Clinical And Electrophysiological Studies In Guillain Barre Syndrome

Guillain Barré syndrome(GBS)is a type of immune mediated acute inflammatory peripheral neuropathy and the most common cause of acute flaccid paralysis worldwide.The incidence of GBS was 0.233 cases per100,000 children per year and 0.829 cases per 100,000 in the adult in China.Acute inflammatory demyelinating polyneuropathies(AIDP)and acute motor axonal neuropathy(AMAN)are the two main subtypes of GBS.Accurate classification of GBS subtypes depends on electrophysiological examinations,however there is no golden electrophysiological criterias at present.In the early 1990 s,it was reported that AMAN subtype was the main subtype of GBS in northern China,while AIDP is the main subtype of GBS in the southern,southwestern,and northeastern regions of China.The epidemiological features of Guillain-Barré syndrome(GBS)were different in different areas.Climate and environmental differences may be the potential reasons for regional differences in GBS.In the past 30 years,the level of economic,medical,and health conditions in Hebei region had significantly improved.It is unclear whether the clinical characteristics and electrophysiological subtypes of GBS have changed and in recent years in the region of Hebei.Therefore,it is necessary to conduct clinical and electrophysiological studies of GBS to update the clinical and electrophysiological research data of GBS in Hebei province.With the development of the pathological mechanism and electrophysiology of GBS,electrophysiological criteria have also been updated.At present,the most widely used GBS electrophysiological classification standards include Hadden criteria,Ho criteria,Rajabally criteria,with the recognition of reversible conduction block as an electrophysiological phenomenon,it was found that traditional electrophysiological criteria are insufficient,and there is currently no golden standard for GBS classification diagnosis.Whether the use of electrophysiological criteria in GBS studies in is also one of the reasons for regional variation is still unkown.Therefore,in this study we collected and analysed the detailed clinical and electrophysiological examination data of GBS patients enrolled in the past6 years in our hospital,in order to 1)update the research data of clinical features and electrophysiological subtype of GBS patients in Hebei.2)to compare the currently available electrodiagnostic criteria for the diagnosis of the AIDP and AMAN subtypes of GBS.Part One A Clinical and Electrophysiological Retrospective Study ofGuillain Barre SyndromeObjective: A clinical and electrophysiological retrospective study was conducted in GBS patients in our hospital to clarify the following questions: 1)whether AMAN is still the main subtype of GBS 2)The clinical characteristics of GBS patients in terms of epidemiology,prodromal factors,clinical symptoms,and mechanical ventilation related risk factors;3)Whether there are any changes in clinical features and electrophysiological subtype compared with the research conducted by Ho et al.in the 1990s;4)Whether there is regional difference when compared with GBS patients in different regions.Methods:A retrospective study was conducted on the demography characteristics,predisposing factors,clinical symptoms,GBS disability score,and related laboratory and neuroelectrophysiological subtypes of patients with GBS who were hospitalized in the Department of Neurology and Pediatric of XX Hospital from January 1,2015 to December 31,2020;The previous study of GBS in Northern China in the 1990 s was compared with the present study;The present study was compared with the GBS studies from the Eastern and Southern China.Results:1.This study ultimately included 294 GBS patients.The onset season is mainly in summer and autumn,with AIDP more common in autumn and AMAN more common in summer;The average age of onset was 53 years old(42.5-63 years old),and the peak of onset was mainly concentrated in 50-69 years old;Male was dominated(male to female ratio 1.53:1);49% of GBS patients had an infection event within 4 weeks before the onset of neurological symptoms,with respiratory tract infection(21.8%)and gastrointestinal tract infection(23.5%)being the main infections;The initial symptoms were mainly limb weakness(n=189,64.3%);All patients reached peak within 4weeks and the time to nadir was 6(4–10)days;The length of hospital stay was14(10–20)days.;Elderly patients(≥56 years old)had significantly higher HFGS scores at discharge than other patients(P<0.001).2.The electrophysiological subtype was mainly AMAN(40.1%),followed by AIDP(37.8%);Gastrointestinal infections were more common in patients with AMAN(29.5% vs 20.2%),while upper respiratory infection was more common in AIDP(25.3% vs 17.1%),however with no significant statistically difference(P =0.161);The proportion of patients with sensory abnormalities as initial symptoms in the AIDP group was higher than that in the AMAN group(36.4% vs 11.4%,P <0.001);The proportion of initial symptoms with limb weakness was lower in the AMAN group(58.6% vs86.7%,P <0.001).The difference in HFGS scores between the two groups at admission was not statistically different(P =0.120).At discharge,the proportion of patients with severe GBS in the AMAN group(HFGS score ≥3)was significantly higher than that in the AIDP group(69.5% vs 55.5%,χ2=4.253,P =0.039)。