Analysis Of Prognostic Factors Of Guillain-Barré Syndrome In Children

Part One Association between clinical characteristics and prognosis in children with Guillain-BarréSyndromeObjective:To identify the progression rate,treatment response,and abundance A-waves in relation to the prognosis of pediatric Guillain-Barrésyndrome(GBS),and to investigate its potential predictive value for prog-nosis.Methods:1.This retrospective study enrolled children diagnosed with GBS in the Pediatric Neurology Department of Hebei Children’s Hospital between June2013 and May 2021.The Hughes functional grade(HG)was used to assess functional disability at admission,nadir,and 1 and 6 months after onset.Patients with HG≥3 were classified as having poor outcomes,while those with HG<3 were classified as having good outcomes.2.Patients were divided into three groups based on the rate of progression from symptom onset to nadir:rapid-onset GBS(within 48 h),gradual-onset GBS(between 3 and 14 days),and slow-onset GBS(between 15and 30 days).Patients were further divided into two groups based on the presence of abundant A-waves:GBS with or without A-waves and acute inflammatory demyelinating polyneuropathy(AIDP)with or without A-waves.Patients were finally divided into two groups according to the treat-ment response,which was defined as at least one HG improvement after IVIg treatment:rapid-response GBS(initial response within 7 days)and slow-response(initial response within 8-60 days).Results:1.A total of 65 children with GBS were enrolled in the study,including38 males and 27 females,aged 5 months to 13 years.The clinical charac-teristics were as follows:72.3%of the patients had preceding infections,13.8%had facial nerve palsy,32.3%had bulbar paralysis,56.9%had neuro-pathic pain,41.5%had autonomic dysfunction,and 15.4%had mechanical ventilation.All of the patients received IVIg,7.7%received IVIg combined with plasmapheresis,and 63.1%received hormone therapy.Further,70.8%had AIDP subtypes and 12.3%had acute motor axonal neuropathy(AMAN)subtypes,while 3 cases were changed by serial neuroelectrophysiological studies.Albumin cytological dissociation was observed in 92.3%of patients,of whom 15.2%tested positive for anti-GM1-Ig M/Ig G antibody.Further,90%of patients showed abnormal T/B lymphocytes,predominantly higher CD4~+T and CD19~+B.Of these patients,69.2%had a good prognosis and 30.8%had a poor prognosis 1 month after onset,while 92.3%had a good prognosis and7.7%had a poor prognosis 6 months after onset.2.Receiver operating characteristic curve analysis of the progression rate for short-term and long-term prognosis showed no statistical difference;the area under the curve(AUC)at 1 month after onset was 0.568,P=0.382,95%confidence interval(CI)0.413–0.724;AUC at 6 months after onset was 0.552,P=0.703,95%CI 0.277–0.826.3.The distal motor latency of GBS with A-waves was longer than that of GBS without A-waves,and A-waves existed only in the AIDP subtype.Univariate logistic regression analysis showed statistical significance(odds ratio[OR],5.844;95%CI,1.118–30.553;P=0.036).4.Kaplan-Meier survival analysis revealed that the predictive value of IVIg treatment for pediatric GBS patients with the ability to walk unaided(HG of 2)1 month after onset was significantly different(log-rank test for trend,P=0.012).Summary:Most children with GBS show a good prognosis.Faster GBS progression does not imply poorer functional outcomes.Abundant A-waves are markers of demyelination and associated with clinical severity and poor short-term prognosis.Abundant A-waves and important prognostic factors in children with GBS.The treatment response to IVIg was defined as seven days,during which time patients had a good short-term outcome.Part Two Correlation between electrophysiological characteristics and outcome in pediatric Guillain-BarrésyndromeObjective:To study the neuroelectrophysiological characteristics of children with Guillain-Barrésyndrome(GBS)and to evaluate the correlation between neuroelectrophysiological classification and outcomes.To explore the sympathetic skin response(SSR)characteristics in children with GBS,compare SSR and nerve conduction studies(NCS),and analyze the value of early diagnosis,efficacy,and prognostic evaluation in GBS complicated with autonomic dysfunction.Methods:This prospective study enrolled 25 pediatric patients with GBS from the neurology units of Hebei Children’s Hospital between 2020 and2022.A retrospective study enrolled 67 pediatric patients with GBS between2017 and 2022.All children underwent serial neuroelectrophysiological tests,and limb disabilities were evaluated according to the Hughes functional grade(HG).HG≥3 was considered as poor prognosis,while HG<3 was considered as good prognosis.Results:1.This retrospective study included 56 patients with acute inflammatory demyelinating polyneuropathy(AIDP)and 11 with acute motor axonal neuropathy(AMAN).Overall,8 patients(11.9%)had facial paralysis,21(31.3%)had bulbar paralysis,41(61.2%)had neuropathic pain,43(64.2%)had autonomic dysfunction,8(11.9%)had mechanical ventilation,7(7/52,13.5%)had anti-glycolipid antibody positivity,and 50(50/54,92.6%)had T/B lymphocyte abnormalities.2.Prolonged distal motor latency(DML)and decreased compound muscle action potential in AIDP patients began to appear in the first week,reached a peak in the 4th(upper limb)-8th(lower limb)week,and gradually recovered thereafter.Motor conduction velocity(MCV)reduced after the first week,and reached its lowest level during the second week.The sensory conduction velocity(SCV)and sensory nerve active potential(SNAP)of the upper limb began to decrease after the first week,reaching the lowest level in the second or third week.Eight patients(32%)had preserved sural sensory potential during the acute phase of the disease.3.AMAN patients showed decreased compound muscle