| چکیده انگلیسی مقاله |
Myasthenia gravis (MG) is a disorder of the neuromuscular junction. In children, the disease may be congenital or due to an autoimmune process. Clinical manifestations may be purely ocular or may include generalized weakness, fatigability, dysphagia, and disorders of speech. In this article, 27 children (14 female, 13 male) were prospectively studied from birth to age fifteen. Disease presentation was ocular in all the cases, with ptosis and diplopia. In 6 patients (all female), the disease was purely ocular, in the remaining 21, the disease was generalized. There was one case of transient neonatal myasthenia born to a myasthenic mother. Nine patients underwent thymectomy; all were reported as hyperplasia. Clinical consequences of the surgery were satisfactory. Five cases had hereditary myasthenia. In three brothers from one family, and a brother and sister from another family, disease presented from the neonatal period. Overall, compared to adults, myasthenia seems to have a more favorable course in children. In the autoimmune form of the disease, thymectomy has a very good result. Thymoma was not observed in these cases. MG in the newborn of myasthenic mothers had a very good prognosis, and treatment is necessary in the first few weeks only in the presence of clinical manifestations. In the congenital form of the disease, there is no indication for administration of steroids, thymectomy, or plasmapheresis. |
