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Guillain-Barré Syndrome (GBS) is a polyneuropathy characterized by acute-onset and immune-mediated processes, triggered by various factors. The occurence of GBS in connection with snakebites is rare. In this report, we present a rare clinical case with neuro-muscular and hematological complications following a snakebite, leading to flask quadriplegia on the 14th day during intensive care follow-up. Cerebrospinal fluid (CSF) analysis indicated albumin-cytological dissociation, and Electromyography (EMG) revealed findings consistent with acute motor axonal neuropathy, resulting in a diagnosis of GBS. After undergoing plasmapheresis once, the patient swiftly developed refractory septic shock, leading to a fatal outcome. In this study, we report a GBS case as a conseqeunce of snakebite together with a systematic review of the medical literature on the development of GBS after snakebite. Although current case reports and findings have broadened the scope of etiologic hypotheses for GBS including snake envenomation, further studies are needed to confirm the association between GBS following snakebite. [ABSTRACT FROM AUTHOR]

Introduction: Guillain-Barré syndrome (GBS) is a rare disease that affects almost 0.8-1.9 cases per 100,000 people worldwide every year. This is the most prevalent cause of subacute flaccid paralyzing illness today. It is a subacute inflammatory demyelinating polyradiculoneuropathy; the typical scenario involves ascending symmetrical flaccid paralysis, but in some circumstances, sensory, autonomic, and cranial neuropathy may also be involved. Several vaccines have been found to have complications since the previous century. Numerous case reports of GBS in the literature have been reported following COVID-19 vaccines in recent times. Objective: This study aimed to conduct a comprehensive examination of GBS cases that have been reported after COVID-19 vaccines; to analyze the descriptive statistical analysis of data gathered regarding clinical, laboratory, electrophysiological, and radiological characteristics; to discuss, based on the available evidence, whether the disease has a preference for a particular vaccine type; and to speculate on the potential pathogenesis. Methodology: This review has been carried out by recommendations of the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Result: Reviewing 60 case reports illustrated that most of them are from the USA (18.1%) and the majority of affected individuals were males (60%). The results favored the association between vector-based SARS-CoV-2 vaccine, particularly AstraZeneca vaccine, and the GBS. The mean of symptoms onset is 11.4 days. The results of diagnostic tests such as LP are consistent mostly with albumin-cytological dissociation (81.81%), where brain and spine MRI was unremarkable in 59.52%. Regarding electrodiagnostic tests, AIDP is the most common variant (61.81%). The management was not consistent among the case reports. However, IVIG is the most frequent way of treating these patients (68.33%). The functional outcome was documented in 47 patients; 65% improved with medical management. Conclusion: This study aimed to conduct a systematic review of reported cases of GBS following COVID-19 vaccines and descriptive statistical analysis of collected data on clinical, laboratory, electrophysiological, and radiological features, to discuss, based on available results, whether the disease has a predilection to a specific vaccine type and to speculate the potential pathogenesis. [ABSTRACT FROM AUTHOR]

The objective of this review was to analyze the pathophysiological role of endoneurial inflammatory edema in initial stages of classic Guillain-Barré syndrome (GBS), arbitrarily divided into very early GBS (= 4 days after symptom onset) and early GBS (= 10 days). Classic GBS, with variable degree of flaccid and areflexic tetraparesis, encompasses demyelinating and axonal forms. Initial autopsy studies in early GBS have demonstrated that endoneurial inflammatory edema of proximal nerve trunks, particularly spinal nerves, is the outstanding lesion. Variable permeability of the blood-nerve barrier dictates such lesion topography. In proximal nerve trunks possessing epi-perineurium, edema may increase the endoneurial fluid pressure causing ischemic changes. Critical analysis the first pathological description of the axonal form GBS shows a combination of axonal degeneration and demyelination in spinal roots, and pure Wallerian-like degeneration in peripheral nerve trunks. This case might be reclassified as demyelinating GBS with secondary axonal degeneration. Both in acute motor axonal neuropathy and acute motor-sensory axonal neuropathy, Wallerian-like degeneration of motor fibers predominates in the distal part of ventral spinal roots abutting the dura mater, another feature re-emphasizing the pathogenic relevance of this area. Electrophysiological and imaging studies also point to a predominant alteration at the spinal nerve level, which is a hotspot in any early GBS subtype. Serum biomarkers of axonal damage, including neurofilament light chain and peripherin, are increased in the great majority of patients with any early GBS subtype; endoneurial ischemia of proximal nerve trunks could contribute to such axonal damage. It is concluded that inflammatory edema of proximal nerve trunks is an essential pathogenic event in early GBS, which has a tangible impact for accurate approach to the disease. [ABSTRACT FROM AUTHOR]

Background and Aims: In the pathophysiology of Guillain–Barre syndrome (GBS), inflammation and immunity are believed to play a key role. The neutrophil‐lymphocyte ratio (NLR), monocyte‐lymphocyte ratio (MLR), and platelet‐lymphocyte ratio (PLR) have been recently identified as potential markers of inflammation or immunity. This study aimed to investigate whether NLR, MLR, and PLR are associated with GBS characteristics in children. We also assessed the impact of the COVID‐19 pandemic on the characteristics of GBS in Iran. Methods: In this retrospective cross‐sectional study, we reviewed the records of all 150 children diagnosed with GBS in the Children's Medical Center hospital affiliated with Tehran University of Medical Sciences (TUMS) from March 2017 until March 2022. The TUMS research ethics committee approved the study (Ethics code: IR.TUMS.CHMC.REC.1399.125). Patients' data including gender, age, clinical symptoms, laboratory findings, and electrodiagnostic study results were collected and analyzed. Results: This study involved 150 children, comprising 93 boys and 57 girls, with an average age of 7.53 ± 3.75 years. The analysis demonstrated that the number of hospitalization days increased with an increase in NLR (p = 0.025). Moreover, patients with abnormal electrodiagnostic study patterns had a higher risk of intensive care unit (ICU) admission (p: 0.027), although according to binary logistic regression, respiratory failure at admission time was the only significant factor increasing the risk of ICU admission (p = 0.035). The study also found that the pandemic has resulted in a shift from acute inflammatory demyelinating polyneuropathy to acute motor axonal neuropathy as the most common EMG‐NCV pattern in our patients (p < 0.001). Conclusion: We found that higher NLR was associated with a longer hospitalization duration and could potentially distinguish between severe and mild cases of GBS. We have also shown that the COVID‐19 pandemic has changed our patients' most frequent electromyography and nerve conduction velocity (EMG‐NCV) patterns. [ABSTRACT FROM AUTHOR]

Guillain–Barré syndrome is an immune-mediated disease of the peripheral nerves characterized by rapidly progressing symmetric weakness, areflexia, and albuminocytological dissociation. Most patients reach their nadir within 2 weeks. Disease severity can be mild to severe, with 20% of patients requiring mechanical ventilation. Intravenous immunoglobulin and plasma exchange are equally effective treatments. Monitoring strength, respiratory function, blood pressure, and heart rate, as well as pain management and rehabilitative therapy are important aspects of management. About 20% of patients require assistance to walk at 6 months. Older age, preceding diarrhea, and lower Medical Research Council (MRC) sum scores predict poor outcome. Death from cardiovascular and respiratory complications can occur in the acute or recovery phases of the illness in 3 to 7% of the patients. Risk factors for mortality include advanced age and disease severity at onset. Neuropathic pain, weakness, and fatigue can be residual symptoms; risk factors for these include axonal loss, sensory involvement, and severity of illness. [ABSTRACT FROM AUTHOR]

