Guillain-Barré Syndrome Disability Score Research Articles

Physiotherapy Management of Guillain-Barré Syndrome in a Tertiary Hospital in South-South Nigeria: A Case Report.

Background: Guillain-Barré Syndrome (GBS) is a potentially life-threatening post infectious disease characterized by rapidly progressive, symmetrical weakness of the extremities. It can cause life-threatening complications, particularly if the breathing muscles are affected or if there is dysfunction of the autonomic nervous system. Case Presentation: This is a case report of Physiotherapy management of a 51-year old female patient with Guillain-Barré Syndrome (GBS). Patient was referred from the neurology unit, department of Internal Medicine, University of Port Harcourt Teaching Hospital (UPTH) to the neurology clinic of the physiotherapy department, UPTH as a case GBS after series of investigations. Appropriate physiotherapy intervention was given for a period of 36 weeks after a thorough assessment of the patient. The Guillain-Barre Syndrome Disability Score was used to assess the overall functional status of the patient while the Oxford Muscle Grading Scale was used to assess for muscle strength. The Numerical Pain Rating Scale (NPRS) was used to assess the level of pain in the limbs while the Berge Balance Scale (BBS) was used to assess the patient’s balance. The case report was presented in line with the CARE: Case Report Guideline for case reports. All ethical procedures were followed. Treatment outcome: Treatment outcome revealed that the Guillain-Barré Syndrome Disability Score reduced from 4 to 2 while that of Berg Balance Scale increased from 3 to 42 after 36 weeks of intervention. Pain level on range of motion of the limbs based on the Numerical Pain Rating Scale reduced from 6/10 to 1/10 on the upper limb and from 8/10 to 2/10 on the lower limbs. Conclusion: Physiotherapy is effective in the management of patients with Guillain-Barré Syndrome. Early referral to Physiotherapy is key to pain management, balance control and overall functional status of the patients.

Comparison of Clinical Features, Short-Term Outcome of Guillain-Barré Syndrome Between Adults and Children: A Retrospective Study in Vietnam

BackgroundDespite extensive research on Guillain-Barré syndrome (GBS) in adults and children, there is a lack of comparison regarding short-term outcomes in various age groups. Our study aims to elucidate the differences in clinical features and short-term outcomes in Vietnam. MethodsAfter retrospective data collection, we compared clinical features in patients with GBS aged ≤16 years at Children's Hospital 2 and aged >16 years at University Medical Center Ho Chi Minh City from 2017 to 2021. A positive short-term outcome was recorded if patients had a GBS Disability Score of 0 to 2 at hospital discharge. ResultsWe analyzed 109 adults (58.7% males; mean age 50.6 ± 17.7) and 111 children (58.6% males; mean age 7.2 ± 4.9). Comparable antecedent infection and immunization incidence in both groups were observed (35.8% vs 45.9%, P > 0.05). Pain and sensory disturbance were the most common onset symptom in adults (57.8%), whereas lower limb weakness predominated in children (61.3%). Ophthalmoplegia (18.3% vs 5.4%), pain, sensory disturbance (85.3% vs 67.6%), ataxia (33.0% vs 15.3%) were more prevalent in adults (P < 0.05). The axonal subtype was prominent in both adults (51.4%) and children (53.2%). Patients were classified into: classic GBS (49.5% and 68.5%), GBS variants (11.0% and 15.3%), classic Miller Fisher syndrome (MFS) (1.8% and 1.8%), MFS variants (2.8% and 0%), and GBS/MFS overlap (34.9% and 14.4%). Short-term outcomes did not significantly differ based on age. ConclusionsAge-related variations in clinical features were observed, but adults and children exhibited similar short-term functional outcomes.

Takotsubo cardiomyopathy in Guillain-Barré syndrome.

