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Objective@#To explore the A wave value in neuroelectrophysiological subtype of Guillain-Barré syndrome(GBS)and the clinical severity and short-term prognosis of acute inflammatory demyelinating polyradiculoneuropathy(AIDP).@*Methods@#From March 2014 to March 2017, a total of 56 children with GBS at Department of Neurology of Wuhan Children′s Hospital Affiliated to Tongji Medical College, Huazhong University of Science & Technology were enrolled.The patients were divided into AIDP subtype(40 cases) and axonal GBS subtype(16 cases) according to the results of electrophysiological examination.According to whether there was existence of A wave or not, the GBS children were divided into 2 groups.The first group was the A wave in GBS group(18 cases), and the second group was non-A wave in GBS group(38 cases). In order to explore classification value for GBS with A wave, clinical data including age, gender, history of prodromal infection, cranial nerve dysfunction, autonomic nerve involvement and conduction blocks were analyzed.To explore A wave value in clinical severity and short-term prognosis of AIDP, the age, gender, clinical severity, conduction blocks, short-term prognosis of the 2 groups were analyzed in A wave with AIDP (18 cases) and non-A wave with AIDP(22 cases).@*Results@#Compared with non-A wave GBS patients, A wave GBS patients had more conduction blocks(10 cases vs.2 cases, χ2=18.021, P=0.000). Age, sex, precedent infections, cranial nerve involvement, autonomic nerve involvement were not significantly statistically different(all P>0.05). A wave was only seen in AIDP subtype(18 cases), and the percentage of A wave in AIDP was 45%(18/40 cases). There was no A wave in axonal GBS.Compared with non-A wave in AIDP, A wave in AIDP patients had more conduction blocks(10 cases vs.2 cases, χ2=9.924, P=0.002), poorer clinical motor function[(3.39±1.09) scores vs.(2.50±1.01) scores, t=2.667, P=0.011]and short-term prognosis[(2.06±0.64) scores vs.(1.55±0.60) scores, t=2.607, P=0.013].@*Conclusions@#A wave is correlated with demyelination subtype in children′s Guillain-Barré syndrome and poor short-term prognosis in AIDP.
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Objective To explore the clinical menifestation, electrophysiological characteristics and prognosis of demyelinating and axonal Guillain-Barré syndrome (GBS) in children. Method A total of 81 children with GBS were divided into demyelinating and axonal subtypes according to the results of two electrophysiological examinations. And the clinical, neuro electrophysiological characteristics and prognosis of the two groups were analyzed. Results There were 60 cases of demyelinating GBS and 21 cases of axonal GBS. In children with axonal GBS, there were 5 cases of reversible conduction block. The interval of onset to fastigium in axonal GBS was shorter than that of demyelinating subtype, and blood antiganglioside antibody was more common, and there were statistically differences (P all<0.05). The age at onset, the history of the prodromal infection, the sensory symptoms, the cranial nerve involvement, the impairment of the autonomic nervous function, the cerebrospinal fluid protein-cell separation, and the HG scores at the time of admission and during fastigium were similar between the two groups (P>0.05). Children with reversible conduction block had faster recovery than those without reversible conduction block in axonal GBS, and there was statistical differences (P<0.01). There was no difference in short-term prognosis (2 months after discharge) and long-term prognosis (1 years after discharge) between the axonal GBS and demyelinating GBS children (P>0.05). Conclusion Axonal GBS clinically progressed more rapidly than demyelinating subtype, but there was no difference in prognosis between them. Also, axonal GBS with a reversible conduction block recovered faster.
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Objective To explore the prognostic factors in Guillain Barre syndrome (GBS) in children. Methods A total of 125 children with GBS were included and grouped according to their independent walking at two and six months after discharge, and their clinical data were analyzed. Results In 125 children (74 males, 51 females) the average age was 84.49±25.32 months, and 41 were under 6 years old. 102 children had a history of prodromal infections. 32 children had cranial nerve involvement and 35 had autonomic nerve involvement. 12 children need assisted respiration. At 2 and 6 months after discharge, when compared with children who could walk independently, the rates of functional score > 3, cranial nerve involvement, and neuroelectrophysiology as denervation potential were higher in children who could not walk independently, and the differences were statistically significant (P all<0.05). Conclusions The factors that affect the short-term prognosis are denervation potential in neuroelectrophysiology, cranial nerve involvement, and functional score > 3. Early identification of uniqueness in patients and subsequent development of targeted rehabilitation training should be carried out to improve the prognosis.
