Long-term clinical outcome after extended thymectomy combined postoperative high-dose steroid therapy for juvenile myasthenia gravis.
Myasthenia gravis (MG) is considered to be an autoimmune disorder of neuromuscular transmission and is rare in childhood. We report 3 juvenile MG (JMG) cases of extended thymectomy (ETMX) combined postoperative high-dose steroid therapy. All patients developed MG symptoms under the age of 14 years and were given cholinergic drugs and had generalized MG: the Myasthenia Gravis Foundation of America classification II was present in 1 case and classification III was in 2. All patients were taking pyridostigmine before surgery; none was treated with prednisone preoperatively. All patients performed the ETMX combined postoperative high-dose steroid therapy. Muscle weakness of extremities and bulbar symptoms improved in all patients, but not all exhibited an unstable phase in their clinical course as a result of combined therapy. There was no postoperative morbidity or mortality. All patients had follicular lymphoid hyperplasia without thymoma. Follow-up for more than 5 years has shown one to be in complete remission and the others to have improved symptoms. Although our results are inconclusive because we used only a few JMG patients, the ETMX combined postoperative high-dose steroid therapy appeared to provide a better chance of remission or control of symptoms.
Kanzaki
Masato
M
Department of Thoracic Surgery, Tokyo Metropolitan Fuchu Hospital, Fuchu, Japan.
Obara
Tetsuya
T
Sasano
Susumu
S
Hikawa
Yoshio
Y
Onuki
Takamasa
T
D000293:Adolescent; D002648:Child; D002800:Cholinesterase Inhibitors / Q000008:administration & dosage; D003131:Combined Modality Therapy; D005938:Glucocorticoids / Q000008:administration & dosage*; D006801:Humans; D008297:Male; D009157:Myasthenia Gravis / Q000188:drug therapy* / Q000601:surgery*; D011241:Prednisone / Q000008:administration & dosage*; D011729:Pyridostigmine Bromide / Q000008:administration & dosage; D013934:Thymectomy; D016896:Treatment Outcome
D002800:Cholinesterase Inhibitors; D005938:Glucocorticoids; D011729:Pyridostigmine Bromide; D011241:Prednisone