Diagnosis Of Guillain-Barre Syndrome Research Articles

New chronic inflammatory demyelinating polyneuropathy/Guillain-Barré syndrome guidelines - impact on clinical practise.

There is no diagnostic biomarker that can reliably detect Guillain-Barré syndrome (GBS) or chronic inflammatory demyelinating polyneuropathy (CIDP). Diagnosis relies upon integrating key clinical characteristics and relevant supportive data. Consequently, misdiagnosis and delayed diagnosis are common. Diagnostic criteria have proven valuable resources to improve diagnosis, but are underutilized during routine clinical care. In 2021, the EAN/PNS CIDP criteria was published, and were followed by the EAN/PNS GBS criteria in 2023. Both guidelines utilized GRADE methodology to formulate evidence-based recommendations that are intended to be used by adult and paediatric clinicians across diverse care settings to optimize diagnostic accuracy and improve patient outcomes during routine clinical care. The EAN/PNS GBS and CIDP criteria detail specific clinical, electrophysiological, and laboratory features that raise diagnostic confidence, and call attention to diagnostic mimics. The sensitivity of EAN/PNS and other modern criteria to detect GBS and CIDP is high, but utilization during clinical practice is low. Complexity is one factor limiting widespread application. Strategies are needed to optimize criteria adoption during routine clinical care such that GBS and CIDP diagnosis can be achieved with greater speed and accuracy.

Post-Infectious Guillain Barré Syndrome Related to COVID-19: Unusual Case Report

Guillain-Barré Syndrome (GBS) is a rare immune mediated inflammatory disease of the peripheral nervous system that is presumed to be triggered by preceding infections. In the era of pandemic COVID-19 (coronavirus disease), there is an emerging case of para/post infectious GBS associated to COVID-19 that are described worldwide. Here, we report a 55-years-old man who was admitted for fatigue, fever and cough related to covid-19. Later, after disappearing of covid-19 symptoms he began presenting an ascending areflexic tetraparesis. CSF and NCS were in favour of GBS diagnosis, in particular pure motor AIDP subtype. Antigangliosides antibodies were negative. Lumbosacral MRI showed contrast enhancement of cauda equina roots. The outcome was good after receiving intravenous immunoglobulins treatment.

Guillain-Barré syndrome: A comprehensive review.

Guillain-Barré syndrome (GBS) is a potentially devastating yet treatable disorder. A classically postinfectious, immune-mediated, monophasic polyradiculoneuropathy, it is the leading global cause of acquired neuromuscular paralysis. In most cases, the immunopathological process driving nerve injury is ill-defined. Diagnosis of GBS relies on clinical features, supported by laboratory findings and electrophysiology. Although previously divided into primary demyelinating or axonal variants, this dichotomy is increasingly challenged, and is not endorsed by the recent European Academy of Neurology (EAN)/Peripheral Nerve Society (PNS) guidelines. Intravenous immunoglobulin and plasma exchange remain the primary modalities of treatment, regardless of the electrophysiological subtype. Most patients recover, but approximately one-third require mechanical ventilation, and 5% die. Disease activity and treatment response are currently monitored through interval neurological examination and outcome measures, and the potential role of fluid biomarkers is under ongoing scrutiny. Novel potential therapies for GBS are being explored but none have yet modified clinical practice. This review provides a comprehensive update on the pathological and clinical aspects of GBS for clinicians and scientists.

