Research Information System
JOURNAL OF CLINICAL AND ANALYTICAL MEDICINE, vol.6, no.4, pp.473-478, 2015 (ESCI, Scopus, TRDizin)
Aim: Juvenile myasthenia gravis accounts for 10% to 15% of all cases and it is even rarer in prepubertal children. Therefore, limited data are available on its presentation, diagnosis, treatment and outcome, particularly in younger children. Early diagnosis is imperative for delaying the progression of the disease, and prevention and adequately management of the myasthenic crisis. Presence of acetylcholine receptor antibodies which occur only in half of prepubertal children wih myastenia helps in the diagnosis. Seronegative cases need to be distinguished from congenital forms which may present with similar symptoms. Material and Method: This article presents the initial presentation, clinical course, and 2-year follow-up results of 3 patients with juvenile myasthenia gravis, and reviewes the disease in general and current treatment modalities. Results: The ages at onset were 3, 4 and 7 years. The initial symptom was fluctuating pitosis in all cases. Acetylcholine receptor antibodies were below the upper limit of normal range in the younger 2 patients, and became positive at 12th months of follow-up with a 2-fold increase. These 2 patients remained symptom-free with pyridostigmine therapy during the 21-month follow-up period. In the remaining patient who had high antibody titers, symptoms progressed to involve other muscle groups at 10th months of follow-up period while receiving pyridostigmine. After lack of response to prednisolone trial, symptoms improved with azathioprine and monthly intravenous immunoglobulin therapy. Discussion: Juvenile myasthenia gravis in younger children with low acetylcholine receptor antibody titers follows a benign course. Acetylcholine receptor antibodies which were negative at onset, may become positive at 1-year of follow-up. Older children with high antibody titers at onset follow more aggressive disease course needing immunosuppressive therapy.