Takotsubo cardiomyopathy (TCM) is a serious autonomic complication of Guillain-Barré syndrome (GBS). However, the association between TCM and GBS has not been investigated in detail. We investigated the characteristics of GBS patients with TCM (GBS-TCM). Clinical features and anti-ganglioside antibody between the GBS-TCM patients and 62 classical GBS patients without TCM as control patients were compared. Eight GBS-TCM patients were identified, in whom TCM was diagnosed at a mean of 6.5 [range 3-42] days after the onset of GBS. The age at onset of GBS was elder in the GBS-TCM patients than in the control GBS patients (76.5 [56-87] vs. 52 [20-88] years, p < 0.01). Notably, cranial nerve deficits, particularly in the lower cranial nerves, were observed in all GBS-TCM patients (100% vs. 41.9%, p < 0.01). Additionally, the GBS-TCM patients showed a higher GBS disability score at nadir (5 [4-5] vs. 4 [1-5], p < 0.01), and lower Medical Research Council sum scores at admission and nadir (37 [30-44] vs. 48 [12-60] at admission, p < 0.05, and 20 [12-44] vs. 40 [0-60] at nadir, p < 0.05, respectively). Mechanical ventilation was more frequently required in the GBS-TCM patients (62.5% vs. 11.3%, p < 0.01). Three GBS-TCM patients were positive for anti-ganglioside antibodies. TCM occurred at a relatively early phase of GBS. The characteristics of GBS-TCM were the elder, lower cranial nerve involvements, severe limb weakness, and respiratory failure.

Mild Form of Guillain Barre Syndrome, Clinical Characteristics and Outcome with or Without Plasmapheresis: A Local Experience

Background: Guillain-Barre syndrome is an immune mediated poly-radiculo-neuropathy with a variable clinical presentation and prognosis. Plasma exchange and Intravenous immunoglobulin are treatment options with proven efficacy by various clinical trials. But these trials demonstrate improvement in a classical and moderate to severe GBS. In clinical setting, problem may occur in treatment of those patients who present with mild symptoms &amp; signs, clinical variants of GBS, or when the duration of weakness is &gt; 2 weeks from onset of symptoms. Objective: To determine the frequency of mild GBS, its clinical characteristics &amp; to compare clinical outcome of patients treated with or without plasmapheresis. Methods: Prospective, Quasi experimental study was done in Neurology, MHL from June, 2020 to June, 2022. Diagnosis of mild GBS was made on basis of patient ability to walk without support/ GBS disability score is ≤ 2. Outcome in these patients was assessed by using MRC sum score at presentation one and 3month post treatment. Results: Patients with diagnosis of GBS (n=154) were admitted and treated during 2years.out of these patients, 22(14.28%) were diagnosed as mild GBS, 18 males and 4 females. MRC Sum score at presentation 18.5, at 1-month 19.7 and at 3 month it was 19.5 on average. MRC sum score improvement noted in patients treated with plasma-pheresis at one and three months with p value less than 0.01. There was no difference in recovery with increased number of sessions of plasma-pheresis 3 versus 5 sessions at one month and 3months with p value 0.18 &amp;0.32 respectively. Conclusion: Mild GBS is not uncommon, remained under-reported in clinical scenario. Plasma-pheresis seems to have a beneficial role in mild form of GBS

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.

Clinical and laboratory findings in scrub typhus associated Guillain-Barré syndrome in South Korea.

Scrub typhus is an endemic disease in the fall season that occurs in a limited number of places known as the Tsutsugamushi Triangle. Peripheral neuropathy is a common complication of scrub typhus. Herein, we encountered several patients with ascending paralysis after scrub typhus infection, who were diagnosed with Guillain-Barré syndrome (GBS). We aimed to investigate the clinical and laboratory characteristics of patients who developed GBS after scrub typhus. Patients were retrospectively recruited from six nationwide tertiary centers in South Korea from January 2017 to December 2021. Patients who had been clinically diagnosed with GBS and confirmed to have scrub typhus via laboratory examination and/or the presence of an eschar before the onset of acute limb paralysis were included. The GBS-associated clinical and electrophysiological characteristics, outcomes, and scrub typhus-associated features were collected. Of the seven enrolled patients, six were female and one was male. The median time from scrub typhus infection to the onset of limb weakness was 6 (range: 2-14) days. All patients had eschar on their bodies. Four patients (57.1%) were admitted to the intensive care unit and received artificial ventilation for respiratory distress. At 6 months, the median GBS disability score was 2 (range, 1-4) points. Patients with scrub typhus-associated GBS have a severe clinical presentation and require intensive treatment with additional immunotherapies. Therefore, GBS should be included in the differential diagnosis when peripheral neuropathies develop during scrub typhus treatment. Notably, scrub typhus is associated to GBS.