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Objective To investigate the probability and timing of childhood Miller-Fisher syndrome (MFS) progressing to Bickerstaff brainstem encephalitis (BBE), classical Guillain-Barre syndrome (GBS), and pharyngeal-cervical-brachial (PCB-GBS). Methods The clinical data of 128 children with confirmed MFS diagnosis were retrospectively analyzed. Results Among 128 children, 60 cases were simple MFS (ocular muscle paralysis, ataxia, reflexes diminished or disappeared, without limbs weakness and lethargy; laboratory tests suggest cerebrospinal fluid protein-cell separation and/or serum anti-GQ1b antibody positive), 28 cases developed MFS/PCB-GBS (met MFS diagnosis criteria, accompanied by weakness of pharynx, neck and upper limb, weakened or disappeared of upper limb reflex, without weakness of lower limb), 22 cases developed MFS/GBS (met MFS diagnosis criteria, accompanied by weakness of limb), 18 cases developed MFS/BBE (met MFS diagnosis criteria, accompanied by lethargy, pyramidal tract positive). There were no differences in the age at onset, the interval from onset to the start of the treatment, Hughes functional grading, and the percentage of cases having a history of preceding infections, the rate of positive serum anti-GQ1b antibody, the ratio of albumin cytological dissociation in cerebrospinal fluid among 4 groups (P>0.05). The interval from MFS onset to progression to MFS/PCB-GBS, MFS/GBS, or MFS/BBE was within 10 days. Conclusions In children with MFS, 50% developed PCB-GBS, GBS, or BBE, which occurred within 10 days after onset. Clinicians should pay attention to the time window and adjust the medicine rationally.
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Objective To explore the features of nerve electrophysiology in Miller-Fisher syndrome (MFS) in children.Methods Thirty-four children with MFS were selected.The examinations of routine motor sensory nerve conduction,H reflexes of soleus muscle and F wave of median and tibial nerevs were performed.At the same time,33 healthy children of the same age were selected as control group and 30 children with typical Guillain-Barre syndrome (GBS) selected as GBS group,which were compared with children with MFS.Results In children with MFS,routine motor nerve conduction velocity and amplitude,and sensory nerve conduction velocity were in normal range.Twelve cases (35.29％) showed a decrease in sensory nerve amplitude potential (SNAP),5 cases (14.71％) showed sural sparing phenomena (the decline degrees of SNAP in median or ulnar nerve greater than in sural nerve),22 cases (64.71％) showed abnormality of H reflex,and 12 cases (35.29％) showed the decline of SNAE Compared with control group,there was no difference in sensory nerve conduction velocity and SNAP in sural nerve in children with MFS (P＜0.05);While there were significant decreases in SNAP in both median nerve and ulnar nerve (P＜0.05).Compared with GBS group,children with MFS had significantly higher sensory conduction amplitude and faster velocity (P＜0.01).Conclusion A decrease of terminal sensory nerve conduction amplitude could be found in children with MFS,but,the SNAP had limited impact on it.H reflex abnormality is the most common electrophysiologic abnormalities in MFS.
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Objective To investigate the factors in diagnosis delay in Guillain-Barré syndrome (GBS) and its impact on prognosis.Methods In this study 118 GBS children including Miller-Fisher syndrome (MFS) and pharyngeal-cervical-brachial Guillain-Barré syndrome (PCB) were studied.All children included were divided into 2 groups as GBS-initially-diagnosed group (GBSid,n=76) and not-GBS-initially diagnosed group (nGBSid,n=42) based on the initial diagnosis.Analysis was performed with age at disease onset,preceding infection,Hughes functional grading (HG),the department where the instial diaghosis is done,main complain,the days from disease onset to seeing doctor,time start to treatment,the discharge time,evaluation by a neurologist.Results Among 118 GBS,90 children were of classical GBS,13 of MFS,and 6 of PCB.Atypical muscle weakness,neuropathic pain and impaired respiration function were more frequently seen in nGBSid group (P＜0.05).At the initial diagnosis,lacking of neurological evaluation was found more frequently in nGBSid group (P＜0.05).The duration from onset to the commencement of treatment was longer in nGBSid group than that in GBSid group (P＜0.05),and short term prognosis was poor in GBSid group (P＜0.05).Conclusions Atypical main complaints including neuropathic pain,the impaired respiration function and atypical muscle weakness,and lack of neurological evaluation were all associated with a delay in considering the diagnosis of GBS.The delay in diagnosis had a significant impact on short term prognosis.