Evaluating algorithms for identifying incident Guillain-Barré Syndrome in Medicare fee-for-service claims

ObjectiveClaims data can be leveraged to study rare diseases such as Guillain-Barré Syndrome (GBS), a neurological autoimmune condition. It is difficult to accurately measure and distinguish true cases of disease with claims without a validated algorithm. Our objective was to identify the best-performing algorithm for identifying incident GBS cases in Medicare fee-for-service claims data using chart reviews as the gold standard. Study design and settingThis was a multi-center, single institution cohort study from 2015 to 2019 that used Medicare-linked electronic health record (EHR) data. We identified 211 patients with a GBS diagnosis code in any position of an inpatient or outpatient claim in Medicare that also had a record of GBS in their electronic medical record. We reported the positive predictive value (PPV = number of true GBS cases/total number of GBS cases identified by the algorithm) for each algorithm tested. We also tested algorithms using several prevalence assumptions for false negative GBS cases and calculated a ranked sum for each algorithm's performance. ResultsWe found that 40 patients out of 211 had a true case of GBS. Algorithm 17, a GBS diagnosis in the primary position of an inpatient claim and a diagnostic procedure within 45 days of the inpatient admission date, had the highest PPV (PPV = 81.6%, 95% CI (69.3, 93.9). Across three prevalence assumptions, Algorithm 15, a GBS diagnosis in the primary position of an inpatient claim, was favored (PPV = 79.5%, 95% CI (67.6, 91.5). ConclusionsOur findings demonstrate that patients with incident GBS can be accurately identified in Medicare claims with a chart-validated algorithm. Using large-scale administrative data to study GBS offers significant advantages over case reports and patient repositories with self-reported data, and may be a potential strategy for the study of other rare diseases.

Guillain-Barré syndrome

Guillain-Barré syndrome (GBS), an acute immune-mediated neuropathy, occurs following immunological stimulation, such as infection, with complement-mediated neuropathy implicated in the pathophysiology of this condition. Glycolipid antibodies produced by molecular mimicry are detected in approximately 60% of cases. Recent studies have suggested the role of cell-mediated immunity in the pathogenesis of GBS. Intravenous immunoglobulin and plasma exchange are established immunotherapies. In this article, based on the latest knowledge, we describe the pathophysiology, diagnosis, treatment, prognosis, and prognostic prediction of GBS. Furthermore, we discuss some GBS guidelines published by the European Academy of Neurology/Peripheral Nerve Society.

Guillain–Barre syndrome following scrub typhus: a case report and literature review

BackgroundScrub typhus is an acute infectious disease caused by Orientia tsutsugamushi. Guillain–Barre syndrome (GBS) is an autoimmune-mediated peripheral neuropathy with a frequent history of prodromal infections, but GBS associated with scrub typhus is very rare.Case presentationWe report a 51-year-old male patient who developed dysarthria and peripheral facial paralysis following the cure of scfrub typhus. CSF examination and electrophysiological findings suggested a diagnosis of GBS. After treatment with intravenous immunoglobulin, the patient’s neurological condition improved rapidly.ConclusionsScrub typhus infection is likely to be a potential predisposing factor in GBS, while scrub typhus-associated GBS has a favorable prognosis.

Guillain-Barré syndrome after surgery: a literature review.

Guillain-Barré syndrome (GBS) is a rare postoperative complication that is sometimes characterized by serious motor weakness and prolonged weaning from mechanical ventilation. Although the exact nature of the relationship between GBS and the surgical procedure is still unclear, there is a clear increased incidence of GBS in post-surgical patients compared to non-surgical patients. GBS after surgery is unique in several ways. The course of post-surgical GBS unfolds more rapidly than in other situations where GBS develops, the condition is often more severe, and respiratory muscles are more commonly involved. Prompt diagnosis and appropriate treatment are essential, and the condition can worsen if treated inappropriately. Postoperative sedation, intubation, and restraint use make the diagnosis of GBS difficult, as the onset of symptoms of weakness or numbness in those contexts are not obvious. GBS is often misdiagnosed, being attributed to other postoperative complications, and subsequently mishandled. The lack of relevant information further obscures the clinical picture. We sought to better understand post-surgical GBS by performing an analysis of the relevant literature, focusing on clearly documenting the clinical characteristics, diagnosis, and management of GBS that emerges following surgery. We underscore the importance of physicians being aware of the possibility of GBS after major surgery and of performing a variety of laboratory clinical investigations early on in suspected cases.

