Hughes Functional Grading Scale Research Articles

Distribution characteristics of antinuclear antibodies in Guillain-Barré syndrome and its relationship with disease severity

BackgroundGuillain-Barré syndrome (GBS), an acquired immune-mediated autoimmune disorder affecting the peripheral nervous system (PNS), is associated with autoimmunity. The presence of autoantibodies in the blood is an important feature of autoimmune diseases. Herein, we explored the distribution characteristics of the antinuclear antibodies (ANAs) in GBS and the correlation between ANAs and disease severity. Materials and methodsWe retrospectively analyzed the clinical data of 170 GBS patients. According to ANAs, GBS patients were divided into ANAs positive and negative groups. The clinical characteristics of these two groups were compared. The distribution difference was also compared between male and female GBS patients. In addition, all enrolled patients were divided into more severe group and milder group according to whether the Hughes score at nadir ≥ 3 or not. Gender, age, and ANAs were compared between the two groups. ResultsIn this study, the positive rate of ANAs was 27.1 % in 170 GBS patients, among which anti-SSA-52/Ro52 antibody and antimitochondrial antibody M2 made up the largest proportion. In the ANAs positive group, GBS patients had longer days of hospitalization, more respiratory function involvement, and higher level of CSF IgG than the ANAs negative group. Compared to the ANAs negative group, Medical Research Council (MRC) scores on admission and at nadir were lower, and Hughes functional Grading Scale (HFGS) scores on admission and at nadir were higher in GBS patients with ANAs positive group. Erasmus GBS Respiratory Insufficiency Score (EGRIS) in ANAs positive GBS patients group was significantly higher than ANAs negative group. Gender had no effects on the distribution of ANAs in GBS patients. Moreover, we found that the anti-SSA-60 antibodies and age were positively correlated with GBS severity. In addition, in the anti-SSA-60 antibody positive group, GBS patients had longer days of hospitalization, more respiratory function involvement, higher HFGS scores on admission/at nadir, and lower MRC scores at nadir compared with the anti-SSA-60 antibody negative group. ConclusionAnti-SSA-52/Ro52 antibody and antimitochondrial antibody M2 were the most common ANAs in GBS patients. Anti-SSA-60 antibodies and age positively correlated with GBS severity. Positive anti-SSA-60 antibodies and age were independent predictors of GBS patient severity.

Clinical features and treatment of acute motor axonal neuropathy

This paper aims to investigate the clinical features and treatment of patients with acute motor axonal neuropathy (AMAN). The clinical data of 9 patients with AMAN treated in our hospital were retrospectively analyzed from prodromal infections, clinical features, neuroelectrophysiological examination, treatment and the scores of Hughes functional grading scale. Diarrhea was the most common prodromal infection, and weakness of four limbs and muscles atrophy acutely emerged. The patient's condition progressed rapidly. In these cases, therapy of immunoglobulin combined with methylprednisolone was effective. Therefore, early diagnosis and timely treating with immunoglobulin therapy are most important methods to improve the prognosis.

Factors Associated with Respiratory Insufficiency in Children with Guillain-Barré Syndrome.

The risk factors for respiratory insufficiency in children with Guillain-Barré syndrome (GBS) are poorly known. This study aimed to investigate the factors associated with respiratory insufficiency in children with GBS. This retrospective study included children diagnosed with GBS by pediatric neurologists and admitted at the Wuhan Children's Hospital and other hospitals from January 2013 to October 2022. The patients were divided into the respiratory insufficiency and nonrespiratory insufficiency groups according to whether they received assist breathing during treatment. The median (interquartile range) age of onset of 103 patients were 5 (3.1-8.5) years, 69 (67%) were male, and 64 (62.1%) had a history of precursor infection. Compared with the nonrespiratory insufficiency group, the respiratory insufficiency group showed more facial and/or bulbar weakness (p = 0.002), a higher Hughes Functional Grading Scale (HFGS) at admission (p < 0.001), and a shorter onset-to-admission interval (p = 0.017). Compared with the acute motor axonal neuropathy (AMAN) subtype, the acute inflammatory demyelinating polyneuropathy (AIDP) subtype showed longer days from onset to lumbar (p = 0.000), lower HFGS at admission (p = 0.04), longer onset-to-admission interval (p = 0.001), and more cranial nerve involvement (p = 0.04). The incidence of respiratory insufficiency between AIDP and AMAN showed no statistical difference (p > 0.05). In conclusion, facial and/or bulbar weakness, HFGS at admission, and onset-to-admission interval were associated with respiratory insufficiency and might be useful prognostic markers in children with GBS.