Construction and evaluation of a prognostic prediction model based on the mEGOS score for patients with Guillain-Barré syndrome

BackgroundGuillain-Barré syndrome (GBS) is an immune-mediated acute peripheral neuropathy in which up to 20% patients remain unable to walk independently after 6 months of onset. This study aimed to develop a clinical prognostic model based on the modified Erasmus GBS Outcome Score (mEGOS) for predicting the prognosis of GBS patients at 6 months of onset.MethodsThe clinical data of 201 GBS patients were retrospectively analyzed. According to the GBS disability score (GBS-DS) at 6 months of onset, patients were divided into a good prognosis group (GBS-DS &amp;lt;3 points) and a poor prognosis group (GBS-DS≥3 points). Univariate and multivariate analysis was used to screen out independent risk factors for poor prognosis, and a prediction model was accordingly constructed for GBS prognosis.ResultsThe mEGOS score, serum albumin (ALB) and fasting plasma glucose (FPG) were independent risk factors for poor prognosis in patients with GBS, and the above risk factors were used to construct a prognostic model of mEGOS-I and a nomogram. The receiver operating characteristic (ROC) curve showed that the area under curve (AUC) of mEGOS-I at admission and at 7 days of admission to predict poor prognosis at 6 months of GBS onset was 0.891 and 0.916, respectively, with sensitivities of 82.7% and 82.6% and specificities of 86.5% and 86.6%, respectively. Decision curve analysis showed that the nomogram had a very high clinical benefit.ConclusionTo our knowledge, this is the first report of the construction of a prognostic prediction model based on the mEGOS score, ALB, and FPG that can accurately and stably predict the prognosis of GBS patients at 6 months of onset.

Predicting of Mechanical Ventilation and Outcomes by Using Models and Biomarker in Guillain-Barré Syndrome.

Guillain-Barré syndrome (GBS) is an acute inflammatory neuropathy. The Erasmus GBS Respiratory Insufficiency Score (EGRIS) and the modified Erasmus GBS Outcome Score (mEGOS) are prognostic models used in the prediction of mechanical ventilation and outcome. Thus far, there are only few biomarkers for the prognosis prediction of GBS patients, and albumin level is one that is promising. Patients diagnosed with GBS from 2013 to 2022 at Renmin Hospital, Wuhan University, China, were included. Patients hospitalized between 2016 and 2022 underwent short- and long-term follow-ups. The correlations between EGRIS/mEGOS and mechanical ventilation and outcome were evaluated. Serum albumin level was examined the day after admission. Furthermore, we also investigated whether the level of serum albumin was useful in predicting disease severity or poor outcome. In all, 145 patients were enrolled. Nineteen patients (13.1%) who required mechanical ventilation had higher Hughes GBS disability score (HGDS) at admission and discharge (P < 0.05 and P < 0.0001, respectively), shorter time from onset to admission and treatment (P < 0.01 and P < 0.001, respectively) and longer hospital stays (P < 0.001) than patients who did not require mechanical ventilation. High EGRIS scores were linked with the need for mechanical ventilation (r = 0.427, P < 0.001, AUC = 0.623). Seventy-one patients were admitted between 2016 and 2022. Of these, 65 patients had a 4-week follow-up and 61 had a 6-month follow-up. Higher mEGOS scores at admission and 7days after admission significantly correlated with short- (P < 0.0001 and P < 0.0001) and long-term (P < 0.05 and P < 0.05) outcomes, respectively. No significant difference in outcome was found between different subtypes (4weeks [P = 0.099] and 6months [P = 0.172]). Patients with lower albumin level tended to have higher HGDS (at admission P < 0.05, at nadir P < 0.001, and at discharge P < 0.001) and higher properties of the need of mechanical ventilation (P < 0.05) and ICU stay (P < 0.05) than those with normal albumin levels. Those with low albumin levels were also unable to walk independently at 6months (P < 0.01). mEGOS scores predicted the outcomes of GBS patients in China, and EGRIS score predicted the need for mechanical ventilation in these patients. Albumin level at admission correlated well with disease severity and outcomes.