Clinical characteristics of Guillain–Barré syndrome in patients with primary Sjögren’s syndrome

To investigate the clinical characteristics of Guillain–Barré syndrome (GBS) in patients with primary Sjögren’s syndrome (SS). Records of patients with positive anti-SSA antibodies hospitalized in the Beijing Tiantan Hospital between December 2011 and May 2020 were retrieved. Patients who fulfilled the criteria for diagnosis of GBS and primary SS were included, and their clinical data were analyzed. Among the 785 patients with positive anti-SSA, 52 patients were identified in this study. They were 27 males and 25 females with median age of 59 years old. Besides anti-SSA antibodies, multiple autoantibodies were detected in these patients including antinuclear antibody, anti-Ro52, anti-mitochondrial M2, anti-thyroid peroxidase and anti-thyroglobulin autoantibodies. Preceding infection was reported in 42 patients. Hyporeflexia/areflexia and limbs weakness were the most common manifestation and 35 patients presented cranial nerve injuries. GBS disability score of 3, 4 and 5 was scaled in 28 (53.8%), 15 (28.8%) and 3 (5.8%) patients respectively. Forty-six patients received intravenous immunoglobulin (IVIG) monotherapy, 5 patients were treated by IVIG plus glucocorticoids, and 51 patients improved during hospitalization. The frequency of male gender among the patients with both GBS and primary SS suggests an independent onset of GBS and the co-existence of these autoimmune diseases in patients with multiple autoantibodies. Majority of patients with GBS and primary SS experience benign disease course.

Pregnancy-related Guillain-Barré syndrome: A Case Series

Introduction: Pregnancy-related Guillain-Barré syndrome (GBS) is a rare autoimmune disorder that affects pregnant women. With an annual incidence ranging from 0.81 to 1.89 cases per 100,000 population, GBS can occur at any trimester of pregnancy, as well as during the postpartum period are susceptible to GBS. The pillars of managing pregnancy-related GBS to improve outcomes include early diagnosis, prompt immune-modulatory therapy, and multidisciplinary input. Case Series: In this study, three case of GBS in pregnancy were reported. The first patient was a 35-year-old woman, G3A1P2 post emergency Transperitoneal Cesarean Section (TPCS), who experienced with lower limb weakness three days before TPCS. After being diagnosed with severe eclampsia and underwent emergency TPCS, her complaint of lower limb weakness worsened. The second patient, a 27-year-old woman, with G2P1A0 experienced weakness in all four limbs. The third patient, a 20-year-old woman with G1P0A0, in the third semester presented with weakness in all four limbs. The electroneurography investigation conducted on these patients supported the diagnosis of GBS, which was subsequently managed with plasma exchange (PE). After the administration of PE, there was observed improvement in the clinical manifestation of GBS. Conclusion: The development of GBS in pregnancy is typically preceded by bacterial or viral infection. Preeclampsia was found to be associated with two folds risk of GBS, which was usually diagnosed based on the neurological examinations with supportive studies, including serological tests, cerebrospinal fluid analysis and electroneurography. The management of pregnancy-related GBS included intravenous immunoglobulin, PE, physiotherapy, and supportive therapy, such as ventilator support.

Cervical Myelopathy with Concomitant Guillain-Barré Syndrome: A Case Report.

We present a case of a 67-year-old female patient with concomitant cervical spondylotic myelopathy (CSM) and Guillain-Barré syndrome (GBS). Surgical intervention, including cervical decompression and arthrodesis, was performed to address cervical myelopathy symptoms. Despite initial improvement, the patient's motor function deteriorated, leading to the diagnosis of GBS. The patient's hospital course was protracted with a complicated recovery. This case emphasizes the clinical details of coexisting CSM and GBS, highlighting the importance of diagnosing and considering demyelinating diseases when determining the optimal timeline for surgical intervention. These findings inform decision-making for clinicians encountering similar patient presentations.