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.

The clinical significance of systemic immune-inflammation index and platelet/neutrophil to lymphocyte ratio in Guillain-Barré syndrome

ObjectiveSystemic immune-inflammation index (SII) and platelet/neutrophil to lymphocyte ratio (P/NLR) are two novel hematological inflammatory indices, this study invested the role of SII and P/NLR in Guillain-Barré syndrome (GBS). MethodsA total of 115 GBS patients and 120 healthy controls were enrolled in this retrospective study, SII and P/NLR were calculated from the value of complete blood counts. The Hughes Functional Grading Scale (HFGS) score on admission and at discharge was used to evaluate the severity and short-term outcome of GBS. The level of SII and P/NLR was compared between GBS patients and healthy controls, and the correlation between SII, P/NLR, and GBS’s severity as well as poor short-term outcome was analyzed. ResultsIncreased SII (p < 0.001) and decreased P/NLR (p < 0.001) were observed in patients with GBS and the level of SII (p = 0.689) and P/NLR (p = 0.879) was not different between GBS subtypes. Patients with severe GBS and poor short-term outcomes had higher levels of SII and lower levels of P/NLR (p < 0.05), SII was positively correlated and P/NLR was negatively correlated with the HFGS score on admission and at discharge (p < 0.05). To predict severe GBS, the optimal cutoff value of SII was 620.87 (AUC0.633, sensitivity 60.8 % and specificity 60.9 %, p = 0.014), the optimal cutoff value of P/NLR was 53.11 (AUC0.635, sensitivity 45.1 %, specificity 82.8 %, p = 0.013). The SII level of 620.87 was found to be optimal predictive cutoff value for the poor short-term outcome of GBS (AUC 0.728, sensitivity of 82.6 %, specificity of 59.8 %, p = 0.001), the P/NLR level of 62.80 was found to be optimal predictive cutoff value for the poor short-term outcome of GBS (AUC 0.669, sensitivity 60.9 %, specificity 71.7 %, p = 0.012). Both SII>620.87 (p = 0.005) and P/NLR<53.11 (p = 0.002) were independent risk factors for severe GBS, and SII>620.87 (p = 0.035) was independently associated with the poor short-term outcome of GBS. ConclusionSII and P/NLR may be useful biomarkers to reflect GBS patients’ severity and short-term outcome.

Three-year follow-up outcomes of adult patients with Guillain-Barré Syndrome after rehabilitation.

