universal safety (VENUS) study

BackgroundTo address the lack of an active vaccine safety surveillance system in Japan, the Vaccine Effectiveness, Networking, and Universal Safety (VENUS) study was initiated in 2021 as a pilot system using existing health insurance claims data and vaccination records. MethodsThis study evaluated the value of the VENUS study by assessing the incidence of immune thrombocytopenic purpura (ITP) and Guillain-Barré syndrome (GBS) following vaccination with the 23-valent pneumococcal polysaccharide vaccine (PPSV23) using a self-controlled case series (SCCS) design. ResultsIncidence rate ratios for ITP during 28-day and 42-day risk periods were 0.89 (95% confidence interval [CI], 0.12–6.4), and 0.58 (95% CI, 0.081–4.2), respectively. Neither was statistically significant. Incidence rate ratios could not be estimated for GBS due to the limited sample size. ConclusionThe VENUS study can provide valuable insights to facilitate the establishment of an advanced vaccine monitoring system in Japan.

Evidence-Based Application of the Plasma Exchange to Improve Muscle Power and Disability Grade in Patients with Guillain-Barré Syndrome

<p>格林-巴利症候群是罕見且病程進展快速的急性周邊神經系統病變，未及時治療，將導致四肢癱瘓及呼吸衰竭。本文描述一位71歲男性，接種AstraZeneca（AZ）疫苗後，出現對稱性肢體無力及右側顏面麻痺，經檢查確診為AZ疫苗誘發格林-巴利症候群的急性發炎性脫髓鞘多發性神經病變合併右側貝爾氏麻痺。筆者以神經科專科護理師角度，評估病人與家屬對治療方案之選擇呈現意見分歧，故運用實證方法探討血漿置換術對改善肌肉力量與殘疾程度之成效。 於Cochrane、Embase、MEDLINE、PubMed、 CINAHL及華藝等資料庫，使用“Guillain-Barre Syndrome”、“plasma exchange”、“intravenous immunoglobulin”、“muscle power”及“disability grade”之關鍵字，搜尋2013 年1月至2023 年2月之中英文文獻。經篩選後共計納入3篇研究進行分析，使用OCEBM評讀文獻，結果顯示治療第2週後，接受血漿置換術之介入措施對於殘疾等級和肌肉力量之改善高於靜脈注射免疫球蛋白。本實證應用結果，病人於住院期間確實恢復肌肉力量及改善殘疾程度。希冀藉由此案例分享，做為爾後照護之參考。</p> <p> </p><p>Guillain-Barré syndrome is a rare and rapidly progressing disease characterized by acute peripheral neuropathy. If left untreated, it can lead to quadriplegia and respiratory failure. In this case, a 71-year-old male developed symmetric limb weakness and right facial palsy after receiving the AstraZeneca (AZ) vaccine. The case was diagnosed with acute inflammatory demyelinating polyneuropathy, a form of Guillain-Barré syndrome, along with right Bell’s palsy. The author, a neurological nurse practi-tioner, was trying to resolve the differences in treatment preferences between the patient and his fami-ly members. Therefore, an evidence-based approach was employed to explore the effectiveness of plas-ma exchange in improving muscle strength and disability grade. An electronic literature search was conducted in databases including Cochrane, Embase, MEDLINE, PubMed, CINAHL, and Airiti Li-brary. Keywords such as “Guillain-Barré Syndrome,” “Plasma Exchange,” “Intravenous Immunoglob-ulin,” “Muscle Power,” and “Disability Grade” were applied. Studies in Chinese or English published between January 2013 and February 2023 were screened and three relevant studies were included in the analysis. The Oxford Centre for Evidence-Based Medicine criteria were further applied to review the literature. The results showed that plasma exchange intervention resulted in a greater improvement in disability grade and muscle power compared to intravenous immunoglobulin after the second week of treatment. Empirical evidence indicated that the patient indeed regained muscle strength and im-proved their disability grade during hospitalization. This case is shared as a reference for future care considerations.</p> <p> </p>

A case report: Guillain Barre syndrome and non-cortical peripheral myoclonus

A case report: Guillain Barre syndrome and non-cortical peripheral myoclonus

The assessment and interpretation of published data on Guillain–Barre syndrome after SARS‐CoV‐2 vaccination requires care

The assessment and interpretation of published data on Guillain–Barre syndrome after <scp>SARS‐CoV</scp>‐2 vaccination requires care

Probability of sequelae following Campylobacter spp. infections: Update of systematic reviews and meta‐analyses

AbstractIntroductionReactive arthritis (REA) and Guillain–Barré syndrome (GBS) are postinfectious complications of Campylobacter enteritis (CE); associations with inflammatory bowel diseases and irritable bowel syndrome (IBS) are also discussed. The objective of this study was to summarize existing evidence on the probability of sequelae following confirmed CE.MethodsAll studies included in previous reviews and meta‐analyses on this topic were retrieved and assessed for eligibility; a systematic literature search was conducted to collect more recent reports. For each sequela, random effects meta‐analyses were performed; the risk of bias and the quality of evidence were evaluated.ResultsIn total, 50 reports of observational studies were included; between 110,765 and 175,839 CE cases were considered for each sequela. The pooled proportion of CE cases that developed a sequela was 1.72% (95% CI 0.81–3.61; prediction interval [PI]: 0.03–47.65) for REA, 0.07% (0.03–0.16; PI: 0.003–1.59) for GBS, 0.22% (0.06–0.73; PI: 0.002–20.69) for Crohn's disease (CD), 0.35% (0.11–1.15; PI: 0.003–28.16) for ulcerative colitis (UC), and 4.48% (1.92–10.08; PI: 0.09–70.62) for IBS. The high between‐study heterogeneity could partially be explained by study size and design, the method of assessing sequelae, and the period between CE and sequelae onset. The quality of evidence was rated as moderate for GBS and UC, and low for REA, CD, and IBS.ConclusionUpdated estimates of the probability to develop sequelae after CE are provided, for CD and UC for the first time. However, uncertainty regarding the true probabilities remains, which is reflected in the broad PIs.

A case report of Guillain Barre syndrome syndrome complicating covid-19 infection

A case report of Guillain Barre syndrome syndrome complicating covid-19 infection

Seasonal incidence of Guillain-Barre Syndrome: A 5 years hospital-based study in Indonesia

Seasonal incidence of Guillain-Barre Syndrome: A 5 years hospital-based study in Indonesia