Sural nerve involvement in patients with acute inflammatory demyelinating polyneuropathy variant of Guillain-Barre syndrome with sural sparing at initial presentation

Introduction: Neurophysiological testing is a valuable tool in the diagnosis of Guillain-Barre syndrome (GBS). Sural sparing is a usual feature of acute inflammatory demyelinating polyneuropathy (AIDP) type GBS. However, sural involvement has been reported in later stages of GBS. It is important to identify patterns of sural nerve involvement to differentiate GBS from its mimickers and to stage the disease. This research aimed to detect the pattern of sural nerve involvement in AIDP-GBS cases with normal electrophysiological responses in the sural nerve at the beginning.Objectives: To determine the location and timing of sural nerve involvement in AIDP-GBS.Methods: This prospective follow up study included diagnosed cases of AIDP-GBS with preserved bilateral sural responses. Nerve conduction and somatosensory evoked potentials (SSEP) were done on admission and weekly thereafter for four consecutive weeks. The last evaluation was done four weeks after the fourth study.Results: All patients (100%) showed normal distal sural responses over the initial four weeks of follow up. They continued to remain normal up to eight weeks in eight patients (53.3%). Two patients had gradual prolongation of their sural SSEP on consecutive studies. One of them had gradual reduction of sural sensory nerve action potential and nerve conduction velocity along with the prolongation of sural SSEP latencies. The difference of SSEP latency increments in the left sural nerve of these two patients was statistically significant (p<0.05). The right sural SSEP latency difference was not significant.Conclusion: Sparing of the distal sural sensory response was demonstrated in 100% of AIDP-GBS cases during the first four weeks of follow up. More than 50% of the cohort demonstrated preserved sural sensory responses for eight weeks from the initial presentation. Two out of fifteen patients showed statistically significant proximal sural sensory pathway involvement with increasing SSEP latencies. This finding suggests that in some patients, the sural sensory pathway may get affected at its proximal segments or at the central nervous system before the distal nerve is affected.

Utility of cerebrospinal fluid protein biomarkers in predicting the outcome of Guillain-Barre syndrome

Objectives The study aimed to investigate the potential of peripheral myelin protein 2 (P2) and Alpha B-crystallin (αBC) as predictive biomarkers in Guillain-Barré syndrome (GBS). Given the unpredictability of GBS prognosis, the need for specific and reliable biomarkers for disease development and intensity assessment is crucial. Material and Methods A prospective observational study was conducted on a cohort of 220 individuals diagnosed with GBS at a tertiary general hospital in South India. P2 and αBC levels in cerebrospinal fluid (CSF) were quantified using ELISA assay kits. The study spanned from March 2021 to April 2023, with participants aged 18–60 years. The study protocol adhered to ethical standards, and the Brighton criteria were employed for GBS diagnosis. CSF samples were collected at admission and two weeks post-onset. Data analysis utilised SPSS, and statistical significance was set at p < 0.05. Results Upon admission, mean P2 levels were 2.2 ± 0.5 ng/mL, and αBC levels were 9.8 ± 2.3 ng/mL. After two weeks, P2 increased to 4.8 ± 0.8 ng/mL, and αBC increased to 15.1 ± 2.3 ng/mL. A positive correlation was observed between the rise in P2 and αBC levels and enhanced muscle strength at 4 weeks and 6 months. Conclusion The study suggests a significant increase in P2 and αBC levels in GBS patients, correlating with improved muscle strength. P2 and αBC ratios in CSF between the second and first weeks may serve as prognostic markers for GBS. Limitations include a small sample size and the absence of a control group, necessitating caution in generalizability.

Injudicious use of cranio-spinal imaging in Guillain-Barré syndrome in a low resource country.