IntroductionGuillain–Barré syndrome (GBS) is a rare disease that affects almost 0.8–1.9 cases per 100,000 people worldwide every year. This is the most prevalent cause of subacute flaccid paralyzing illness today. It is a subacute inflammatory demyelinating polyradiculoneuropathy; the typical scenario involves ascending symmetrical flaccid paralysis, but in some circumstances, sensory, autonomic, and cranial neuropathy may also be involved. Several vaccines have been found to have complications since the previous century. Numerous case reports of GBS in the literature have been reported following COVID-19 vaccines in recent times.ObjectiveThis study aimed to conduct a comprehensive examination of GBS cases that have been reported after COVID-19 vaccines; to analyze the descriptive statistical analysis of data gathered regarding clinical, laboratory, electrophysiological, and radiological characteristics; to discuss, based on the available evidence, whether the disease has a preference for a particular vaccine type; and to speculate on the potential pathogenesis.MethodologyThis review has been carried out by recommendations of the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines.ResultReviewing 60 case reports illustrated that most of them are from the USA (18.1%) and the majority of affected individuals were males (60%). The results favored the association between vector-based SARS-CoV-2 vaccine, particularly AstraZeneca vaccine, and the GBS. The mean of symptoms onset is 11.4 days. The results of diagnostic tests such as LP are consistent mostly with albumin-cytological dissociation (81.81%), where brain and spine MRI was unremarkable in 59.52%. Regarding electrodiagnostic tests, AIDP is the most common variant (61.81%). The management was not consistent among the case reports. However, IVIG is the most frequent way of treating these patients (68.33%). The functional outcome was documented in 47 patients; 65% improved with medical management.ConclusionThis study aimed to conduct a systematic review of reported cases of GBS following COVID-19 vaccines and descriptive statistical analysis of collected data on clinical, laboratory, electrophysiological, and radiological features, to discuss, based on available results, whether the disease has a predilection to a specific vaccine type and to speculate the potential pathogenesis.

Abstract Background and Aims In the pathophysiology of Guillain–Barre syndrome (GBS), inflammation and immunity are believed to play a key role. The neutrophil‐lymphocyte ratio (NLR), monocyte‐lymphocyte ratio (MLR), and platelet‐lymphocyte ratio (PLR) have been recently identified as potential markers of inflammation or immunity. This study aimed to investigate whether NLR, MLR, and PLR are associated with GBS characteristics in children. We also assessed the impact of the COVID‐19 pandemic on the characteristics of GBS in Iran. Methods In this retrospective cross‐sectional study, we reviewed the records of all 150 children diagnosed with GBS in the Children's Medical Center hospital affiliated with Tehran University of Medical Sciences (TUMS) from March 2017 until March 2022. The TUMS research ethics committee approved the study (Ethics code: IR.TUMS.CHMC.REC.1399.125). Patients' data including gender, age, clinical symptoms, laboratory findings, and electrodiagnostic study results were collected and analyzed. Results This study involved 150 children, comprising 93 boys and 57 girls, with an average age of 7.53 ± 3.75 years. The analysis demonstrated that the number of hospitalization days increased with an increase in NLR (p = 0.025). Moreover, patients with abnormal electrodiagnostic study patterns had a higher risk of intensive care unit (ICU) admission (p: 0.027), although according to binary logistic regression, respiratory failure at admission time was the only significant factor increasing the risk of ICU admission (p = 0.035). The study also found that the pandemic has resulted in a shift from acute inflammatory demyelinating polyneuropathy to acute motor axonal neuropathy as the most common EMG‐NCV pattern in our patients (p

Few case reports exist on the association of acute motor axonal neuropathy, a subtype of the Guillain-Barré syndrome (GBS), with systemic lupus erythematosus. Standard therapeutic guidelines for concomitant acute motor axonal neuropathy and systemic lupus erythematosus in the acute phase are not established, and no studies have reported physical medicine and rehabilitation perspective management in the plateau and recovery phases. A 50-yr-old woman with systemic lupus erythematosus presented with upper and lower limb weakness that progressed to an inability to walk. Neurological examination, radiologic evaluation, serologic analysis, and electrodiagnostic study were conducted, and she was diagnosed with acute motor axonal neuropathy. Subsequently, intravenous immunoglobulin therapy was administered. She complained of residual upper and lower extremity weakness and an inability to walk 3 mos after symptom onset. She underwent an intensive inpatient rehabilitation program for 6 wks and showed remarkable recovery in muscle strength and functional status (Berg Balance Scale, modified Barthel index, and Guillain-Barré syndrome disability scale). To our knowledge, this is the first reported case that focused on the functional outcomes after the rehabilitation program in acute motor axonal neuropathy with a history of systemic lupus erythematosus. This case report emphasizes the need for rehabilitation intervention for functional recovery in the plateau and recovery phases. [ABSTRACT FROM AUTHOR]

Abstract Background Guillain–Barré Syndrome is an immune mediated polyneuropathy. Ulcerative Colitis is an immune mediated chronic inflammatory condition mainly of the large intestine. Guillain–Barré Syndrome can present as a rare extraintestinal manifestation of Ulcerative Colitis when in remission or in a relapse. However, the concomitant presentation of Guillain–Barré Syndrome during a relapse of Ulcerative Colitis is very rare and only a few cases are reported to date. Case presentation A 24 year old young male diagnosed of Ulcerative Colitis presented with bloody diarrhea of frequency more than six times a day. He had been in clinical remission even after defaulting treatment for more than a year. He had also noted difficulty in walking prior to admission to the hospital. He was managed as for a severe relapse of Ulcerative Colitis and Guillain–Barré Syndrome. Appropriate management of both the illnesses helped him to recover. Conclusion Immune mediated diseases can have rare coexisting presentations. We report a case of Ulcerative Colitis with concomitant Guillain–Barré Syndrome. It is essential to be open minded and timely, appropriate treatment led to successful management of both the illnesses.

Acute motor axonal neuropathy (AMAN) is a rare immune-mediated disorder characterized by acute flaccid paralysis with elevated levels of GM1 antibodies. It is also known as a subtype of the Guillain-Barre syndrome (GBS) and develops since antigens serve as antibodies in the spinal cord. We report a case diagnosed as AMAN with symptoms of ascending limb symmetrical weakness. A neurological examination revealed a flaccid paralysis with multiple cranial nerve palsies. Electromyography showed an axonal type of GBS. The patient refused bone marrow fluid aspiration. Intravenous immunoglobulin was administered at the high care unit. Unfortunately, despite the standard therapy, an optimal recovery was not obtained. Hyperbaric oxygen (HBO) therapy has been known to be common in illnesses and some clinical diseases. Although it has not been indicated for peripheral neuropathy, a remarkable recovery was soon visible in the HBO-treated AMAN case. The HBO mechanisms involved here are anti-inflammation and immunomodulation. [ABSTRACT FROM AUTHOR]

Background: Guillain-Barre syndrome is uncommon neurological disease that occurs in acute phase as generalized polyradiculo-neuropathy. It has multiple variants some of them being uncommon and rarely encountered. Objective: The objective of the study was to determine the frequency and clinical characteristics of Guillain-Barre syndrome variants. Methods: A cross-sectional study was carried out in neurology department, Mayo hospital from July to December 2022. Patients clinically diagnosed as Guillain-Barre syndrome by using Brighton criteria were included. All the patients underwent electro diagnostic procedures and they were categorized into five basic variants i.e., Acute motor axonal neuropathy, Acute motor sensory axonal neuropathy, acute inflammatory demyelinating polyradiculo-neuropathy, acute sensory axonal neuropathy and mixed variety acute polyradiculo-neuropathy. Results: Out of total 85 patients, males were 56 (65.9%) and 29 (34.1%) were females. Adults were more affected with a mean age of 36±15.3. Frequency of acute motor axonal neuropathy (35.3%) was high followed by acute motor sensory axonal neuropathy (31.8%), mixed variety acute polyradiculo-neuropathy (16.5%), acute inflammatory demyelinating polyradiculo-neuropathy (12.9%) and acute sensory axonal neuropathy (3.5%) respectively. Conclusion: Acute motor axonal neuropathy is frequently encountered Guillain-Barre syndrome variant in our setup. Also, the frequency of acute sensory axonal neuropathy and mixed variety acute polyradiculo-neuropathy has increased with a declining trend of acute inflammatory demyelinating polyradiculo-neuropathy.