SERUM ALBUMIN: AN INDEPENDENT INDICATOR IN GUILLAIN–BARRE SYNDROME PATIENTS RECEIVING TREATMENT OF PLASMAPHERESIS

This study aimed to assess the role of serum albumin as an independent indicator in Guillain–Barre Syndrome patients receiving plasmapheresis treatment. This descriptive case series was conducted at Hayatabad Medical Complex Peshawar, Pakistan, from December 2022 to May 2023. Total 80 patients with GBS syndrome. Patients were divided into two groups equally. Group A patients had lower serum albumin, and group B had normal serum albumin. In both groups, serum albumin as an independent indicator was assessed. Chi-square and Independent T-tests were applied in both groups, keeping the P value less than 0.05. The mean age of the patients was 31.90±8.63 years. The frequency of male patients was 62.5%, while female patients were 37.5%. In group A, 27.5% of patients had good outcomes, while 75.5% had poor outcomes, while in group B, 57.5% had good outcomes, and 42.5% had outcomes. The outcome in group B was significantly better than in group A (P = 0.007). In the normal serum albumin group, the Guillain-Barre syndrome disability score was significantly lower after receiving plasmapheresis treatment than in patients with low serum albumin levels.

Clinical characteristics and management outcomes of Guillain-Barré syndrome: eight-year experience at a tertiary center in jordan - a retrospective cohort study.

This retrospective study looks at adult patients admitted to a major tertiary referral hospital in the north of Jordan between 2013 and 2021. A total of 30 patients met the inclusion/exclusion criteria. Males were predominantly affected (70%) with a male-to-female ratio of 2.33. Acute inflammatory demyelinating polyradiculoneuropathy variant was encountered in 60% of cases, whereas axonal variants, namely, acute motor axonal neuropathy and acute motor axonal and sensory neuropathy variants were seen in about 23% of cases. ICU admission was reported in 37% of patients and 6.7% required mechanical ventilation. Most patients had a favorable outcome with a GBS disability score of three or better at out-patient follow-up visits. Our cohort of patients showed a significant deviation in disease expression from that reported in other parts of the globe. This deviation was obvious in more prominent male predominance, frequencies of different GBS variants, and more favorable short-term morbidity/mortality outcomes. However, larger multicenter prospective studies are needed for confirmation of these results.

Establishment of a single-center-based early prognostic scoring system for Guillain-Barré syndrome

BackgroundPrevious studies have developed clinical prognostic models for Guillain-Barré syndrome including EGOS and mEGOS, they have good reliability and accuracy, but individual entries are poor. This study aims to establish a scoring system to predict the early prognosis, in order to provide additional treatment for patients with poor prognosis and shorten the length of hospital stay.MethodsWe retrospectively analyzed risk factors affecting the short-term prognosis of Guillain-Barré syndrome, and developed a scoring system for early determination of disease prognosis. Sixty two patients were divided into two groups based on the Hughes GBS disability score at discharge. Groups were compared for differences in gender, age at onset, antecedent infection, cranial nerve involvement, pulmonary infection, mechanical ventilation support, hyponatremia, hypoproteinemia, impaired fasting glucose, and peripheral blood neutrophil-to-lymphocyte ratio. Statistically significant factors were included in a multivariate logistic regression analysis, and a scoring system to predict the short-term prognosis was established based on the regression coefficients. The receiver operating characteristic curve of this scoring system was plotted, and the area under the ROC curve was calculated to assess the accuracy of the prediction model.ResultsUnivariate analysis revealed that age at onset, antecedent infection, pneumonia, mechanical ventilation support, hypoalbuminemia, hyponatremia, impaired fasting glucose, and elevated peripheral blood neutrophil-to-lymphocyte ratio were risk factors for poor short-term prognosis. The above factors were included in the multivariate logistic regression analysis, and pneumonia, hypoalbuminemia, and hyponatremia could be used as independent predictors. The receiver operating characteristic curve was plotted with a calculated area under the ROC curve of 82.2% (95% CI 0.775—0.950, P < 0.0001). The best cut-off value for the model score was 2, with a sensitivity of 0.9091, a specificity of 0.7255, and a Youden index of 0.6346.ConclusionPneumonia, hyponatremia, and hypoalbuminemia were independent risk factors for poorer short-term prognosis in patients with Guillain-Barré syndrome. The short-term prognosis scoring system of Guillain-Barré syndrome we constructed using these variables had some predictive value, and the short-term prognosis with quantitative scores of 2 or more was worse.