Background: Many patients being investigated for Guillain-Barré syndrome (GBS) undergo unnecessary neuroimaging. The objective of this study was to determine the proportion of patients with GBS undergoing neuroimaging investigation, and to investigate any association with different GBS variants using the Brighton criteria. Methods: This cross-sectional observational study was conducted in the leading tertiary care hospital in Pakistan; 148 patients being investigated for, and subsequently diagnosed with GBS between January 2017 and March 2020 were enrolled. Participants were asked if they had undergone neuroimaging of the craniospinal axis before or during hospital admission, and the purpose of any computed tomography (CT) scan was investigated. We enquired whether fundoscopy had been performed before lumbar puncture (LP) and determined the level of certainty based on the Brighton criteria. Results: The majority of participants were men (n = 107, 73%), with a mean age of 42.85 ± 18.40 years. The mean waiting time to their first interaction with a neurologist was 5.20 ± 4.01 days, and the demyelinating variant of GBS was more common than the axonal variant (1.6:1). Most patients were diagnosed with level I certainty using the Brighton criteria (n = 113, 76%). Brain and spine magnetic resonance imaging (MRI) were performed ahead of admission in 48 (32%) and 59 (39%) patients, respectively. Brain CT scan was performed in 121 (82%) patients before LP, while 27 (18%) only underwent fundoscopic examination before LP. Conclusion: Clinical examination is fundamental in the diagnosis of GBS. Neuroimaging may be inappropriate and unnecessary, and may detract attention from crucial peripheral neuropathy measures while misusing limited resources.

DAMAGE TO THE PERIPHERAL NERVOUS SYSTEM IN COVID-19 INFECTION

The article provides information about damage to the peripheral nervous system during COVID-19 infection and clarifies the possible mechanisms of development. Also in modern medical literature, a large amount of data on neurological pathologies associated with COVID-19 - damage to the central and peripheral nervous system - has been reviewed and epidemiological indicators of these injuries have been analyzed. The role of immune disorders in the occurrence and further development of damage to the peripheral nervous system was assessed. Guillain-Barre syndrome (GBS), one of the rare neurological pathologies that develops due to COVID-19, is described. Literary data were analyzed to determine the clinical course of GBS and elucidate its probable autoimmune pathogenesis. A clinical observation of the development of GBS against the background of typical manifestations of COVID-19 in a 56-year-old male patient is presented. The interval from the first manifestations of COVID-19 to the manifestation of GBS was 13 days. The GBS that developed was severe, required invasive ventilation, and developed acute kidney injury. It is suggested that the information presented about the pathogenetic mechanism, clinical manifestations, diagnosis and treatment of GBS in patients may stimulate research towards bringing greater clarity to these issues.

Predictors of Treatment Outcome and Clinical Profile among Guillain- Barre Syndrome Patients in South India.

Guillain-Barre syndrome (GBS) is one of the principal causes of acute neuromuscular weakness and paralysis worldwide. Its clinic-epidemiological profile and factors influencing its treatment outcomes in developing countries are very minimally studied. The study aimed to find out the risk factors, clinical presentation, management, and predictors of treatment outcomes among GBS patients admitted in two tertiary care hospitals. Medical records of 121 inpatients with GBS confirmed based on the Brighton criteria over the recent five-year period from June 2017 to May 2022 were examined. Assessment of the severity of GBS was done using the Hughes functional grading scale. The mean age at onset was 36.8 ± 18.9 years. The majority of the patients [82 (67.8%)] were males. Antecedent illnesses within 1 month of onset of GBS were present among 34 (28.1%) patients. The majority of them developed respiratory tract illnesses [13 (38.2%)]. Recurrent history of GBS was observed among 4 (3.3%) patients. The median time gap between the onset of antecedent illnesses and the onset of GBS was 5 days (IQR 3, 10). The most common symptom among GBS patients was the weakness of the muscles of the extremities [117 (96.7%)]. The pattern of progression of weakness among 53 (45.3%) of these patients was from the lower to upper limbs. The most common sign noted was hypotonia [64(52.9%)]. Complications due to GBS were observed among 12 (9.9%) patients. The most common complication among them was respiratory distress in 11 (91.7%) patients, followed by autonomic dysfunctions in 8 (66.7%). Albuminocytological dissociation in cerebrospinal fluid was noted among 48 (39.7%) patients. The majority of patients in nerve conduction studies had acute inflammatory demyelinating polyneuropathy [61(50.4%)]. The majority of the GBS patients [68 (56.2%)] were treated using intravenous immunoglobulin (IVIG). 95 (78.5%) patients improved with treatment at the time of discharge. In multivariable analysis, the absence of antecedent illnesses (p =0.029), Brighton's diagnostic certainty levels 1 and 2 of GBS (p =0.024), and being on IVIG treatment (p =0.05) were associated with improvement in disease condition among the patients. Appropriate diagnosis of GBS using both clinical and laboratory evidence and providing appropriate treatment along with more supervision among GBS patients with a history of antecedent illnesses will help improve their prognosis at the time of discharge.