Guillain-Barré syndrome (GBS), the most common cause of acute paralytic neuropathy, covers a number of recognizably different variants. We aimed to evaluate the clinical characteristics of the patients with GBS and the outcome results of the patients after rehabilitation. We enrolled 24 adult patients with GBS and evaluated their demographic characteristics, signs, complications, functional levels, and residual symptoms at admission, discharge, and during the 1st and 3rd-year follow-up visits. Functional Independence Scale (FIM), Functional Ambulation Scale (FAS), Hughes functional grading scale, Six-Minute Walking Test (6MWT), and Fatigue Severity Scale (FSS) were used for patient evaluation. In this study, patients with a mean age of 47.29 ± 16.2 years (40% female) were hospitalized for an average of 28.91 ± 25.6 days. The predominant symptoms experienced by these patients were fatigue (100%), neuropathic pain (70.8%), joint pain (54.2%), and autonomic dysfunction (50%). Significant changes were observed in FIM, Hughes functional grading scale, FAS, 6MWT, and MRC score at admission, discharge, and 1st/3rd-year follow-ups (p=0.000, p=0.000, p=0.000, p=0.001, p=0.000, respectively). Fatigue and Hughes score increased significantly with age (p=0.019, r=0.475; p=0.041, r=0.419, respectively). Negative correlations were found between age and FAS, 6MWT, and MRC score at 1st-year follow-up (p=0.025, r=-0.456; p=0.027, r=-0.450; p=0.008, r=-0.528). FSS was above 4 before admission and in 53.1% at 3rd-year follow-up, correlating negatively with 6MWT and MRC sum score. GBS clinical types showed no significant differences. Rehabilitation improves functional improvement in GBS patients, with long-term benefits observed. However, residual symptoms such as fatigue and neuropathic pain may persist despite functional improvement. These findings highlight the importance of incorporating rehabilitation into the management of GBS and addressing residual symptoms to improve patient outcomes.

Pattern of Autonomic Involvement in Adult Patients with Guillain Barre Syndrome in a Tertiary Hospital

Background: Guillain Barre Syndrome (GBS) is an acute post infectious immune mediated peripheral neuropathy with a marked variation in pathology, clinical presentation and prognosis. Autonomic dysfunction is one of the important manifestations of GBS which may lead to significant morbidity and mortality. Objective: The aim of the study is to assess the autonomic involvement, to determine its frequency and pattern of involvement in adult patients with GBS Methods: An observational, descriptive, cross sectional study was carried out in the Department of Neurology, BSMMU, Dhaka from March, 2015 to September, 2017. Total 43 patients of GBS and 35 apparently healthy controls were recruited as the study population. On the basis of nerve conduction study patients were classified into different groups: acute inflammatory demyelinating polyneuropathy (AIDP), acute motor axonal neuropathy (AMAN) and acute motor sensory axonal neuropathy (AMSAN) and other variants. Disability status at the time of autonomic testing was measured by Hughes functional grading scale. The following tests of autonomic nervous system were performed in both patients and controls 1) resting heart rate and heart rate on changing posture (30: 15 ratio) 2) supine blood pressure and blood pressure on changing posture 3) heart rate response to valsalva maneuver 4) heart rate response to deep breathing and E: I ratio 5) sphincter disturbance by symptoms questionnaire. Results: The mean age of patients was 35±12 years (range18 to 65 years) and 58.1% were male. Around 88.4 % of patients showed some sort of autonomic dysfunction. Variation of heart rate by different maneuver like posture change, deep breathing and valsalva maneuver was found commonly. Among them 30:15 ratio was abnormal in majority of the patients (82.4%) followed by abnormal max-min HR/min (58.1%) and abnormal valsalva ratio (37.2%). Other abnormalities were postural hypotension (38.2%), sinus tachycardia (25.6%), hypertension (16.3%), hypotension (2.7%), sinus arrhythmia (4.7%), constipation (30%), urinary retention (7%) and urinary incontinence (4.7%). Conclusion: In this study different patterns of autonomic dysfunction was found in 88.4% of patients with GBS involving both sympathetic and parasympathetic components. The present study found no significant association between autonomic dysfunction and motor disability scores. Thus autonomic function assessment is essential in every patient with GBS in addition to motor &amp; sensory function. Bangladesh Medical Res Counc Bull 2022; 48(3): 174-179

Characteristics of Guillain-Barré syndrome in super-elderly individuals.