Dysautonomia and related outcomes in Guillain-Barre syndrome

Guillain-Barre syndrome (GBS) presents an annual incidence of 1.2-2.3 per 100,000. Sympathetic and parasympathetic nervous systems' peripheral control of visceral organs is affected by GBS aberrant immune response. Associated cardiovascular, gastrointestinal, sudomotor, pupillary, and other systems disturbances cause significant morbidity and mortality. This study aims to evaluate the dysautonomia spectrum in GBS patients, its relationship with patient outcomes, and compare it with those without autonomic disturbances. We performed an ambispective review study of patients with GBS and dysautonomia admitted to the Institute of Neurology from 2017 to 2021. We recorded demographics, comorbidities, nerve conduction studies, clinical course, hospital complications, and functional outcomes. We included 214 patients, mean age 46.44±16.49years, 51 (31%) presented dysautonomia, hypertension in most of the patients 39 (84.8%), hypotension 35 (76.1%), tachycardia 35 (76.1%), enteric dysmotility 35 (76.1%), and need for vasopressor 27 (58.7%) were common characteristics. Twenty (39.2%) with a demyelinating form and twenty (39.2%) with an axonal motor form. The bivariate analysis report factors associated with dysautonomia, were lower cranial nerves (VII, IX, X) involvement (p=0.002), need for mechanical ventilation (p=0.0001) and intensive care (p=0.0001), higher mEGOS (p=0.05), EGRIS (p=0.004), GBS disability score (p=0.004), and delirium presence (p=0.001). Kaplan-Meier survival analysis showed that dysautonomic patients needed more days for the independent walk (p=0.004). There was no associated mortality. Autonomic dysfunction in GBS significantly affects the peripheral nervous system. With consequently worse functional results. Further investigation needs to clarify whether more aggressive treatment is beneficial in this category of GBS.

Validation of elevated levels of interleukin-8 in the cerebrospinal fluid, and discovery of new biomarkers in patients with GBS and CIDP using a proximity extension assay.

Biomarkers for diagnosis of inflammatory neuropathies, assessment of prognosis, and treatment response are lacking. CSF and EDTA plasma from patients with Guillain-Barré syndrome (GBS), chronic inflammatory demyelinating polyneuropathy (CIDP), healthy controls (HC) and disease controls were analyzed with Olink multiplex proximity extension assay (PEA) from two independent cohorts. Levels of interleukin-8 (IL8) were further analyzed with ELISA in patients with GBS, CIDP, paraproteinemia-related demyelinating polyneuropathy (PDN), multifocal motor neuropathy (MMN), HC and disease controls. ROC analysis was performed. Outcome was measured with the GBS-disability score (GBS-ds) or Inflammatory Neuropathy Cause and Treatment (INCAT) score. In CSF, multiplex PEA analysis revealed up-regulation of IL8 in GBS compared to CIDP and HC respectively, and CIDP compared to HC. In addition, levels of IL2RA were upregulated in GBS compared to both HC and CIDP, SELE in GBS compared to HC, and ITGAM, IL6, and NRP1 in GBS compared to CIDP. In plasma, levels of MMP3, THBD and ITGAM were upregulated in CIDP compared to HC. Validation of multiplex IL8 results using ELISA, revealed increased levels of IL8 in CSF in patients with GBS and CIDP versus HC and non-inflammatory polyneuropathies (NIP) respectively, as well as in PDN versus NIP and HC. Levels of IL8 in CSF correlated with impairment in the acute phase of GBS as well as outcome at 6-months follow up. IL8 in CSF is validated as a diagnostic biomarker in GBS and CIDP, and a prognostic biomarker in GBS. Multiplex PEA hereby identifies several potential biomarkers in GBS and CIDP.

