| Objective: Analyze the clinical data of ocular myasthenia gravis in220 children,to explore the clinical features,therapeutic efficts of different treatments and prognostic factors.Methods: The clinical data of ocular myasthenia gravis in 220 children were collected,who were followed up by clinic and telephone(144 cases were followed up successfully for more than 6 months).The clinical features and auxiliary examinations were analyzed retrospectively,and therapeutic efficts of different treatments and prognostic factors were evaluated.Results:1.The male-to-female sex ratio is 1 to 2.55.The average age of onset was 3.67±3.06 years,and the most cormnon age of onset was 1 to3 years old(58.18%).2.All the patients had extraocular myasthenia symptoms,but the first symptoms were different.At onset,199 cases had ptosis,20 cases had ptosis and diplopia simultaneously,and 1 case had only diplopia.40 cases had predisposing factors,10 cases(4.55%)had family history and 8 cases(3.64%)had allergic history.3.The positive rate of the Neostigmine test,Repetitive Neuroelectric Stimulation,myasthenia gravisrelated antibody,thyroid function,and autoantibody was 98.18%,24.43%,39.13%,16.58%,and 13.71%.The abnormal rate of complement and immunoglobulin was 34.11%,and the abnormal rate of thymus was 1.02%.4.144 patients were successfully followed up.The remission rate was49.09%(54/110)in 110 patients treated with pyridostigmine bromide,and after added with glucocorticoid or/and IVIG,the remisson rate can reach 77.27%(85/110).62 patients were treated with pyridostigmine bromide and glucorticosteroid,and the remission rate was 83.87%(52/62).There were no differences in the efficacy of three groups of different glucorticosteroids treatment(p>0.05).11 patients were treated with IVIG combined with pyridostigmine bromide or/and glucorticosteroid,the remission rate was 63.64%(7/11).5.In 144 cases,111 cases(111/144,77.08%)reached the state of remission at the end of the follow-up and did not turn into generalized myasthenia gravis,while 4 cases(4/144,2.78%)developed into GMG,of which 2 cases had myasthenia crisis.Patients with myasthenia crisis can be relieved by ventilator support,neostigmine,glucocorticoid,IVIG or plasma exchange.The prognosis of OMG had an sinificant correlation with repeated neuroelectrical stimulation(p<0.05),but not related to gender,family history,predisposing factors,first symptoms,thyroid function and autoantibody.Conclusion: 1.The morbidity of OMG in female children is more than male,and the peak ages is 1 to 3 years old.Ptosis and / or diplopia aremostly found,and some are accompanied by ocular movement disorders.Ptosis is the most symptom at onset.2.Few children with OMG are combined with other autoimmune diseases or thymus abnormality.The abnormal rate of thyroid function is 16.58%,but often lack of clinical manifestations.which should be vigilant and screening.3.Cholinesterase inhibitors can be considered at first when thinking about treatment of ocular myasthenia gravis in children,and glucocorticoid and/or IVIG should be used when symptoms are not relieved,after the combined treatments,a higher rate of remission can be obtained.The curative efficacy of different GC treatments in OMG in children was similar.4.The proportion of OMG developed into GMG is low(2.78%),myasthenia crisis is rare(1.39%),and patients with myasthenia crisis should be given the comprehensive treatment of the ventilator support,neostigmine,glucocorticoid and IVIG in time.5.The abnormal rate of repetitive neuroelectrical stimulation may have an impact role on the prognosis of OMG,while the other factors were not found in this study,which should be further studied in the future reseach. |
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