Japan has the world's largest super-aging population, and the number of elderly patients with various diseases is increasing. Herein, we reported the characteristics of super-elderly patients, aged over 80years, with Guillain-Barré syndrome (GBS), a typical neuroimmune disease. During the period 2019-2021, 74 patients over the age of 80years diagnosed with GBS at Kindai university were analyzed as the super-elderly group patients. The control group comprised 74 consecutive patients aged < 79years, under the same conditions. GBS was diagnosed using Brighton diagnostic criteria. Electrophysiology was assessed using the Ho criteria. The mean age was 83.5years in the super-elderly group and 51.7years in the control group. Prior infection was recognized in 50% of cases in the super-elderly group and 77% of cases in the control group with fewer cases in the super-elderly group. The mean number of days until peak symptom presentation was longer in the super-elderly group. The percentage who required a ventilator was significantly higher among the super-elderly group than among the control group. Hughes functional grading scale was more severe in the super-elderly group. Electrophysiological examination revealed the demyelinating form was particularly common in the super-elderly group. Intravenous immunoglobulin was the most common treatment in both the groups, with no difference in efficacy. Super-elderly onset GBS tends to be severe, therefore it is important to diagnose and treat appropriately, even in the absence of prior episodes of infection.

Sural Sparing Pattern and Sensory Ratio as Electrodiagnostic and Prognostic Markers in Pediatric Guillain-Barré Syndrome.

We aimed to evaluate the presence of sural sparing pattern (SSP) and sensory ratio in pediatric Guillain-Barré syndrome (GBS), their distribution to subtypes, and their relationship with demographic and clinical features with a focus on the disability and muscle strength. This single-center retrospective study was conducted on pediatric GBS patients of both sexes with 2 years follow-up and two nerve conduction studies in which SSP and sensory ratio were calculated. Three subgroups of SSP were formed by separate calculation of median (SSP-m) and ulnar (SSP-u) and both median and ulnar sensory nerve action potentials (SNAPs; SSP-total). Muscle strength and disability were evaluated with the Medical Research Council (MRC) sum score and Hughes functional grading scale (HFGS), respectively. SSP total was identified in 70.6% (n: 24) of the patients, while sensory ratio >1 was observed in 20 (66.7%) patients. Patients with SSP-m, SSP-u, SSP-total, or sensory ratio >1 had higher HFGS scores, while patients with SSP-m, SSP-u, or SSP-total had lower MRC sum scores. SSP parameters were significantly associated with muscle strength and disability scores in acute motor axonal neuropathy patients. Both SSP and sensory ratio can be used for diagnostic and prognostic purposes. Disability and muscle strength are associated with SSP and sensory ratio in pediatric GBS.

Clinical Spectrum and Outcome of Guillain-Barré Syndrome with Plasmapheresis

Context: Guillain-Barré syndrome (GBS) is an acute inflammatory neuropathy and has several electrophysiological subtypes and clinical variants. Treatment is mainly supportive and immunotherapy is given to shorten the disease course. Aims: The aim of this study was to define the outcome of GBS with plasmapheresis and to determine its clinical spectrum. Materials and Methods: The prospective study was done at Mayo Hospital, Lahore, for 1 year from November 2020 to November 2021. The diagnosis of GBS was made on Brighton criteria for GBS. The outcome of therapy was assessed at 3 and 6 months, using the Medical Research Council Scale and Hughes Functional Grading Scale (HFGS). Results: A total of 50 patients were enrolled in the study. Twenty-seven (54%) were males and 23 (46%) were females. Seventeen (34%) had acute motor axonal neuropathy, 16 (32%) had acute motor and sensory axonal neuropathy, 15 (30%) had acute inflammatory demyelinating polyradiculoneuropathy, and 2 (4%) had Miller-Fisher syndrome. Plasmapheresis was done in all patients. HFGS score was assessed at discharge, at 3 months, and after 6 months of illness onset. The improvement in mean HFGS score was 2.79 ± 0.41 at 3 months and 1.94 ± 0.25 at 6 months of symptoms onset from the mean score of 3.46 ± 0.93 at the time of discharge from the hospital. Conclusion: Excellent outcome was observed after plasmapheresis. Majority of participants were able to walk without support at the 6-month follow-up. Plasmapheresis should be initiated early in the management of GBS where intravenous immunoglobulins are costly.