action potentials;decreased CMAP began to appear in the first week,peaked in the third week,and gradually recovered thereafter.The DML of the tibial nerve showed transient prolongation at the 4th week after onset,and the MCV decreased transiently during the third week of follow-up.4.There was no significant difference between SCV and MCV in the early diagnosis of the AIDP subtype(P>0.05).The positivity rate for GM1antibodies in patients with AMAN was higher than that in patients with AIDP(x~2=14.86,P<0.001).The HGs of patients with AIDP were lower than those of patients with AMAN at 1 and 6 months after onset(Z=1.541/2.343,P=0.123/0.025).Overall,40 and 51 patients in the AIDP group and 7 and 11patients in the AMAN group had good short-and long-term prognoses,respectively.There were no significant differences in prognosis between the two groups(P>0.05).5.A total of 25 children with GBS were enrolled in this prospective study;66.7%of patients had autonomic dysfunction,72%had abnormal SSR,52%had AD combined with SSR abnormality,20%had only SSR abnormality,and 24%had mechanical ventilation.6.The SSR of the GBS group showed an absence of waveform or prolonged latency in the acute phase;eight patients(32%)had no waveforms,three(12%)had prolonged latency of both the upper and lower limbs,and seven(28%)had only prolonged latency of the lower limbs.The sensitivity,specificity,and accuracy of SSR combined with the nerve conduction study in diagnosing GBS were 84%,100%,and 92.7%,respectively,while in the recovery phase,there were 6 patients(24%)with abnormal SSR and 17patients(68%)with abnormal NCS,showing a statistically significant difference(P=0.003).7.HGs were not significantly different on admission,at nadir,or at 1 and6 months after symptom onset between patients with GBS with or without an abnormal SSR(P>0.05).Seven patients(28%)had a poor prognosis one month after symptom onset,all of whom had an abnormal SSR.All children with GBS had a good prognosis 6 months after onset.Summary:The trends of prolonged DML and decreased CMAP in patients was consistent,and the peak time of MCV was earlier than those of DML and CMAP.Patients with AMAN require longer recovery periods.There was no difference between SCV and MCV in the early diagnosis of the AIDP subtype.The majority of patients who tested positive for the GM1antibody had the AMAN subtype.The short-term prognosis of AIDP was better than that of AMAN,but there was no difference in the long-term prognosis.Overall,these results suggest that sympathetic skin response could be used for the early diagnosis of GBS and monitoring the treatment response,and could also be slightly helpful for evaluating disease severity and short-term prognosis.Part Three Association between CSF neurofilament light chain and pro-gnosis in pediatric Guillain-BarrésyndromeObjective:To explore the relationship between cerebrospinal fluid neurofilament light chain(CSF-Nf L)levels and the clinical characteristics of GBS in children,and to analyze the value of early diagnosis and prognosis.Methods:This prospective study enrolled 26 pediatric patients with GBS and 48 healthy controls(HCs)from the neurology units of Hebei Children’s Hospital between 2020 and 2022.CSF-Nf L levels were measured using enzyme-linked immunosorbent assay.Disability was evaluated using the Hughes functional grade(HG)at nadir,and at 1 and 6 months after onset.Receiver operating characteristic(ROC)curve analysis was performed to assess the prognostic value of CSF-Nf L for the possibility of walking aid 1month after symptom onset.Results:1.The CSF-Nf L levels were obviously increased in GBS patients(108.91 pg/m L)compared to HCs(79.96 pg/m L)(Z=5.384,P<0.001).At follow-up,CSF-Nf L levels after treatment(70.32 pg/m L)were significantly lower than those before treatment(Z=3.296,P=0.001).2.The sensitivity of the CSF protein test alone and the combination of CSF NFL and protein for early diagnosis of GBS were higher.1.CSF-Nf L levels upon admission significantly correlated with protein levels(r_s=0.392,P=0.048)and HG at nadir(r_s=0.461,P=0.018).CSF-Nf L levels in GBS patients with poor prognosis(130.5 pg/m L)were significantly higher than those with good prognosis(104.9 pg/m L)(t=2.40,P=0.025).3.ROC curve analysis of the predictive value of CSF-Nf L levels with respect to the inability to walk unaided within one month showed a statisti-cally significant difference(AUC:0.797,95%CI:0.626–0.968;P=0.022).Summary:CSF-Nf L levels in healthy children were 79.96 pg/m L,while those in pediatric patients with GBS increased upon admission and decreased to baseline after treatment.CSF-Nf L levels are associated with CSF protein levels,disease severity,and short-term prognosis,and may be used for the early diagnosis and prognostic evaluation of GBS.Conclusions:1.Abundant A-waves are markers of demyelination and associated with clinical severity and poor short-term prognosis.The treatment response time to IVIg was defined as seven days,during which time patients had a good short-term outcome.Faster progression of GBS does not imply a poorer functional outcome.2.There was no difference between SCV and MCV in the early diagnosis of the AIDP subtype.The short-term prognosis of AIDP patients was superior to that of AMAN patients,but with no difference in the long-term prognosis.Sympathetic skin response could be used for the early diagnosis of GBS and monitoring the treatment response,and may also be helpful in evaluating disease severity and short-term prognosis.3.CSF-Nf L levels in healthy children were 79.96 pg/m L on average,while levels in pediatric patients with GBS increased upon admission and decreased to baseline after treatment.CSF-Nf L levels are associated with CSF protein levels,disease severity,and short-term prognosis,and may be used for the early diagnosis and prognostic evaluation of GBS.