Clinical analysis of anti-ganglioside antibody-positive variant Guillain-Barré svndrome spectrum diseases
Objective To investigate the clinical characteristics and prognosis of children with anti-ganglioside antibody-positive variant Guillain-Barré syndrome(GBS)spectrum disease.Methods The clinical data and prognosis of 6 children with variant GBS spectrum disease in Henan Provincial People's Hospital from August 2022 to July 2023 were analyzed,including clinical symptoms,signs,cerebrospinal fluid biochemical examination,interleukin(IL)-1β,IL-5,IL-6,IL-8,IL-17,anti-ganglioside antibodies,neuroelectrophysiological examination,head MRI scan and treatment outcomes.Results In 6 patients,there were 3 patients with Miller-Fisher syndrome,2 with Bickerstaff's brainstem encephalitis,and 1 with facial palsy complicated with mydriasis.The clinical manifestations included visual acuity loss in 4 patients,eye movement disturbance in 2,eyelid ptosis in 2,bulbar paralysis in 2,limb weakness and weakened tendon reflex in 2,hyperesthesia in 1,painful ophthalmoplegia in 1,and facial paralysis with dilated pupil in 1.Cerebrospinal fluid detected protein-cell separation in 2 patients.Mycoplasma pneumoniae antibody was positive in 1 patient,Epstein-Barr virus antibody was positive in 1,herpes simplex virus Ⅰ antibody was positive in 2,cytomegalovirus antibody was positive in 3 and rubella vires antibody was positive in 3.IL-1β,-5,-6,-8 and-17 increased in different degrees in 5 patients.Single anti-ganglioside antibody was positive in 3 patients,two anti-ganglioside antibodies were positive in 2,and six anti-ganglioside antibodies were positive in 1.Neuroelectrophysiological examination was performed in 5 patients,among whom 3 patients had flattened amplitude and prolonged latency of facial nerve conduction,1 patient had low amplitude and slowed conduction velocity of motor nerve conduction in the upper limb,and 1 had disappeared H wave in the lower limb.Head MRI showed no specific changes in 6 patients.Glucocorticoid treatment was effective.The follow-up survey was conducted for 1 year,showing GBS disability sacle score ≥2,and a good prognosis.One patient relapsed in 1 month after onset and one patient relapsed in 2 months after onset,who were relieved after readministration of glucocorticoid.One patient was complicated with epilepsy,and no clinical seizure of epilepsy was found at present after oral administration of levetiracetam tablets and magnesium valproate sustained-release tablets for more than 3 years.Conclusions The variant GBS spectrum disease is diverse and overlapping.The more the types of positive anti-ganglioside antibodies,the severer the clinical symptoms.Some of the patients develop recurrences and the overall prognosis is good.