Elevated monocyte to high-density lipoprotein cholesterol ratio correlates with clinical severity in acute inflammatory demyelinating polyradiculoneuropathy patients.

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.

The prediction effects of thyroid function in the severity of Guillain-Barré syndrome

Guillain-Barré syndrome (GBS), an acquired immune-mediated inflammatory disorder affecting the peripheral nervous system (PNS), is usually complicated with autoimmune diseases including thyroid diseases. Herein, we explored roles of thyroid function and thyroid autoantibodies in the disease severity and its short-term prognosis of GBS. In addition, we further investigated the predictive value of thyroid function for GBS respiratory insufficiency. We retrospectively analyzed the clinical data of 219 GBS patients. According to the thyroid function, the enrolled subjects were divided into 2 groups, that is, patients with abnormal thyroid function (case group) and those with normal thyroid function (control group). The clinical characteristics, disease severity, and short-term prognosis of the patients in 2 groups were compared. In addition, we also divided the 219 GBS patients into mechanical ventilation (MV) group and non-MV group according to whether MV was performed within 1week after admission. The clinical characteristics, disease severity, short-term prognosis, Erasmus GBS respiratory insufficiency score (EGRIS), and the thyroid function were compared in the two groups. We found that GBS patients with abnormal thyroid function had longer duration of hospitalization, higher frequency of cranial nerve damage, and higher incidence of weakened tendon reflexes. Medical Research Council (MRC) scores on admission, at nadir, and at discharge were lower, and Hughes Functional Grading Scale (HFGS) scores on admission and at discharge were higher in GBS patients with abnormal thyroid function group. More patients in the abnormal thyroid function group had myelin, axonal, and myelin-axonal injuries. In the MV group, the time from onset to admission, MRC scores on admission, and the levels of free triiodothyronine (FT3) were lower; the levels of thyroglobulin antibody (TgAb) and EGRIS were significantly higher than those in the non-MV group. The combination of EGRIS and FT3 serum levels to predict GBS patients with MV, the area under the curve (AUC) was 0.905 (95% CI: 0.861 to 0.948, P < 0.05), sensitivity was 88.9%, and specificity was 84.7%. Our results suggest that the serum FT3 levels are negatively correlated with disease severity; the serum FT3 might be a biomarker for the incidence and severity of GBS. Both EGRIS and serum FT3 have a predictive value for the occurrence of acute respiratory insufficiency in GBS patients, and the combination of these two indicators can more accurately predict the risk of acute respiratory insufficiency in GBS patients.

Serum thyroid-stimulating hormone is an independent risk factor of recurrent Guillain-Barré syndrome.

Guillain-Barré syndrome (GBS) is generally considered to be monophasic, but some patients have recurrences. The purpose of this study was to clarify the possible link between thyroid parameters and recurrent GBS (RGBS) patients in China. In this retrospective study we enrolled patients who were admitted to the Department of Neurology of The First Affiliated Hospital of Zhengzhou University from 2014 to 2020 and fulfilled the diagnostic criteria of GBS or Miller Fisher syndrome. We evaluated clinical characteristics; cerebrospinal fluid parameters; and serum levels of thyroid-stimulating hormone (TSH), free thyroxine, and free triiodothyronine in 320 individuals, including 302 with monophasic GBS and 18 with recurrent GBS. Serum levels of TSH in monophasic GBS patients were significantly lower than those in RGBS patients (P< .001), whereas FT3 levels were higher in the monophasic GBS group (P= .022). Age at onset, incidence of antecedent illness, time from onset to nadir, proportion with acute inflammatory demyelinating polyradiculoneuropathy, and Hughes Functional Grading Scale score at nadir were statistically significant between monophasic GBS patients and RGBS patients (P< .05). The multivariate regression analysis revealed that antecedent illness, AIDP, and high TSH were independent risk factors for RGBS. Our receiver-operating characteristic curve analysis showed that the risk of recurrence in GBS patients increases when the TSH concentration is higher than 3.87 μIU/mL. Our results demonstrate an association between TSH and RGBS. Oxidative stress is one of the possible interpretations for this association.