Association Between Guillain-Barré Syndrome and COVID-19 Infection and Vaccination: A Population-Based Nested Case-Control Study.

Existing data regarding occurrence of Guillain-Barré syndrome (GBS) after coronavirus disease 2019 (COVID-19) infection and vaccination are inconclusive. We aimed to assess the association between GBS and both severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection and COVID-19 vaccine. We conducted a nested case-control study in a cohort of 3,193,951 patients aged 16 years or older, without a diagnosis of prior GBS, from the largest health care provider in Israel. Participants were followed from January 1, 2021, until June 30, 2022, for the occurrence of GBS. Ten randomly selected controls were matched to each case of GBS on age and sex. We assessed both SARS-CoV-2 infection and COVID-19 vaccine administration in the prior 6 weeks in cases and controls. Overall, 76 patients were diagnosed with GBS during follow-up and were matched to 760 controls. A positive test for SARS-CoV-2 was detected in 9 (11.8%) cases and 18 (2.4%) controls. An administration of COVID-19 vaccine was detected in 8 (10.5%) cases (all Pfizer-BioNTech [BNT162b2] vaccine) and 136 (17.9%) controls (134 Pfizer-BioNTech vaccine). Multivariable conditional logistic regression models showed that the odds ratio for GBS associated with SARS-CoV-2 infection and COVID-19 vaccine administration was 6.30 (95% CI 2.55-15.56) and 0.41 (95% CI 0.17-0.96), respectively. The results were similar when exposure to SARS-CoV-2 infection or COVID-19 vaccine administration was ascertained in the prior 4 and 8 weeks, although did not reach statistical significance for COVID-19 vaccine at 4 weeks. Our study suggests that SARS-CoV-2 infection is associated with increased risk of GBS, whereas Pfizer-BioNTech COVID-19 vaccine is associated with decreased risk of GBS.

European Academy of Neurology/Peripheral Nerve Society Guideline on diagnosis and treatment of Guillain-Barré syndrome.