Compared with the AIDP group,patients in the AMAN group had shorter time to nadir,longer hospital stay,and more severe HFGS scores at discharge,with significant differences(P =0.012,P =0.016,P=0.007);3.With univariate analysis,we found that patients requiring MV had higher HFGS score,higher cerebrospinal fluid protein levels,and a higher proportion of dysphagia and autonomic dysfunction at admission(P <0.05).With multivariate logistic regression analysis,we found that HFGS score,dysphagia,and autonomic dysfunction at admission were independent risk factors for GBS patients requiring MV(P <0.05).4.Compared with the research results of GBS patients in the early 1990 s in our hospital,this study we found that the middle-aged and elderly people were the main population of GBS instead of children and the young,with the onset season changing from a high incidence in summer to summer and autumn.The proportion of gastrointestinal and respiratory infection as antecedent events decreased compared with the previous study.Although the proportion of AMAN subtypes in GBS electrophysiological subtypes decreased compared with that in the 1990s(46.6% vs 65%),AMAN subtype is still the main electrophysiological subtype.5.Compared with the clinical and epidemiological studies of GBS in southern and eastern China,there is a statistically significant difference in the proportion of electrophysiological subtypes and antecedent infections among different regions(P <0.01).AMAN is the main electrophysiological subtype of GBS patients in our study(40.1%)and eastern China(35%),while AIDP is the main electrophysiological subtype of GBS patients in southern China.Gastrointestinal tract infection is the main precursor infection trigger for GBS in our study(23.5%),while upper respiratory tract infection is the main antecedent trigger for GBS in the south(35%)and east(29%).Part Two Study on the classification of Guillain Barre Syndrome subtypesbased on different electrophysiological criteriaObjective: A study was conducted on GBS patients in our hospital using three commonly used electrophysiological classification criteria to clarify the following issues:(1)whether the classification results of GBS patients by using different electrophysiological standards are different;(2)Does electrophysiological examination at different time periods have an impact on the electrophysiological subtype of GBS patients;(3)Whether sensory nerve conduction data could make sense for the differentiate of subtypes;(4)The transformation of GBS patient classification through serial electrophysiological examinations;(5)The impact of conduction block electrophysiological phenomena on classification and its relationship with prognosis;(6)Improve understanding of different classification criteria in for a better classification of GBS patients.Methods: Patients diagnosed with GBS who were hospitalized in the Neurology and Pediatrics departments of the second Hospital of Hebei medical university from January 1,2015 to December 31,2020 were enrolled and the GBS patients were classified into different subtypes with different electrophysiological criteria.We studied the correlation between the Sural-sparing pattern and different electrophysiological subtypes and investigated the correlation between conduction block and clinical prognosis;GBS patients who had undergone two or more electrophysiological examinations were studied and the changes in electrophysiological subtypes of GBS patients were also studied.Results:1.A total of 262 GBS patients who had underwent electromyographic examination in this study were enrolled.The average time from clinical symptom onset to initial electromyographic evaluation was 11.84 days(range2-31 days).2.The 262 GBS patients were classified using three electrophysiological criteria: Ho’s criteria,Hadden’s criteria,and Rajabally’s criteria.AMAN was the main subtype of GBS patients in our hospital.There was a difference in the proportion of AMAN,AIDP,and Equivocal subtypes with different electrophysiological criteria.According to Rajabally’s criteria,the AIDP proportion was the lowest(19.1%)while the AMAN proportion was the highest(59.5%),and the Equivocal subtype proportion was the lowest(9.9%),According to the Ho’s criteria,the Equivocal subtype proportion is the highest(12.6%),according to the Hadden’s criteria,the AIDP proportion is the highest(37.8%)and the AMAN proportion is the lowest(40.1%).3.Using Hadden’s criteria as a reference for subtype classification in electrical diagnosis,the sensitivity and specificity of the Rajabally’s criteria for diagnosing AIDP are 50.5% and 100%,respectively;For the diagnosis of AMAN,the sensitivity of the Rajabally’s criteria is 100%,and the specificity is 67.5%.With Fleiss Kappa test we compared the consistency of three electrophysiological criteria,the kappa value was 0.751,P<0.001,indicating relatively strong consistency among the three electrophysiological criteria.4.In the first and second week after onset,there was a statistically significant difference in GBS subtypes with the three electrophysiological criteria(P =0.003,P =0.007).More patients were classified as AIDP with Hadden’s criteria,while more patients were classified as AMAN with Rajabally’s criteria;At weeks 3 and 4,the GBS subtyps with the three electrophysiological criteria was more closer(P =0.718,P =0.32).5.Compared with AMAN,we found that the AIDP subtype had the highest proportion of Sural-sparing pattern,with a statistically significant difference(P <0.001)6.Compared with patients without conduction block,patients of AMAN subtype with conduction block had lower HFGS scores at discharge(P=0.012).7.The initial electrophysiological results were classified as AIDP 14cases(45.2%),AMAN 10 cases(32.3%),Equivocal 7 cases.In the second electrophysiological examination,AMAN in 11 cases(54.8%),AIDP in 10cases(32.5%),Equivocal 1 case(3.2%),and rapid recovery to normal in 3cases(9.7%).Conclusions:1.This study supplemented and updated the clinical and electrophysiological classification data of GBS patients in Hebei region.2.There are regional differences in GBS patients compared with other regions in China.3.Electrophysiological classification: AMAN is the main subtype of GBS patients in our hospital,independent of the electrophysiological classification criteria.4.The results of three electrophysiological classification standards were consistent;The use of the Hadden standard may underestimate the proportion of AMAN subtypes in the early stages of the disease(≤ 2 weeks),and the electrophysiological subtypes tend to be consistent after 3 weeks;The phenomenon of sural nerve spare is helpful for the diagnosis of AIDP subtype;Serial electrophysiological examinations can provide more accurate classification for GBS patients.5.Prognosis: AMAN subtypes,age≥56 years old,high admission HFGS score,difficulty in swallowing,and presence of autonomic dysfunction are important risk factors for poor prognosis.However,using the Rajabally classification criteria,AMAN patients with conduction block have better short-term prognosis;...