Retrospective evaluation of Guillain-Barre syndrome in children: Asingle-center experience.

Although Guillain-Barré syndrome (GBS) is now the most common cause of acute flaccid paralysis in children, information on the long-term follow-up of GBS is still limited. Identification of prognostic factors can play an important role in treatment strategies and the follow-up of patients. This study aimed to evaluate the effectiveness of monitoring the GBS disability score (DS) in predicting morbidity and mortality. The patients were separated into two groups those with DS≥ or <3 on admission. These groups were compared in respect of demographic data, clinical and laboratory findings, and the DS recorded on admission and at first, third, sixth, 12th, and 24th months. The study included 44 patients (54.5% male, 45.5% female) with a median age of 5 years. The most common involvements during the disease were weakness, ataxia, neuropathic pain, cranial neuropathy, respiratory distress, autonomic dysfunction, and psychiatric symptoms, respectively. In patients with a DS of ≥3, the time from onset of symptoms to hospital admission was shorter, and the length of hospital stay was longer. Children with back pain and autonomic dysfunction had a DS of ≥3. A high 3-month DS was found to be a significant predictor for the development of sequelae. Although progressive muscle weakness and inability to walk are the most common symptoms of GBS, it should be kept in mind that atypical manifestations such as hemiplegia and ophthalmoplegia may also occur. For an objective assessment of clinical improvement during follow-up, the DS for motor functions can be used.

GUİLLAİN-BARRÉ SENDROMUNDA AĞIR ÖZÜRLÜLÜĞÜN ERKEN BELİRTEÇLERİ

Objective&#x0D; Guillain-Barré syndrome (GBS) is an autoimmune&#x0D; neurological disorder with non-negligible morbidity&#x0D; and mortality. This study aimed to evaluate different&#x0D; patient characteristics and laboratory findings as early&#x0D; predictors of severe disability in GBS patients.&#x0D; Material and Method&#x0D; We retrospectively reviewed the medical records of&#x0D; 121 patients diagnosed with GBS between January&#x0D; 1, 2018, and December 31, 2021. Data regarding&#x0D; demographic characteristics, presenting complaints,&#x0D; co-morbidities, previous infection history, neurological&#x0D; examination findings, GBS Disability Scores (GDS)&#x0D; on the 1st day and by the end of the first month,&#x0D; laboratory parameters of serological and cerebrospinal&#x0D; fluid (CSF) examination, electromyoneurography&#x0D; results, GBS subtypes, treatments, treatment-related&#x0D; complications, and prognoses were recorded.&#x0D; Results&#x0D; The median age of the 121 patients was 58 (20–87)&#x0D; years (n = 73 males, 60%). The average GDS was&#x0D; 3 on admission and 2 at the end of the first month.&#x0D; The serum C-reactive protein (CRP) and CSF protein&#x0D; levels were raised, while vitamin D levels were&#x0D; reduced. Advanced age, cranial nerve involvement,&#x0D; history of infection, admission to the intensive care&#x0D; unit (ICU), need for mechanical ventilation (MV),&#x0D; presence of complications, high plasma CRP levels,&#x0D; neutrophil-lymphocyte ratio (NLR), and plateletlymphocyte&#x0D; ratio (PLO) were significantly associated&#x0D; with severe disability in GBS patients at day 1 and at&#x0D; the end of the first month.&#x0D; Conclusion&#x0D; We identified multiple characteristics that can predict&#x0D; severe disability in GBS patients.