Background & Objective: This study aimed to analyze the frequency Guillain-Barré syndrome (GBS) subtypes and their relationship with clinical characteristics, seasonal variations and early prognosis in Van City, Turkey. Methods: Patients with GBS who were admitted between January 2007 and December 2017 and diagnosed with acute inflammatory demyelinating neuropathy (AIDP), acute motor axonal neuropathy (AMAN) or acute motor sensory axonal neuropathy (AMSAN) were reviewed. Demographics, season of clinical onset, history and type of preceding infection, the Hughes Disability Score (HDS) at admission and discharge were recorded. Results: Of a total 100 patients, 51% was diagnosed with AIDP, 25% with AMAN and the remaining 24% with AMSAN subtype. The most common seasonal onset was during the spring (34%), followed by the fall (30%). The history of gastroenteritis (GE) was present in 26% of the patients and these patients were more likely to have AMAN and AMSAN subtypes. HDS on admission and at discharge were significantly higher in patients with AMAN and AMSAN compared to those with AIDP (p=0.003 and p<0.001, respectively). The most important predictor of poor outcome at discharge was HDS on admission explaining between 50% and 80% of the total variance. Conclusion: There is a high prevalence of AMAN and AMSAN subtypes in Eastern region of Turkey. The history of GE, which is also commonly found in patients with GBS in this region, is more likely detected in patients with AMAN and AMSAN subtypes. Finally, clinical severity on admission is the most important contributor to clinical outcome at discharge. [ABSTRACT FROM AUTHOR]

Guillain-Barré syndrome (GBS) is a rare acquired immune-mediated polyneuropathy. Updated population-based data concerning paediatric GBS is needed. Paediatric patients aged below 18 years diagnosed with GBS between 2009 and 2018 in all 11 paediatric departments in Hong Kong were identified from the Hong Kong Hospital Authority Clinical Data Analysis and Reporting System. The collected data from medical health records were reviewed by paediatric neurologist from each department. Estimated incidence of paediatric GBS was calculated. We also compared our findings with other paediatric GBS studies in Asia. 63 subjects of paediatric GBS were identified, giving an estimated annual incidence of 0.62 per 100,000 population. Half of the subjects had acute inflammatory demyelinating polyneuropathy (AIDP) (n = 31; 49.2%), one quarter had Miller Fisher Syndrome (MFS) (n = 16; 25.4%), one-fifth had axonal types of GBS (n = 12; 19.0%), and four were unclassified. Paediatric subjects with axonal subtypes of GBS compared to the other 2 subtypes, had significantly higher intensive care unit (ICU) admission rates (p = 0.001) and longest length of stay (p = 0.009). With immunomodulating therapy, complete recovery was highest in those with MFS (100%), followed by AIDP (87.1%) and axonal GBS (75%). Our study also confirms a higher MFS rate for paediatric GBS in East Asia region and our study has the highest MFS rate (25.4%). Our population-based 10-year paediatric GBS study provides updated evidence on estimated incidence, healthcare burden and motor outcome of each subtype of paediatric GBS and confirmed a higher occurrence of paediatric MFS in East Asia. [ABSTRACT FROM AUTHOR]

Acute Motor Axonal Neuropathy (AMAN) is an immune-mediated disorder of the peripheral nervous system, part of the spectrum of the Guillain-Barre syndrome (GBS). An infectious event most often triggers it reported a few weeks before the onset. The reported case is of a 56 years-old woman who developed acute motor axonal neuropathy three weeks after respiratory infection with influenza A virus subtype H1N1. Despite early treatment with plasmapheresis and intravenous immunoglobulins, the patient remained tetraplegic, mechanically ventilated for five months, with repetitive unsuccessful weaning trails. The probable cause was considered to be phrenic nerve palsy in the context of acute motor axonal neuropathy. This case highlights that acute motor axonal neuropathy is a severe and life-threatening form of Guillain-Barre syndrome associated with significant mortality and morbidity. Neurological and physical recovery strongly depend on the inter-professional effort in an intensive care unit and neurology professionals.

We initially described two children who developed Guillain-Barré syndrome (GBS) complicated by rhabdomyolysis (RML), and reviewed five adult patients from the literature. Through analysis of the clinical features, laboratory examination, treatment and prognostic data from these seven patients, we found that when GBS “meets” RML, the most prominent characteristics were the following: male dominance; limb weakness, pain and respiratory failure could be caused by multiple factors; limb weakness and respiratory muscle paralysis were more serious than with GBS alone; and the probability of mechanical ventilation was increased. Neuroelectrophysiological studies revealed axonal lesions. Close monitoring and timely identification and intervention to remedy potentially fatal complications such as electrolyte disorder multisystem complications and kidney injury are crucial. With plasma exchange, peritoneal dialysis and supportive treatment, the long-term outcome of most patients was satisfactory.

While monocyte to high-density lipoprotein cholesterol ratio (MHR) has been reported to be associated with nervous system lesions, the role of MHR has not been determined in patients with Guillain-Barré Syndrome (GBS). The purpose of our study was to explore the role of MHR in patients with GBS. A total of 52 GBS patients were involved in the study retrospectively, including patients with acute inflammatory demyelinating polyradiculoneuropathy (AIDP), acute motor axonal neuropathy (AMAN), and acute motor sensory axonal neuropathy (AMSAN). We used Hughes Functional Grading Scale (HFGS) score to evaluate functional status in GBS patients. Among patients with different subtypes of GBS, MHR was significantly elevated in those with demyelination compared to patients without demyelination (p < 0.001); AIDP patients had an increased MHR compared with AMAN or AMSAN patients (p = 0.001; p = 0.013). There was a positive correlation between MHR and HFGS score (r = 0.463, p = 0.006) in AIDP patients, but not in AMAN or AMSAN. Multiple linear regression analysis revealed that MHR was independently associated with HFGS score (beta = 0.405, p = 0.013) in AIDP patients. Our study suggests that MHR as an inflammatory marker is elevated in patients with AIDP compared to AMAN or AMSAN patients, while MHR has a positive correlation with clinical severity in AIDP patients, suggesting that MHR may provide an additional information to reflect the pathophysiology of AIDP. [ABSTRACT FROM AUTHOR]

The complete genome sequence of Edwardsiella tarda strain GBS0709, isolated from an 81-year-old Japanese patient with the acute motor axonal neuropathy subtype of Guillain-Barré syndrome, was determined. It comprised a 3,632,068 bp circular chromosome and a 5,386 bp plasmid. The overall guanine and cytosine content was 57.3%., Competing Interests: The authors declare no conflict of interest.