Guillain-Barré syndrome (GBS) is an acute polyradiculoneuropathy. Symptoms may vary greatly in presentation and severity. Besides weakness and sensory disturbances, patients may have cranial nerve involvement, respiratory insufficiency, autonomic dysfunction and pain. To develop an evidence-based guideline for the diagnosis and treatment of GBS, using Grading of Recommendations, Assessment, Development and Evaluation (GRADE) methodology, a Task Force (TF) of the European Academy of Neurology (EAN) and the Peripheral Nerve Society (PNS) constructed 14 Population/Intervention/Comparison/Outcome questions (PICOs) covering diagnosis, treatment and prognosis of GBS, which guided the literature search. Data were extracted and summarised in GRADE Summaries of Findings (for treatment PICOs) or Evidence Tables (for diagnostic and prognostic PICOs). Statements were prepared according to GRADE Evidence-to-Decision (EtD) frameworks. For the six intervention PICOs, evidence-based recommendations are made. For other PICOs, good practice points (GPPs) are formulated. For diagnosis, the principal GPPs are: GBS is more likely if there is a history of recent diarrhoea or respiratory infection; CSF examination is valuable, particularly when the diagnosis is less certain; electrodiagnostic testing is advised to support the diagnosis; testing for anti-ganglioside antibodies is of limited clinical value in most patients with typical motor-sensory GBS, but anti-GQ1b antibody testing should be considered when Miller Fisher syndrome (MFS) is suspected; nodal-paranodal antibodies should be tested when autoimmune nodopathy is suspected; MRI or ultrasound imaging should be considered in atypical cases; and changing the diagnosis to acute-onset chronic inflammatory demyelinating polyradiculoneuropathy (A-CIDP) should be considered if progression continues after 8 weeks from onset, which occurs in around 5% of patients initially diagnosed with GBS. For treatment, the TF recommends intravenous immunoglobulin (IVIg) 0.4 g/kg for 5 days, in patients within 2 weeks (GPP also within 2-4 weeks) after onset of weakness if unable to walk unaided, or a course of plasma exchange (PE) 12-15 L in four to five exchanges over 1-2 weeks, in patients within 4 weeks after onset of weakness if unable to walk unaided. The TF recommends against a second IVIg course in GBS patients with a poor prognosis; recommends against using oral corticosteroids, and weakly recommends against using IV corticosteroids; does not recommend PE followed immediately by IVIg; weakly recommends gabapentinoids, tricyclic antidepressants or carbamazepine for treatment of pain; does not recommend a specific treatment for fatigue. To estimate the prognosis of individual patients, the TF advises using the modified Erasmus GBS outcome score (mEGOS) to assess outcome, and the modified Erasmus GBS Respiratory Insufficiency Score (mEGRIS) to assess the risk of requiring artificial ventilation. Based on the PICOs, available literature and additional discussions, we provide flow charts to assist making clinical decisions on diagnosis, treatment and the need for intensive care unit admission.

European Academy of Neurology/Peripheral Nerve Society Guideline on diagnosis and treatment of Guillain-Barré syndrome.

Guillain-Barré syndrome (GBS) is an acute polyradiculoneuropathy. Symptoms may vary greatly in presentation and severity. Besides weakness and sensory disturbances, patients may have cranial nerve involvement, respiratory insufficiency, autonomic dysfunction and pain. To develop an evidence-based guideline for the diagnosis and treatment of GBS, using Grading of Recommendations, Assessment, Development and Evaluation (GRADE) methodology a Task Force (TF) of the European Academy of Neurology (EAN) and the Peripheral Nerve Society (PNS) constructed 14 Population/Intervention/Comparison/Outcome questions (PICOs) covering diagnosis, treatment and prognosis of GBS, which guided the literature search. Data were extracted and summarised in GRADE Summaries of Findings (for treatment PICOs) or Evidence Tables (for diagnostic and prognostic PICOs). Statements were prepared according to GRADE Evidence-to-Decision (EtD) frameworks. For the six intervention PICOs, evidence-based recommendations are made. For other PICOs, good practice points (GPPs) are formulated. For diagnosis, the principal GPPs are: GBS is more likely if there is a history of recent diarrhoea or respiratory infection; CSF examination is valuable, particularly when the diagnosis is less certain; electrodiagnostic testing is advised to support the diagnosis; testing for anti-ganglioside antibodies is of limited clinical value in most patients with typical motor-sensory GBS, but anti-GQ1b antibody testing should be considered when Miller Fisher syndrome (MFS) is suspected; nodal-paranodal antibodies should be tested when autoimmune nodopathy is suspected; MRI or ultrasound imaging should be considered in atypical cases; and changing the diagnosis to acute-onset chronic inflammatory demyelinating polyradiculoneuropathy (A-CIDP) should be considered if progression continues after 8 weeks from onset, which occurs in around 5% of patients initially diagnosed with GBS. For treatment, the TF recommends intravenous immunoglobulin (IVIg) 0.4 g/kg for 5 days, in patients within 2 weeks (GPP also within 2-4 weeks) after onset of weakness if unable to walk unaided, or a course of plasma exchange (PE) 12-15 L in four to five exchanges over 1-2 weeks, in patients within 4 weeks after onset of weakness if unable to walk unaided. The TF recommends against a second IVIg course in GBS patients with a poor prognosis; recommends against using oral corticosteroids, and weakly recommends against using IV corticosteroids; does not recommend PE followed immediately by IVIg; weakly recommends gabapentinoids, tricyclic antidepressants or carbamazepine for treatment of pain; does not recommend a specific treatment for fatigue. To estimate the prognosis of individual patients, the TF advises using the modified Erasmus GBS outcome score (mEGOS) to assess outcome, and the modified Erasmus GBS Respiratory Insufficiency Score (mEGRIS) to assess the risk of requiring artificial ventilation. Based on the PICOs, available literature and additional discussions, we provide flow charts to assist making clinical decisions on diagnosis, treatment and the need for intensive care unit admission.