Serum C3 complement levels predict prognosis and monitor disease activity in Guillain-Barré syndrome

ObjectiveBiomarkers are needed to predict prognosis and disease activity in patients with Guillain-Barré syndrome (GBS). The complement system is a key player in the pathogenesis of GBS. This study aimed to assess the potential utility of serum complement proteins as novel biomarkers in GBS. MethodsWe reviewed the medical records of 76 GBS patients with C3 and C4 measurements during hospitalization between 2010 and 2021. Clinical outcomes were correlated with baseline serum C3, C4, and seven additional predictors: four existing biomarkers (GM1, albumin, immunoglobulin G, neutrophil-lymphocyte ratio) and three clinical factors from the modified Erasmus GBS outcome score model. Five complement activation products (C3a, C4a, C5a, soluble C5b-9, factor Bb) were measured in 35 patients and were compared with C3 and C4 levels. Longitudinal changes in C3 and C4 levels were compared with the disease course in 12 patients. ResultsHigher C3, but not C4, was associated with poorer outcomes: lower Medical Research Council sum scores (MRCSS), higher GBS disability score (GBSDS), longer hospitalization, and more frequent treatment-related fluctuations. Age, MRCSS at admission, and baseline serum C3 were significant independent indicators of 1- and 3-month GBSDS. We found that C3 was positively correlated with C3a (r = 0.32) and C5a (r = 0.37), which indicates an activated complement cascade with high C3. Longitudinal change of C3 coincided with clinical severity of the disease course. InterpretationThis study highlights the use of serum C3 as a novel mechanistic biomarker in GBS. Larger prospective studies are needed to validate our findings.

Guillain-Barré syndrome temporally associated with COVID-19 vaccines in Victoria, Australia

Guillain–Barré syndrome (GBS) is an adverse event of special interest (AESI) for surveillance systems monitoring adverse events following immunisation (AEFI) with COVID-19 vaccines. Emerging data support a temporal association between GBS and adenovirus-vector COVID-19 vaccines. We present a case series of GBS reports submitted between February and November 2021 to our enhanced spontaneous surveillance system (SAEFVIC) in Victoria, Australia, following vaccination with either the adenovirus-vector vaccine Vaxzevria ChadOx1-S (AstraZeneca) or an mRNA vaccine (Comirnaty BNT162b2 [Pfizer-BioNTech] or Spikevax mRNA-1273 [Moderna]). For each report, Brighton Collaboration case definitions were used to describe diagnostic certainty. Severity was graded using the GBS Disability Score. The observed incidence of GBS following immunisation against COVID-19 was compared to expected background ICD10-AM G61.0 coded hospitalisations. There were 41 total cases of GBS reported to SAEFVIC following Vaxzevria (n = 38), Comirnaty (n = 3), or Spikevax (n = 0) vaccines. The observed GBS incidence rate exceeded the expected background rate for Vaxzevria only, with 1.85 reports per 100,000 doses following dose 1, higher than the expected rate of 0.39 hospital admissions per 100,000 adults within 42 days of vaccination. Of 38 GBS reports following Vaxzevria, the median age at vaccination was 66 years and median onset of symptoms was 14 days following immunisation. There was one death. Four cases initially categorised as GBS were later reclassified as acute-onset chronic inflammatory demyelinating polyneuropathy. Fatigue was the predominant persisting symptom reported at follow up. Additional global studies are required to characterise risk factors, clinical variability, and to provide precision and generalizability regarding AEFI risks such as GBS associated with different vaccine platforms, which will help inform communication of the potential benefits and risks of COVID19 vaccination.

Guillain-Barré Syndrome in Northern China: A Retrospective Analysis of 294 Patients from 2015 to 2020.

Acute motor axonal neuropathy (AMAN) was first reported to be the main subtype of Guillain-Barré syndrome (GBS) in northern China in the 1990s. About 30 years has passed, and it is unknown whether the disease spectrum has changed over time in northern China. We aimed to study the epidemiological, clinical, and electrophysiological features of GBS in northern China in recent years. We retrospectively analyzed the medical records of GBS patients admitted to the Second Hospital of Hebei Medical University in northern China from 2015 to 2020. A total of 294 patients with GBS were enrolled, with median age 53 years and 60.5% of participants being male, and a high incidence in summer and autumn. AMAN was still the predominant subtype in northern China (40.1%). The AMAN patients had shorter time to nadir, longer hospitalization time, and a more severe HFGS score at discharge than acute inflammatory demyelinating polyneuropathies (AIDP) (p &lt; 0.05). With SPSS multivariable logistic regression analysis, we found the GBS disability score (at admission), dysphagia, and dysautonomia were independent risk factors for GBS patients requiring MV (p &lt; 0.05). In comparison with other regions, the proportion of AMAN in northern China (40.1%) was higher than in eastern (35%) and southern (19%) China. AMAN is still the predominant subtype in northern China after 30 years, but there have been changes over time in the GBS spectrum since the 1990s. There are regional differences in GBS in China.