Abstract Background Guillain–Barre syndrome (GBS) is an inflammatory polyradiculoneuropathy characterized by rapidly evolving weakness and areflexia, reaching nadir within 4 weeks. Data on the characteristic of GBS in Saudi Arabia are limited. This study aimed to describe the clinical, electrophysiological, and laboratory characteristics and outcome of a multicenter cohort of patients with GBS. Methods This is a retrospective multicenter nationwide study. Patients who had GBS, identified through Brighton Criteria, between January 2015 and December 2019 were included. Data collected included demographics, clinical features, cerebrospinal fluid profile, reported electrophysiological patterns, treatment, and outcome. Reported GBS subtypes were compared using chi-square, Fisher's exact, or Mann–Whitney U tests, as appropriate. Results A total of 156 patients with GBS were included (men, 61.5%), with a median age of 38 (interquartile range, 26.25–53.5) years. The most commonly reported antecedent illnesses were upper respiratory tract infection (39.1%) and diarrhea (27.8%). All but two patients (98.7%) had weakness, 64.1% had sensory symptoms, 43.1% had facial diplegia, 33.8% had oropharyngeal weakness, 12.4% had ophthalmoplegia, and 26.3% needed mechanical ventilation. Cytoalbuminological dissociation was observed in 69.1% of the patients. GBS-specific therapy was administered in 96.8% of the patients, of whom 88.1% had intravenous immunoglobulin, and 11.9% had plasmapheresis. Approximately half of the patients were able to walk independently within 9 months after discharge, and a third regained the ability to walk independently thereafter. Death of one patient was caused by septicemia. Acute inflammatory demyelinating polyradiculoneuropathy was the most commonly reported GBS subtype (37.7%), followed by acute motor axonal neuropathy (29.5%), and acute motor-sensory axonal neuropathy (19.2%). Conclusion The clinical and laboratory characteristics and outcome of GBS in the Arab population of Saudi Arabia are similar to the international cohorts. The overall prognosis is favorable.

Background: As a global health pandemic, the novel severe acute respiratory syndrome-coronavirus 2 (SARS- CoV2) outbreak began in December 2019 which rapidly spread to more than 200 countries. Respiratory complications and fever are the most obvious symptoms. Sometimes the neurological features are superimposed on the main disease and complicate patientchr('39')s status. Case Presentation: We describe 6 patients with COVID-19 and concomitant quadriparesia who underwent electrodiagnosis using EMG/NCS and results indicated 3 axonal variants of Guillain–Barré syndrome (GBS), including; 2 cases AMAN (acute motor axonal neuropathy), 1 case AMSAN (acute motor and sensory axonal neuropathy), three myopathies, including one combination of CIN/CIM (critical illness neuropathy/critical illness myopathy), one CIM and one acute polymyositis in these cases. Conclusion: Early diagnosis of the neuromuscular disorders of coronavirus could help for correct planning in the treatment of COVID-19 patients. Since GBS and inflammatory myopathies have an autoimmune basis, the immunotherapies such as IVIG, steroids, plasma exchange and other novel treatments as hemoperfusion can promise better and faster recovery in respiratory function and neuromuscular activity among COVID-19 patients who have musculature paralysis concomitantly. However, all these treatments are challenging and further clinical trials should be done to confirm the efficacy and safety of mentioned therapies.

Introduction: Guillain-Barré syndrome (GBS) has been classified into demyelinating and axonal subtypes or forms, such as acute motor axonal neuropathy (AMAN) and regional pharyngeal-cervical-brachial variant (PCBv). Objective: To study the relationship between motor nerve conduction blocks (CBs) and prognosis in AMAN and PCBv. Patients and Methods: We retrospectively analyzed six cases of AMAN and PCBv with serial nerve conduction studies (NCS) and electromyography (EMG). Results: The serial NCS (1st−2nd and 3rd week) showed, as the most constant data, a decreased amplitude of the compound muscle action potential (CMAP) in 100% of cases. CBs were present in 66.6% of cases. EMG (3rd week) showed signs of severe denervation in 33.3%. All patients were treated from the 1st−2nd week of evolution with intravenous immunoglobulins (IVIGs). Patients with CBs (1st−2nd and 3rd week), showed reversible CBs or reversible conduction failure (RCF) and complete recovery at 1 month. Patients without CBs, with persistent reduced distal CMAP amplitude (dCMAP), showed severe acute denervation due to axonal degeneration (3rd week and 1st−3rd month) and a slow recovery of several months. Conclusions: Not all axonal forms of GBS have a poor prognosis. This study of AMAN and PCBv shows that patients with CBs can have reversible CBs or RCF, and good prognosis. Patients without CBs, with persistent reduction of dCMAP amplitude decrement, have severe acute denervation, and a worse prognosis. AMAN and PCBv have a continuous spectrum ranging from CBs due to dysfunction/disruption of Nodes of Ranvier, called nodopathies, with reversible CBs or RCF and good prognosis, to axonal degeneration with worse prognosis. [ABSTRACT FROM AUTHOR]

Introduction:Guillain-Barré syndrome (GBS) has been classified into demyelinating and axonal subtypes or forms, such as acute motor axonal neuropathy (AMAN) and regional pharyngeal-cervical-brachial variant (PCBv).ObjectiveTo study the relationship between motor nerve conduction blocks (CBs) and prognosis in AMAN and PCBv.Patients and MethodsWe retrospectively analyzed six cases of AMAN and PCBv with serial nerve conduction studies (NCS) and electromyography (EMG).ResultsThe serial NCS (1st−2nd and 3rd week) showed, as the most constant data, a decreased amplitude of the compound muscle action potential (CMAP) in 100% of cases. CBs were present in 66.6% of cases. EMG (3rd week) showed signs of severe denervation in 33.3%. All patients were treated from the 1st−2nd week of evolution with intravenous immunoglobulins (IVIGs). Patients with CBs (1st−2nd and 3rd week), showed reversible CBs or reversible conduction failure (RCF) and complete recovery at 1 month. Patients without CBs, with persistent reduced distal CMAP amplitude (dCMAP), showed severe acute denervation due to axonal degeneration (3rd week and 1st−3rd month) and a slow recovery of several months.ConclusionsNot all axonal forms of GBS have a poor prognosis. This study of AMAN and PCBv shows that patients with CBs can have reversible CBs or RCF, and good prognosis. Patients without CBs, with persistent reduction of dCMAP amplitude decrement, have severe acute denervation, and a worse prognosis. AMAN and PCBv have a continuous spectrum ranging from CBs due to dysfunction/disruption of Nodes of Ranvier, called nodopathies, with reversible CBs or RCF and good prognosis, to axonal degeneration with worse prognosis.