Association between serum low‐density neutrophils and acute‐onset and recurrent Guillain–Barré syndrome

Background and AimGuillain–Barré syndrome (GBS) is one of the most common causes of acute flaccid paralysis. A timely assessment of this disease condition and its treatments are of vital importance to patients diagnosed with GBS. The purpose of this study is to investigate the variation trend of neutrophils along with disease courses and assess the prognostic value of serum low‐density neutrophils (LDNs) in the acute‐onset and recurrence of GBS.MethodsA total of 176 GBS patients were recruited. Patients were evaluated with Medical Research Council (MRC) sum score and the Hughes Functional Grading Scale score upon admission. Peripheral blood samples were collected for routine testing. Flow cytometry analysis was performed to identify LDNs. All patients were followed up to collect disease condition data.ResultsThe total neutrophil ratios and counts were significantly higher in patients with acute‐onset GBS compared to healthy controls (HCs). These counts/ratios decreased during remission and re‐elevated in recurrent GBS patients. However, no correlation was observed between the total neutrophil counts/ratios and the MRC sum score. The LDNs collected from different GBS courses were identified using flow cytometry. The counts and ratios were significantly higher in acute‐onset GBS and recurrent GBS compared to HCs and patients in remission. The LDN counts/ratios displayed a negative correlation with the MRC sum scores in acute‐onset GBS and recurrent GBS.ConclusionOur findings suggest that LDN counts/ratios are positively correlated with the acute‐onset and recurrence of GBS and its severity. Therefore, LDNs might serve as an accessible prognostic indicator for disease progression monitoring.

Association Between the Rate of Treatment Response and Short-Term Outcomes in Childhood Guillain-Barré Syndrome.

Introduction: Few studies have examined the association between the rate of treatment response and the outcome of pediatric Guillain-Barré syndrome (GBS). Therefore, our study aimed to identify treatment response in relation to the short-term outcomes of GBS. Further, we investigated its potential predictive value for prognosis.Methods: Our retrospective study included children diagnosed with GBS in the Pediatric Neurology Department of the Children's Hospital of Hebei Province from 2016 to 2020. According to the rate of response from the standard intravenous immunoglobulin (IVIg) treatment, patients were divided into two groups: rapid-response GBS (initial response within 7 days) and slow-response (initial response within 8–30 days). The GBS disability score (Hughes Functional Grading Scale) was used to assess the children's functional disability at nadir, 1 month, and 6 months after onset.Results: Among the 36 children included in the study, 18 (50%) and 18 (50%) were rapid and slow responders, respectively. Time from IVIg treatment to the initial response was significantly shorter in the rapid-response group (5 [3–6.25] days vs. 10.5[8.75–15] days in slow-response GBS, p < 0.001). Hughes score at 1 month was worse than the rapid responders (Fisher's exact test, p = 0.006). Survival analysis (Kaplan–Meier) with respect to regaining the ability to walk independently (Hughes Functional Grading Scale of 2) within 1 month after onset was significantly different among the two groups (log-rank test for trend, p = 0.024). The abnormal levels of cerebral spinal fluid proteins and autonomic dysfunction were more frequent in the slow-response group than those in the rapid group (p < 0.05).Conclusion: The rate of response to IVIg treatment was correlated with short-term outcomes in children with GBS and had predictive value for prognosis. The role of patient's initial responses to treatment could be significantly valuable in developing more effective and efficient treatment options.