Reliability and validity of prognostic indicators for Guillain-Barré syndrome in children.

To explore the clinical characteristics and prognostic predictors of Guillain-Barré syndrome (GBS) in Chinese paediatric patients. The clinical features of children with GBS hospitalized in the Children's Hospital of Chongqing Medical University were summarized retrospectively. The correlation between the Erasmus GBS Outcome Score (EGOS)/modified Erasmus GBS Outcome Score (mEGOS), GBS disability score (GDS)/modified Rankin Scale (MRS), Erasmus GBS Respiratory Insufficiency Score (EGRIS), and mechanical ventilation were evaluated. One hundred forty-two patients (86 males, 56 females; median 62.50 months [interquartile range 41.00-97.50]) with classic GBS were enrolled in the study. In the present GBS cohort, 134 (94.37%) patients could walk independently (GDS ≤2) and 121 (85.21%) could manage without assistance (MRS ≤2) at 6 months. Eighteen (12.68%) patients with GBS required mechanical ventilation. The performance of mEGOS on admission, mEGOS on day 7, and EGOS-predicted GDS outcome at 4 weeks, 3 months, and 6 months in the paediatric patients with GBS admitted within 2 weeks of disease onset and that of the MRS outcome were evaluated. The EGRIS in individuals who required mechanical ventilation was significantly higher than in patients without mechanical ventilation (median=6 vs median=3, p < 0.001). In Chinese paediatric patients with GBS who were admitted 2 weeks after disease onset, the mEGOS and EGOS are validated indicators for the prediction of clinical outcomes 6 months after onset. EGRIS is helpful in predicting the implementation of mechanical ventilation in the acute phase. The Erasmus Guillain-Barré syndrome (GBS) Outcome Score (EGOS) and modified EGOS are reliable prognostic predictors in paediatric patients with GBS. The Erasmus GBS Respiratory Insufficiency Score (EGRIS) is an effective predictor of mechanical ventilation in paediatric patients with GBS. An EGRIS of ≥5 indicates a high risk of mechanical ventilation in the acute phase.

Intravenous immunoglobulin therapy for HIV-associated Guillain–Barré syndrome

ObjectiveTo compare the efficacy of intravenous immunoglobulin (IVIG) added to antiretroviral therapy (ART) and ART alone in people living with human immunodeficiency virus-associated Guillain–Barré syndrome (HIV-associated GBS). MethodsThe study was a retrospective analysis of clinical records of HIV-associated GBS patients from department of neurology at West China Hospital between January 2015 and October 2020. Patients treated with ART alone were compared with those treated with IVIG + ART. The primary outcome was the GBS disability score at 4 weeks, which was assessed with multivariable ordinal regression. Additional outcomes include the GBS disability scale at 1 week, improvement of ≥ 1 point on the GBS disability score at 1 and 4 weeks, median change in the MRC sum score at 1 and 4 weeks, number of patients who were able to walk independently at 4 weeks. ResultsTwo hundred and fifty-two (252) individuals living with HIV were recruited in the study. According to the inclusion and exclusion criteria, 21 HIV-associated GBS patients were finally included, of whom 8 were treated with IVIG + ART and 13 were treated with ART alone. At the fourth weeks after treatment, the GBS disability scale grade was significantly lower in patients treated with IVIG + ART than those with ART alone (1 vs. 2, P = 0.02). The adjusted OR for a lower GBS disability scale was 10.6 (95 % CI 1.15 to 98.05; P = 0.03) for the IVIG + ART group. Moreover, 6 of 8 (75 %) patients treated with IVIG + ART were able to walk independently at four weeks after treatment. ConclusionsThe introduction of IVIG combined with ART may be efficacious in the treatment of HIV-infected GBS and may provide better clinical outcomes.