Ni Komang Sri Dewi Untari,1,2 Kurnia Kusumastuti,3 Guritno Suryokusumo,4 I Ketut Sudiana5 1Department of Hyperbaric, Drs. Med. Rijadi S. Phys. Naval Health Institute, Surabaya, Indonesia; 2Doctoral Program, Faculty of Medicine, Airlangga University, Surabaya, Indonesia; 3Department of Neurology, Faculty of Medicine, Airlangga University, Surabaya, Indonesia; 4Department of Hyperbaric, Faculty of Medicine, Pembangunan Nasional University, Jakarta, Indonesia; 5Department of Pathology Anatomy, Faculty of Medicine, Airlangga University, Surabaya, IndonesiaCorrespondence: Ni Komang Sri Dewi UntariDepartment of Research and Development, Drs. Med. R. Rijadi S., Phys. Naval Health Institute, Surabaya, IndonesiaEmail komang_neuro@yahoo.comAbstract: We reported a rare case demonstrating that the hyperbaric oxygen chamber provided faster clinical improvement in a patient with a variant of Guillain-Barre Syndrome (GBS). A patient with progressive, acute weakness of upper extremity locomotor muscles and with difficulty breathing and swallowing was diagnosed with axonal GBS. Despite life-saving conventional therapies, there was no significant improvement until day 5. During hyperbaric oxygen therapy, there were daily gradual improvements until day 20, at which time the patient was capable of walking slowly without using a walking aid.Keywords: acute motor axonal neuropathy, hyperbaric oxygen

Background: Internationally approved electrodiagnostic criteria for Guillain Barre syndrome lack in children. We intended to compare the diagnostic accuracy of the currently available five electrophysiological criteria for childhood Guillain Barre Syndrome (GBS) at the time of sentinel assessment. Methodology: In this single-center study, data of children diagnosed with GBS between January 2013 to December 2017 were retrieved. Patient charts were reviewed for clinical features, electrophysiological recordings. The electrodiagnostic results (4 motor nerves and two sensory nerves in upper limbs and lower limbs) were reanalyzed and were classified based on Dutch group; Ho; Hadden; Hughes and Rajabally criteria for GBS. Results: During this study period, of the 205 children with clinical features of GBS, 15 children had incomplete electrophysiological data, and four children were excluded due to missing data. The mean age of onset of the 186 children enrolled was 77 months; the median duration from symptom onset to electrodiagnostic evaluation was seven days; pure motor and motor-sensory form of GBS was seen in 71 and 115 children. Based on the Hadden criteria, a demyelinating pattern was noted in 57 children; axonal in 37; Inexcitable in 84 and Equivocal in 8 children. The sensitivity of the various criteria ranged from 71% to 100% for demyelination, 97% to 100% for axonal. The degree of agreement using Hadden and Rajabally criteria for Equivocal subtypes was 0.93. Conclusions: The Rajabally criteria showed the best sensitivity, specificity and diagnostic accuracy for electrodiagnosis of GBS in children when compared against Hadden criteria.

Neurological complications following SARS-CoV-2 infections are increasingly recognized. Despite that, sequelae from SARS-CoV-2 infection in gravid women are uncommonly reported. We present a case of acute motor axonal neuropathy, a variant of Guillain-Barre syndrome, in a 26-year-old primigravida at 34 weeks' gestation. She presented with worsening diplopia, dysphagia, and lower extremity sensory and motor deficits after testing positive for SARS-CoV-2 2 weeks earlier. Due to rapidly progressive symptoms, she required endotracheal intubation and mechanical ventilation. Initially she was diagnosed with Miller Fisher syndrome, but serology and electromyography studies were consistent with acute motor axonal neuropathy. Spontaneous preterm delivery, supportive care, and intravenous immunoglobulin therapy resulted in clinical improvement. Steady recovery was achieved by a prolonged rehabilitation program. [ABSTRACT FROM AUTHOR]

Introduction/Aims: Patients with acute motor axonal neuropathy (AMAN) generally have pure motor neuropathy and clinicians usually do not link pain with AMAN. The aim of this retrospective study was to describe the character, location, and intensity of pain in AMAN and acute inflammatory demyelinating polyneuropathy (AIDP) in the acute phase. Methods: This was a retrospective study in 44 patients with Guillain‐Barré syndrome (GBS) having progressive weakness of more than one limb. The information, including the demographic characteristics, preceding infections, clinical symptoms and signs, severity at nadir, the characteristics of pain, use of analgesics, laboratory and electrophysiological data, and the medical treatment for GBS, were collected from the medical records. Results: In 44 patients, 40.9% were diagnosed as AMAN, and 34.1% as AIDP. Pain was more prevalent in AMAN (76.5%) than in AIDP (26.7%, P =.02). Low back and extremities were the most common locations of pain in AMAN (7/13 and 7/13, respectively) and AIDP (2/4 and 2/4, respectively). Discussion: Pain was a common symptom in AMAN in the acute stage. The presence or absence of pain is not useful for distinguishing AIDP from AMAN. [ABSTRACT FROM AUTHOR]

Objective: In the young generations with nitrous oxide abuse (N2O), featured electrophysiological response of the peripheral neuropathy caused by nitrous oxide remains to be defined. Methods: Patients with nitrous oxide abuse (20 cases), two variants of Guillain-Barré syndrome (GBS), that is, acute inflammatory demyelinating polyradiculoneuropathy (GBS-AIDP, 19 cases) and acute motor axonal neuropathy (GBS-AMAN, 18 cases), as well as diabetic peripheral neuropathy (DPN, 20 cases) were enrolled into this study. Electrophysiological parameters including distal motor latency (DML), motor nerve conduction velocity (MNCV), sensory nerve conduction velocity (SNCV), amplitudes of compound muscle action potential (CMAP), and sensory nerve action potential (SNAP) were measured and analyzed by comparing the parameters between the aforementioned patients groups as well as normal control group (20 subjects). Results: Compared to normal control subjects, patients with nitrous oxide abuse showed prolonged DML, slower MNCV and SNCV in the limbs, lower amplitudes of CMAP in the median, tibial and peroneal nerves, and lower SNAP in median and ulnar nerves. Abnormalities of MNCV and amplitudes of CMAP in the lower limbs were significantly higher than that in the upper limbs. Abnormal electrophysiological features of patients with nitrous oxide abuse were dramatically different from those in GBS-AIDP or DPN patients, but similar to those in GBS-AMAN patients. Conclusions: Nitrous oxide abuse could cause abnormal electrophysiological response in the limbs. Some of the parameters (DML, MNCV, SNCV, CMAP and SNAP) appeared significantly different between the patients with nitrous oxide abuse, GBS with AIDP or AMAN, and DPN patients. Significance: Electrophysiological examination could be considered as an important supporting factor in differential diagnosis for nitrous oxide abuse, GBS with AIDP or AMAN, and DPN. [ABSTRACT FROM AUTHOR]

• Few reports discuss GBS patients not classified to have AIDP or AMAN. • Upper and lower extremity F wave frequency was high in the unclassified group. • Unclassified subgroup showed better prognosis using the Rajabally criteria. • Classification with the Rajabally criteria is useful for predicting prognosis. Electrophysiological classification of Guillain-Barré syndrome (GBS) is important for predicting its clinical course; however, few reports discuss GBS patients who do not conform to the acute inflammatory demyelinating polyneuropathy (AIDP) or acute motor axonal neuropathy (AMAN) classifications. Therefore, the present study assessed the features of unclassified types of GBS and compared them to those of AIDP and AMAN. We compared clinical symptoms, nerve conduction, and laboratory data among patients with AIDP, AMAN, and unclassified subtypes of GBS, according to criteria developed by Rajabally, Hadden, and Ho. According to the Rajabally criteria, the F wave frequency in the upper and lower extremities was higher in the unclassified subgroup than in the AIDP and AMAN subgroups; however, according to the Hadden and Ho criteria, the F wave frequency in only the lower extremities was higher in the unclassified subgroup than in the other subgroups. The unclassified subgroup showed better prognosis using the Rajabally criteria. Classification with the Rajabally criteria is useful for predicting prognosis and determining treatment in patients with GBS. Moreover, unclassified patients exhibit the quickest recovery. [ABSTRACT FROM AUTHOR]