Relationship Between Acute Phase Reactants and Disability in Guillian-Barré Syndrome During the COVID-19 Pandemic

BackroundGuillain-Barré syndrome (GBS) is an immune-mediated disease that affects the peripheral nervous system and may occur after some bacterial-viral infections.AimThe aim of this study is to determine and compare the epidemiological, clinical and laboratory characteristics of the patients followed up in our clinic with the diagnosis of GBS in the 15 month periods before and after March 2020. At the same time, we aimed to examine the importance of these markers as prognostic indicators by investigating the relationship of D-dimer, CRP, albumin and transferrin levels with Hughes functional grading scale score (HFGSS).Material and MethodsThe medical files of the patients who were followed up with the diagnosis of GBS between December 2018 and May 2021 were retrospectively analyzed. The patients were divided into groups as pandemic, pre-pandemic, post-COVID-19 and non-COVID-19. Epidemiological and clinical characteristics of GBS patients and plasma D-dimer, serum albumin, CRP and transferrin levels were recorded.ResultsNo significant difference was found between the pandemic and pre-pandemic periods in terms of age, gender, GBS subtype, seasonal distribution and treatment characteristics of GBS patients. PostCOVID-19 GBS patients had significantly higher HFGSS both at admission and at discharge (p <0.05). In post-COVID-19 GBS patients good-excellent negative correlation between transferrin and albumin levels and HFGSS at hospital admission and discharge, positive correlations with CRP levels were observed.ConclusionPost-COVID-19 GBS patients had worse HFGSS at both admission and discharge. CRP was positively correlated with HFGSS whereas transferrin and albumin showed negative correlation with HFGSS.

Elevated blood and cerebrospinal fluid glucose levels affect the severity and short-term prognosis of Guillain-Barré syndrome

ABSTRACT Objective This study aimed to explore the correlation of elevated glucose levels in the blood and cerebrospinal fluid with the progression and short-term prognosis of Guillain-Barré syndrome (GBS). Methods The medical records of 982 patients who were diagnosed with GBS in 31 representative tertiary hospitals, located in 14 provinces in southern China, were collected and retrospectively reviewed. Patients were grouped according to the levels of fasting plasma glucose (FPG) and cerebrospinal fluid (CSF) glucose, as well as the concentration of blood hemoglobinAlc (HbA1c). The Hughes grade scale was used to quantify functional outcomes. Results Compared to patients with normal FPG and CSF glucose levels, those in the high FPG and high CSF glucose groups were characterized by a higher proportion of severe patients (HFGS ≥ 3) at admission (58.8 vs. 73.1, P = 0.000; 57.6 vs. 71.2, P = 0.000), at nadir (67.4 vs. 83.0, P = 0.000; 66.2 vs. 80.4, P = 0.000), and at discharge (29.8 vs. 46.3, P = 0.000; 26.4 vs. 45.0, P = 0.000). Patients in the high HbA1c group also had more severe disability at admission (74.6 vs. 56.1, P = 0.005) and at nadir (80.3 vs. 64.3, P = 0.012) compared to the normal HbA1c group. Moreover, elevated levels of FPG and CSF glucose were significantly correlated with more severe disability at admission, at nadir, and at discharge. Conclusions The present study showed that elevated glucose levels in the blood and cerebrospinal fluid were associated with the severity and short-term prognosis of GBS. Trial registration chicTR-RRc-17,014,152. Abbreviations GBS, Guillain-Barré syndrome; FPG, fasting plasma glucose; CSF, cerebrospinal fluid; HFGS, Hughes Functional Grading Scale; HbA1c, hemoglobin A1c. DM, diabetes mellitus; NCS, nerve conduction study; AIDP, acute inflammatory demyelinating polyneuropathy; AMAN, acute motor axonal neuropathy; AMSAN, acute motor sensory axonal neuropathy; MV, mechanical ventilation.

Increased systemic immune-inflammation index can predict respiratory failure in patients with Guillain-Barré syndrome.