Case study of Guillain-Barré syndrome with acute flaccid paralysis

Guillain-Barre syndrome (GBS) is a sudden and a complicated degenerative neurological disorder which can be acute or chronic in nature. Etiology remains unclear, but pathophysiology shows demyelination of spinal nerve roots characterised by symmetrical limb weakness and muscle stiffness. Death is rare. Early diagnosis and prompt treatment should occur in severe cases. Here we discuss a rare case of GBS as paraesthesia and acute flaccid paralysis. This case report focuses on a 9-year-old male patient presenting with c/o progressive muscle weakness and paraesthesia in the lower extremities. The clinical examination revealed bilateral lower limb weakness, absent deep tendon reflexes, and diminished vibration sense, acute flaccid paralysis. Diagnostic evaluations of CT scan of brain and spine and other preliminary tests confirmed the diagnosis of GBS by demonstrating characteristic findings of demyelinating polyneuropathy and elevated protein levels without pleocytosis, respectively. Prompt initiation of intravenous immunoglobulin therapy and supportive measures, such as physical therapy, were implemented. Over a four-week period, the patient showed progressive improvement in muscle strength, starting from the slight movement in fingers and toes. This case report underscores the importance of accurate diagnosis, and timely initiation of appropriate treatment strategies in managing GBS, highlighting the efficacy of intravenous immunoglobulin therapy as a first-line intervention for GBS patients.

Vaccine patterns among patients diagnosed with Guillain-Barré Syndrome and matched counterparts in a Medicare supplemental population, 2000–2020

Some vaccines have a small risk of Guillain-Barré Syndrome (GBS), a rare autoimmune disorder characterized by paralysis if untreated. The CDC’s Advisory Committee on Immunization Practices (ACIP) guidelines do not consider GBS a precaution for future vaccines unless GBS developed within six weeks after a tetanus-toxoid–containing vaccine or influenza vaccine. Our goal was to describe vaccine patterns before and after GBS diagnosis. We matched each of 709 patients diagnosed with GBS from 2002 to 2020 with Medicare supplemental insurance to 10 counterparts without GBS (1:10) on age and sex. Propensity score-based weighting balanced covariates between groups, and we estimated weighted mean cumulative counts (wMCC) of vaccines/person before and after GBS diagnosis. Among patients with GBS, 7% were diagnosed within 42 days after a vaccine. Prior to GBS diagnosis, the wMCC of vaccines per person was similar between GBS cases and matched counterparts, but after two years of follow-up, GBS patients received 21 fewer vaccines/100 people than counterparts (wMCC difference −0.21 vaccines/person, 95% CI −0.24 to −0.18); GBS patients received 16 vaccines/100 people while matched counterparts received 36/100. Vaccine use was reduced following GBS diagnosis despite no ACIP precaution for most (93%) patients in this study. The observed drop in vaccines after GBS diagnosis indicates a disconnect between clinical practice and current recommendations.