The recent outbreak of Zika virus infection increased the incidence of Guillain–Barré syndrome (GBS). Following the first reported case of GBS after Zika virus infection in 2013, there has been a considerable increase in the incidence of GBS in endemic countries, such as French Polynesia and Latin American countries. The association between coronavirus disease 2019 (COVID‐19), caused by the novel severe acute respiratory syndrome coronavirus 2 (SARS‐CoV‐2), and GBS is another emerging research hotspot. Electrophysiological studies have suggested that GBS patients associated with Zika virus infection or COVID‐19 tend to manifest acute inflammatory demyelinating polyneuropathy, rather than acute motor axonal neuropathy (AMAN). Causative autoantibodies, such as anti‐ganglioside antibodies in AMAN associated with Campylobacter jejuni infection, have not been identified in GBS associated with these emerging infectious diseases. Nevertheless, recent studies suggested molecular mimicry between these viruses and human proteins related to GBS. Recent studies have shown the efficacy of new vaccines, containing artificial messenger RNA encoding the spike protein of SARS‐CoV‐2, against COVID‐19. These vaccines are now available in many countries and massive vaccination campaigns are currently ongoing. Although there are long‐standing concerns about the increased risk of GBS after inoculation of conventional vaccines, the risk of GBS is not considered a legitimate reason to limit administration of currently available vaccines, because the benefits outweigh the risks. [ABSTRACT FROM AUTHOR]

Background: Guillain-Barre syndrome (GBS) is an inflammatory polyradiculoneuropathy characterized by rapidly evolving weakness and areflexia, reaching nadir within 4 weeks. Data on the characteristic of GBS in Saudi Arabia are limited. This study aimed to describe the clinical, electrophysiological, and laboratory characteristics and outcome of a multicenter cohort of patients with GBS.Methods: This is a retrospective multicenter nationwide study. Patients who had GBS, identified through Brighton Criteria, between January 2015 and December 2019 were included. Data collected included demographics, clinical features, cerebrospinal fluid profile, reported electrophysiological patterns, treatment, and outcome. Reported GBS subtypes were compared using chi-square, Fisher's exact, or Mann-Whitney U tests, as appropriate.Results: A total of 156 patients with GBS were included (men, 61.5%), with a median age of 38 (interquartile range, 26.25-53.5) years. The most commonly reported antecedent illnesses were upper respiratory tract infection (39.1%) and diarrhea (27.8%). All but two patients (98.7%) had weakness, 64.1% had sensory symptoms, 43.1% had facial diplegia, 33.8% had oropharyngeal weakness, 12.4% had ophthalmoplegia, and 26.3% needed mechanical ventilation. Cytoalbuminological dissociation was observed in 69.1% of the patients. GBS-specific therapy was administered in 96.8% of the patients, of whom 88.1% had intravenous immunoglobulin, and 11.9% had plasmapheresis. Approximately half of the patients were able to walk independently within 9 months after discharge, and a third regained the ability to walk independently thereafter. Death of one patient was caused by septicemia. Acute inflammatory demyelinating polyradiculoneuropathy was the most commonly reported GBS subtype (37.7%), followed by acute motor axonal neuropathy (29.5%), and acute motor-sensory axonal neuropathy (19.2%).Conclusion: The clinical and laboratory characteristics and outcome of GBS in the Arab population of Saudi Arabia are similar to the international cohorts. The overall prognosis is favorable. [ABSTRACT FROM AUTHOR]

Background: As a global health pandemic, the novel severe acute respiratory syndromecoronavirus 2 (SARS-CoV2) outbreak began in December 2019 which rapidly spread to more than 200 countries. Respiratory complications and fever are the most obvious symptoms. Sometimes the neurological features are superimposed on the main disease and complicate patient's status. Case Presentation: We describe 6 patients with COVID-19 and concomitant quadriparesia who underwent electrodiagnosis using EMG/NCS and results indicated 3 axonal variants of Guillain--Barré syndrome (GBS), including; 2 cases AMAN (acute motor axonal neuropathy), 1 case AMSAN (acute motor and sensory axonal neuropathy), three myopathies, including one combination of CIN/CIM (critical illness neuropathy/critical illness myopathy), one CIM and one acute polymyositis in these cases. Conclusion: Early diagnosis of the neuromuscular disorders of coronavirus could help for correct planning in the treatment of COVID-19 patients. Since GBS and inflammatory myopathies have an autoimmune basis, the immunotherapies such as IVIG, steroids, plasma exchange and other novel treatments as hemoperfusion can promise better and faster recovery in respiratory function and neuromuscular activity among COVID-19 patients who have musculature paralysis concomitantly. However, all these treatments are challenging and further clinical trials should be done to confirm the efficacy and safety of mentioned therapies. [ABSTRACT FROM AUTHOR]

Axonal variants of Guillain–Barré syndrome (GBS) mainly include acute motor axonal neuropathy, acute motor and sensory axonal neuropathy, and pharyngeal-cervical-brachial weakness. Molecular mimicry of human gangliosides by a pathogen's lipooligosaccharides is a well-established mechanism for Campylobacter jejuni-associated GBS. New triggers of the axonal variants of GBS (axonal GBS), such as Zika virus, hepatitis viruses, intravenous administration of ganglioside, vaccination, and surgery, are being identified. However, the pathogenetic mechanisms of axonal GBS related to antecedent bacterial or viral infections other than Campylobacter jejuni remain unknown. Currently, autoantibody classification and serial electrophysiology are cardinal approaches to differentiate axonal GBS from the prototype of GBS, acute inflammatory demyelinating polyneuropathy. Newly developed technologies, including metabolite analysis, peripheral nerve ultrasound, and feature selection via artificial intelligence are facilitating more accurate diagnosis of axonal GBS. Nevertheless, some key issues, such as genetic susceptibilities, remain unanswered and moreover, current therapies bear limitations. Although several therapies have shown considerable benefits to experimental animals, randomized controlled trials are still needed to validate their efficacy. [ABSTRACT FROM AUTHOR]

Background: Internationally approved electrodiagnostic criteria for Guillain Barre syndrome lack in children. We intended to compare the diagnostic accuracy of the currently available five electrophysiological criteria for childhood Guillain Barre Syndrome (GBS) at the time of sentinel assessment. Methodology: In this single-center study, data of children diagnosed with GBS between January 2013 to December 2017 were retrieved. Patient charts were reviewed for clinical features, electrophysiological recordings. The electrodiagnostic results (4 motor nerves and two sensory nerves in upper limbs and lower limbs) were reanalyzed and were classified based on Dutch group; Ho; Hadden; Hughes and Rajabally criteria for GBS. Results: During this study period, of the 205 children with clinical features of GBS, 15 children had incomplete electrophysiological data, and four children were excluded due to missing data. The mean age of onset of the 186 children enrolled was 77 months; the median duration from symptom onset to electrodiagnostic evaluation was seven days; pure motor and motor-sensory form of GBS was seen in 71 and 115 children. Based on the Hadden criteria, a demyelinating pattern was noted in 57 children; axonal in 37; Inexcitable in 84 and Equivocal in 8 children. The sensitivity of the various criteria ranged from 71% to 100% for demyelination, 97% to 100% for axonal. The degree of agreement using Hadden and Rajabally criteria for Equivocal subtypes was 0.93. Conclusions: The Rajabally criteria showed the best sensitivity, specificity and diagnostic accuracy for electrodiagnosis of GBS in children when compared against Hadden criteria. [ABSTRACT FROM AUTHOR]

