| Background & ObjectiveGuillain-Barré syndrome(GBS),an autoimmune peripheral neuropathy,is one of the most common neurological disorders leading to acute paralysis after the advent of polio vaccine.In adults,we can make a preliminary judgment according to the patient’s complaint,but for children who are unable to describe their symptoms accurately,it is easy to cause diagnostic delay,which may lead to serious consequences or even death.In addition,early studies showed that GBS mainly involved children and young adults in northern China,and axon subtypes accounted for the majority.The purpose of this study was to investigate the epidemiological,clinical and electrophysiological characteristics of GBS in children in southern China,and to analyze its short-term prognostic factors.Methods[1] Participants: The data of the patients from 24 hospitals in 10 provinces /municipalities / autonomous regions in southern China(south of Huaihe River)were collected,including 13 first-class hospitals in Hubei Province and 11first-class hospitals outside Hubei Province.From January 2013 to September2016,patients under the age of 18 who were discharged and diagnosed as GBS were mostly hospitalized patients from Department of Neurology,some of them were from Department of Pediatrics.[2] Data extraction and sorting: 1)Demographic data--Gender,age,residence.2)Time process--Time of admission and discharge time;hospitalization days.3)Clinical manifestations--Onset time,preceding events,initial symptoms,vital signs,other neurological signs such as cranial nerves,tendon reflexes and autonomic nerves,complications;4)Results of routine laboratory tests--Registering mainly the routine tests results of cerebrospinal fluid and related antibody examination.5)Electrophysiological results--Registering mainly several parameters of routine nerve conduction studies,including amplitude,distal motor latency,motor and sensory velocity of compound muscle action potential and sensory nerve action potential of median,ulnar,tibia and fibula nerves;F wave latency and its occurrence rate.6)Disease evolution--Hughes GBS disability scores at admission,nadir and discharge;whether mechanical ventilation required and the duration of use;whether or not to die and the time.7)Treatment plan--Mainly the drugs used.[3] Inclusion Criteria: After extracting the patients’ data from the medical records according to the registered information,two neurologists in our hospital discussed and jointly decided whether to meet the inclusion criteria of this study.All patients must meet the diagnostic criteria of Asbury and Cornblath and the National Guidelines.[4] Patient grouping: 1)Age and place of residence--The patients were divided into≤ 5-years-old group and > 5-years-old group with 5-years-old as the boundary;the patients were divided into rural and urban areas,according to the long-term residential address at the time of referral.2)Seasons--Four seasons according to the climatic conditions of southern China--Spring(March to May),summer(June to July),autumn(September to November)and winter(December to February of the following year).3)Whether there was a precursor event.4)Whether there was cerebral nerve involvement.5)Whether autonomic nerve functions were involved-mainly including heart rate / rhythm,blood pressure,gastrointestinal and urinary symptoms,sweating,etc.6)Electrophysiological type--Subtypes were classified according to the currently recognized electrophysiological standards.[5] Statistical analysis: Measurement data conforming to normal distribution were expressed by mean ± standard deviation,independent t-test is used for inter group comparison,chi square analysis and Fisher accurate test were used for inter group comparison of count data.Results[1] General data: A total of 78 children with GBS were included.There were 50 males and 28 females,with a sex ratio of 1.78:1.The average age was 9.53 ± 5.44 years.The peak age of onset was 1 and 14 years.There were 43 cases were in rural areas and 35 in urban areas.The ratio of the patients’ onset in the seasons of winter and spring accounted for 61.5% and summer and autumn 38.5%(χ~ 2 =4.154,P = 0.042).The first symptom in 84.6% patients(66 / 78)was limb weakness,10.2% patients(8 / 78)had limb pain,2.6%(2 / 78)patients had ataxia,and 10.2%(8 / 78)patients had hyponatremia.[2] Age comparison: There was no difference between two groups of ≤ 5 and >5-years-old in gender composition,season of onset,Hughes score at nadir and discharge and proportion of the patients with cranial nerve involvement and autonomic dysfunctions;the proportion of patients with Hughes score > 3 in the group of ≤ 5 years-old was slightly higher than that in the group of > 5 years old,but there was no statistical difference.[3] Preceding events: The proportion of pediatric GBS patients with preceding events was 64.1%(50/78).Of the 50 patients with preceding events,56%(28 / 50)were respiratory infection.There were no differences in age,sex composition,residence,Hughes disability score,the proportion of cranial and autonomic nerve involvement between the two groups with and without preceding events;the proportion of patients with preceding events in spring and winter tended to increase.[4] Cranial nerve involvement: The proportion of cranial nerve involvement in GBS children was 35.9%(28 / 78).There were 6 cases of multiple cranial nerves and22 cases of single cranial nerve.The proportion of bulbar palsy was 71.4%(20 /28),of which 2 cases needed assistant respiration.The proportion of facial nerve palsy was 42.9%(12 / 28),including 3 cases of unilateral facial palsy.There were3 cases with involvement of cranial nerve Ⅲ,1 case with involvement of cranial nerve Ⅵ and 1 case with involvement of cranial nerve Ⅷ.Compared to the group without cranial nerve involvement,the proportion of patients with cranial nerve involvement was significantly less(P = 0.021),and the number of days in hospital stay tended to increase(P = 0.067).[5] Autonomic nerve involvement: The proportion of children with GBS in autonomic nerve involvement was 28.3%(22 / 78).Among the 18 patients,there were 12 cases with sinus tachycardia,some with sinus arrhythmia,,hypertension,etc.;remaining 4 patients with urinary retention,1 gastroparesis and 1 sweating,1case with both sinus tachycardia and urinary retention.Among the 18 patients,there were 5 patients required mechanical ventilation,1 died during hospitalization and 3 cases(3.8%)complicated with cranial nerve involvement.In patients with autonomic dysfunction,cranial nerve involvement occurred much less,mechanical ventilation required and disability at discharge was severe in higher proportions compared with those without autonomic nerve involvement.[6] Electrophysiology: Among GBS children with complete electrophysiological data,demyelination subtype accounted for 37.7%(20 / 53),axon subtype 22.6%(12 /53),uncertain 12,and nerve conduction was normal in 9(χ~2 = 5.038、P = 0.169).There were no differences between two groups of demyelinating and axonal subtypes in age,gender,place of residence,Hughes score,cranial nerve involvement,autonomic dysfunction and length of hospital stay.[7] Therapeutic regimens.Among 78 children with GBS,there were 30 cases(38.5%) treated with intravenous immunoglobulin(IVIg),21 combined medication of steroid and IVIg,8 steroid alone,2 plasma exchange(PE)alone,2 nerve growth factor of rat alone,1 combined medication of steroid and PE,1 combined medication of IVIg and PE,and 13 without special treatment.ConclusionIn children with GBS of southern China,boys are more common,there is a bimodal age distribution(1-year-old,14-years-old);there is increased trend of incidence in winter and spring;more than half of the preceding events are respiratory tract infections.It is worth noting that more than 15% of GBS children whose first symptom are not limb weakness,of them most manifest pain in the limbs.Although the patients with symptoms of autonomic dysfunction rarely have cranial nerve involvement at the same time,their short-term prognosis is poor;once autonomic dysfunction occurs,strengthen monitoring is required.Different from GBS predominated with axon subtype in northern China,demyelination subtype is more common in GBS children in southern China.From the perspective of onset season,age,preceding events,first symptoms,cranial nerve involvement,autonomic dysfunction and electrophysiological classification,this study made a retrospective analysis of children with GBS in southern China,providing a strong basis for the prevention and further study of children with GBS. |
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