Guillain-Barré syndrome (GBS) is a common peripheral neuropathy with relatively heterogenous clinical manifestations. In severe cases, it can result in disabling neuromuscular symptoms and respiratory failure. In this study, we assessed whether the systemic immune-inflammation index (SII) is associated with respiratory failure in GBS patients. We retrospectively analyzed clinicopathological data from GBS patients diagnosed in the First Affiliated Hospital of Kunming Medical University. SII was calculated based on counts of neutrophils (N), platelets (P), and lymphocytes (L). Receiver operating characteristic (ROC) curve analysis was used to determine the optimal cutoff values for SII, N, P, and L. Univariate and multivariate logistic regression were performed to determine the ability of the SII to predict respiratory failure. Among the 369 GBS patients in this study, 61 (16.5%) suffered respiratory failure. Multivariate logistic regression showed that N ≥ 6.03 × 109/l, Hughes Functional Grading Scale (HFGS) score ≥ 3 at admission, and SII ≥ 863.04 × 109/l can independently predict respiratory failure in GBS patients. SII showed higher predictive ability than N, based on area under the ROC curve (0.75 vs. 0.72). SII may be a novel independent prognostic indicator of respiratory failure in GBS patients, helping physicians identify those at risk in a timely manner.

Risk factors associated with the need for mechanical ventilation in children with Guillain-Barré syndrome.

To study the risk factors associated with the need for mechanical ventilation in children with Guillain-Barré syndrome (GBS). The medical data of 107 children with GBS were retrospectively reviewed. According to whether mechanical ventilation was required, the children were divided into a mechanical ventilation group with 16 children and a non-mechanical ventilation group with 91 children. The risk factors associated with the need for mechanical ventilation in children with GBS were identified by a multivariate logistic regression analysis. Among the 107 children, 16 (15.0%) required mechanical ventilation. Compared with the non-mechanical ventilation group, the mechanical ventilation group had a significantly higher proportion of children with facial and/or bulbar muscle weakness, a significantly shorter duration from the onset to the peak of the disease, and a significantly higher Hughes Functional Grading Scale score at the first visit (P<0.05). Facial and/or bulbar muscle weakness and short duration from the onset to the peak of the disease were risk factors associated with the need for mechanical ventilation in children with GBS (OR=5.053 and 1.239 respectively, P<0.05). Facial and/or bulbar muscle weakness and short duration from the onset to the peak of the disease may increase the risk of mechanical ventilation in children with GBS. Citation.

Guillain-Barr\xe9 syndrome triggered by surgery in a Chinese population: a multicenter retrospective study

BackgroundSurgery is a potential trigger of Guillain-Barré syndrome (GBS), a disorder which leads to an autoimmune-mediated attack of peripheral nerves. The present study was designed to explore clinical features of post-surgical GBS compared with those of general GBS in order to provide better clinical advice to patients undergoing surgery.MethodsThe medical records of GBS patients who were seen at 31 tertiary hospitals in southern China between January 1, 2013 and September 30, 2016 were retrospectively analyzed. Post-surgical GBS was defined as symptoms of GBS within 6 weeks after surgery. Clinical features of post-surgical GBS are described and are compared with general GBS.ResultsAmong the 1001 GBS patient cases examined in this study, 45 (4.5%) patient cases exhibited symptoms of GBS within 6 weeks of undergoing surgery. Within this group, 36 (80.0%) patients developed initial symptoms of limb weakness. The average interval between surgery and symptom onset was 13.31 days. The most common type of surgery which triggered GBS was orthopedic surgery, followed by neurological surgery. Compared to general GBS, post-surgical GBS was characterized by a higher proportion of severe patients (Hughes functional grading scale (HFGS) score ≥ 3) upon admission and at nadir, higher HFGS scores at discharge, and longer hospital stays. Post-surgical GBS patients also had a significantly higher frequency of the acute motor axonal neuropathy subtype (37.9 vs. 14.2, respectively; P = 0.001).ConclusionSurgery is probably a potential trigger factor for GBS, especially orthopedic surgery. Infections secondary to surgery may play a role. The possibility of preceding (post-operative) infections was not excluded in this study. Clinical presentation of post-surgical GBS is characterized by a more severe course and poorer prognosis, and should be closely monitored.Trial registrationchicTR-RRc-17,014,152.