Introduction: The prognosis of Guillain-Barré Syndrome (GBS) at an early stage with explicit biomarkers is critical to distinguish patients with possibility of poor recovery. Cerebrospinal Fluid (CSF) serves as an impending source for biomarkers that portrays the exact biochemical changes. Aim: To find out if there is any prognostic value of high CSF phosphorylated Neurofilament Heavy subunit (pNf-H) levels, measured during first two weeks of onset of GBS, as assessed by the level of disability at six months after the onset of GBS. Materials and Methods: The cohort study was conducted in the Department of Neurology and Department of Immunology and Molecular Medicine, at the Sher-I-Kashmir Institute of Medical Sciences, Soura, Srinagar, Jammu and Kashmir, India, over a period of two years from August 2015 to August 2017. Sixty two patients who satisfied the required diagnostic standards for GBS (study group) and 35 patients with tension-type headache (control group) were selected for the study. After clinical and electrophysiological assessment, CSF samples were collected. A commercially available sandwich enzyme immunoassay kit, manufactured by BioVendor-Laboratorní medicína (Czech Republic), was used for measuring human pNf-H quantitatively. Results: Mean CSF pNf-H level in patients with good outcome was 325.3 pg/mL whereas, in patients with poor outcome it was 3655.2 pg/mL. CSF pNf-H levels were found to be suggestively higher in GBS patients with poor outcome as compared to those with good outcome. Only eight patients in good outcome group had pathologically high CSF Nf-H levels whereas 10 patients in poor outcome group had CSF Nf-H levels ≤730 pg/mL. The odds ratio was 17.1 (95% Confidence Interval (CI) 3.83-76.29). Thus, high CSF Nf-H levels on admission predicted poor outcome in GBS (p-value

Rationale: Guillain Barre syndrome (GBS) is an acute neurological illness leading to quadriparesis with respiratory involvement. It can be triggered by infections, vaccinations, surgery, trauma, transplantation and drugs. Anti-rabies cell culture vaccines introduced to overcome the high rate of neurological complications associated with tissue based rabies vaccine, can be very rarely associated with GBS. Patient concerns: A 50-year-old female presented with acute severe upper back pain evolving into pure motor quadriparesis following administration of human diploid cell vaccine for rabies. Diagnosis: Acute motor axonal neuropathy variant of GBS following anti-rabies human diploid cell vaccine. Interventions: Intravenous high dose steroids. Outcomes: Patient recovered completely within 1 month. Lessons: Although anti-rabies cell culture vaccines are highly immunogenic and safe, they are rarely associated with GBS. Clinicians should be aware of this link because prompt diagnosis and treatment can result in complete recovery and avoid complications.

• Electrodiagnostic (EDX) criteria for GBS that rely on motor nerve conduction studies (NCS) are suboptimal early in the course of the disease and for GBS variants. • Sensory NCS show added diagnostic value in GBS. • EDX criteria should be reconsidered in the future in an attempt to optimize their diagnostic yield. Electrophysiology plays a determinant role in Guillain–Barré syndrome (GBS) diagnosis, classification, and prognostication. However, traditional electrodiagnostic (EDX) criteria for GBS rely on motor nerve conduction studies (NCS) and are suboptimal early in the course of the disease or in the setting of GBS variants. Sensory nerve conduction studies, including the sural-sparing pattern and the sensory ratio are not yet included in EDX criteria despite their well-established role in GBS diagnosis. The aim of this review is to discuss the diagnostic value of sensory NCS in GBS, their role in establishing the diagnosis and predicting the outcome according to the various subtypes of the disease. [ABSTRACT FROM AUTHOR]

Background: The most common subtypes of Guillain-Barré syndrome (GBS) are acute inflammatory demyelinating polyneuropathy (AIDP) and acute motor axonal neuropathy (AMAN). In the first days after the onset of weakness, standard nerve conduction studies (NCS) may not distinguish GBS subtypes. Reduced nerve excitability may be an early symptom of nerve dysfunction, which can be determined with the compound muscle action potential (CMAP) scan. The aim of this study was to explore whether early changes in motor nerve excitability in GBS patients are related to various subtypes.Methods: Prospective case-control study in 19 GBS patients from The Netherlands and 22 from Bangladesh. CMAP scans were performed within 2 days of hospital admission and NCS 7-14 days after onset of weakness. CMAP scans were also performed in age- and country-matched controls.Results: CMAP scan patterns of patients who were classified as AMAN were distinctly different compared to the CMAP scan patterns of the patients who were classified as AIDP. The most pronounced differences were found in the stimulus intensity parameters.Conclusions: CMAP scans made at hospital admission demonstrate several characteristics that can be used as an early indicator of GBS subtype. [ABSTRACT FROM AUTHOR]

Ganglioside antibodies can help diagnose distinct acute and chronic inflammatory neuropathies including axonal variants of Guillain-Barre syndrome, Miller-Fisher syndrome (MFS), multifocal motor neuropathy, and chronic sensory ataxic neuropathies. Because ganglioside antibody testing may be routinely ordered in patients with suspected inflammatory neuropathy, we sought to evaluate its yield and utilization in clinical practice. We performed a retrospective chart review of all patients at London Health Sciences Centre who underwent ganglioside antibody testing between April 2019 and August 2023. The disease phenotype was determined for each patient, and the proportion of all tests that yielded a true-positive result was calculated. Ganglioside antibody positivity was classified as a true-positive result if the disease phenotype was robustly associated with the detected ganglioside antibody and there was no other more likely diagnosis. We identified 92 patients who underwent ganglioside antibody testing. One patient (1%) was classified as having a true-positive result; this patient had GQ1b-IgG positivity with MFS. Among 92 patients tested, 20 patients (22%) had a disease phenotype that was considered to be robustly associated with ganglioside antibody positivity. The yield of ganglioside antibody testing in clinical practice is low. We found that this testing is frequently ordered in patients with disease phenotypes that are not robustly associated with ganglioside antibody positivity, indicating that suboptimal test utilization is a primary contributor to its low yield. Restricting ganglioside antibody testing to patients with characteristic disease phenotypes would be valuable to improving yield and utilization of this testing. • We found the yield of ganglioside antibody testing at our centre to be low at 1%. • We identified the potential for clinically irrelevant positive antibody results. • Testing was frequent among patients without characteristic disease phenotypes. • Inappropriate patient selection was likely a primary contributor to low test yield. • Restricting testing to those with characteristic disease phenotypes is warranted. [ABSTRACT FROM AUTHOR]

We propose the finger drop sign as a new clinical variant of acute motor axonal neuropathy (AMAN) defined by immunological and radiological evidence. We identified eight consecutive patients who had AMAN. All of them developed prominent involvement of the finger extensors. We performed magnetic resonance imaging (MRI) of the extremity muscles and serological assays for antiganglioside antibodies and Campylobacter jejuni. Patients with AMAN showed characteristic and a markedly sustained weakness of the finger extensors with a distinctive pattern of the finger drop sign. Limb MRI revealed unevenly distributed abnormal signals in the muscles mainly innervated by the posterior interosseous nerve. All tested patients showed positivity for immunoglobulin G antibody against ganglioside complex of GM1 and phosphatidic acid. A pathophysiological understanding of this unique syndrome can provide further insight into antiganglioside-antibody-mediated axonal injury in Guillain-Barré syndrome. [ABSTRACT FROM AUTHOR]

Aims and Objectives: To study clinical profile and outcome in Pediatric Guillain Barre Syndrome (GBS).Materials and Methods: Retrospective study of 30 patients (age 1 month to 12 years) admitted with GBS enrolled over a period of 2 years (August 2016-July 2018) from Department of Pediatrics of tertiary centre.Results: Mean age was 5.4 years (21 males; 9 females). Most common presenting complaints- symmetrical lower limb weakness (26 cases; 86.67% cases), respiratory complaints (6 cases; 20% cases), quadriparesis (4 cases; 13.33% cases) and facial palsy (2 cases; 6.67%). Antecedent illnesses- gastrointestinal (6 cases) and respiratory (3 cases). Two patients had varicella (in preceding one week) and one had mumps (one month prior to presentation). MRI-spine done in 12 patients; of whom 9 had features of GBS (thickening and contrast enhancement of the intrathecal and cauda equina nerve roots on T1 weighted MRI). Nerve conduction studies done in 16 patients, of which Acute Motor Axonal Neuropathy was seen in 10 cases. Intravenous immunoglobulin was given to 27 patients while 3 received methylprednisolone in addition. 90% patients receiving IVIG showed improvement. Sixteen patients were admitted to the intensive care unit and 7 required mechanical ventilation. Average hospital stay was 13 days. Two patients had recurrent episodes. Common complications included- pneumonia (6 cases; 2 aspiration and 4 ventilator associated) and autonomic disturbances (6 cases). Two patients died due to autonomic disturbances and presence of autonomic disturbances predicted higher mortality (P = 0.034).Conclusions: Gastrointestinal illness was common antecedent illness for GBS. Symmetrical lower limb weakness was commonest complaint. Pneumonia and autonomic disturbances were commonest complications. Presence of autonomic disturbances predicted higher mortality. [ABSTRACT FROM AUTHOR]

Background: Information regarding the clinical presentation and outcome of Guillain-Barré Syndrome (GBS) in adults from Latin America is limited.Objective: To identify clinical characteristics and short-term outcome predictors in adult Mexican patients with GBS.Patients and Methods: We included adult patients with clinical and electrophysiological data with confirmed GBS, admitted to a tertiary hospital in Western Mexico, from January 2002 to February 2011. A good outcome at hospital discharge was considered if patients had a Hughes score of 0-2 and at 3 and 6 months, a Hughes score of 0-1.Results: A total of 115 patients were analyzed (68% men, mean age 44 years old, range 18-84). Previous infection occurred in 63% of cases. Descendent pattern of weakness was observed in 40 (35%) patients. GBS subtypes were: acute motor axonal neuropathy in 31%, acute inflammatory demyelinating polyneuropathy in 29%, sensory axonal neuropathy (AMSAN) in 18%, and equivocal in 22%. A total of 73 (63%) patients received induction therapy: 50 (68%) received plasmapheresis and 13 (18%) received intravenous immunoglobulin (IVIG). In-hospital mortality occurred in 14 (12%) patients. Early gait complaints and emergency room admission with mild Hughes score (0-2) were predictors for a good outcome at hospital discharge (P < 0.05); meanwhile, age >75 years; dysarthria and higher Hughes score were associated with a poor outcome(P < 0.05).Conclusions: Axonal pattern, motor involvement, and the descendent pattern of presentation were the main clinical GBS findings in our cohort. Higher Hughes scale scores at hospital admission were a strong predictor for a bad outcome at hospital discharge and short-term follow-up, independently of treatment type or in-hospital management. GBS in Mexico still carries considerable mortality. [ABSTRACT FROM AUTHOR]

COVID-19, which is considered a global pandemic, is constantly being renewed. Acute motor axonal neuropathy (AMAN) is a rare, axonal variant of Guillain-Barré syndrome (GBS). This article presents a 69-year-old female patient diagnosed with AMAN due to a COVID-19 infection. Sixty-nine-year-old female patient who started myalgia, headache, cough 14 days ago, and was added diarrhea, fever, loss of smell and taste. Nasopharyngeal SARS-CoV-2 polymerase chain reaction (PCR) was found to be positive. Ten days later, numbness and weakness developed in the distal extremities. As a result of the patient's neurological examination, acute motor axonal neuropathy (AMAN) was diagnosed. [ABSTRACT FROM AUTHOR]

• AIDP patients tends to recover independent walk sooner than patients with AMAN. • AMAN is the most common electrophysiological subtype in our center in Mexico. • AMAN represents a severe form of the disease and predicts worse outcomes in the short term. The clinical characteristics of electrophysiological subtypes and prognostic factors of Mexican adults diagnosed with Guillain-Barré Syndrome (GBS) have not been described. A single center, ambispective, cohort study was performed (2015–2019). GBS was defined following the Asbury and Cornblath criteria. Electrodiagnosis was made according to Hadden criteria. Clinical, biochemical and electrodiagnostic parameters were described, compared and analyzed using a multivariate model. Only patients who completed a 3-month follow-up were included. 137 GBS patients (92 males; mean age 46.6 ± 16.6). 132 (96.3%) underwent an electrodiagnostic assessment. 68 (51.5%) were classified as axonal GBS, with further classified into two groups: acute motor axonal neuropathy (AMAN) 45.4%, and acute motor and sensory axonal neuropathy (AMSAN) 8,6%. The following characteristics were lower in the AMAN group: Medical Research Counsel sumscore (MRC) 30.1 ± 16.3 vs 36.4 ± 14.4, unilateral facial palsy 10% vs 25.9% and albuminocytologic dissociation 41.3% vs. 71.7%. Multivariate analysis found AMAN as an independent predictor of an unfavorable outcome OR: 3.34 (p = 0.03) AMAN subtype is the most frequent presentation of GBS in Mexican adult patients and an independent predictor of inability to walk independently at 3 months after discharge. [ABSTRACT FROM AUTHOR]

Introduction. Guillain–Barre syndrome (GBS) is the most common cause of acute flaccid paralysis in healthy infants and children. Acute motor axonal neuropathy (AMAN) is a type of GBS characterized by motor syndrome with no sensory symptoms. Case outline. Authors describe a six-and-a-half year old girl with atypical clinical presentation of AMAN with severe painful lower limbs and refusal of the leg reliance with typical findings on nerves conduction studies. Conclusion. Despite the nerve conduction study findings, atypical forms of AMAN and GBS are possible. Pain symptoms must be taken very seriously and treated careful by the clinicians.

Background: Coronavirus disease 2019 (COVID-19) is associated with muscle and nerve injuries as a consequence of prolonged critical illness or the infection itself. In this study, we evaluated neuromuscular involvement in patients who underwent post-acute intensive rehabilitation after COVID-19. Methods: Clinical and neurophysiological evaluations, including nerve conduction studies and electromyography, were performed on 21 consecutive patients admitted for rehabilitation after COVID-19. Results: Clinical signs suggesting muscle or nerve involvement (weakness, reduced deep tendon reflexes, impaired sensitivity, abnormal gait) were found in 19 patients. Neurophysiological examinations confirmed neuromuscular involvement in 17 patients: a likely association of critical illness myopathy (CIM) and critical illness polyneuropathy (CIP) was found in 5 patients; CIM alone was found in 4 patients; axonal sensory-motor polyneuropathy was found in 4 patients (CIP in 2 patients, metabolic polyneuropathy in 2 patients); Guillain-Barré syndrome was found in 2 patients (classical demyelinating sensory-motor polyneuropathy and acute motor axonal neuropathy, respectively); peroneal nerve injury was found in 1 patient; and pre-existing L4 radiculopathy was found in 1 patient. Conclusions: Neuromuscular involvement is a very common finding among patients admitted for rehabilitation after COVID-19, and proper investigation should be conducted when muscle or nerve injury is suspected for adequate rehabilitative strategy planning.