Multidisciplinary treatment for prepubertal juvenile myasthenia gravis with crisis.
The management of juvenile myasthenia gravis (MG) remains controversial. We report herein the case of a 12-year-old girl with prepubertal juvenile MG with respiratory crisis who underwent thymectomy following methylprednisolone pulse therapy. The patient initially developed progressively worsening fatigability, eyelid ptosis, and diplopia, followed by worsening generalized weakness, dysphagia, and dyspnea. Even after i.v. immunoglobulin, the patient presented with rapid onset of severe dyspnea requiring respiratory support with mechanical ventilation and was graded as Myasthenia Gravis Foundation of America class V. After a course of i.v. methylprednisolone pulse therapy, successful control of respiratory crisis was achieved, and trans-sternal thymectomy was performed. Partial remission was achieved postoperatively with oral pyridostigmine without immunosuppressive agents such as steroids or calcineurin inhibitors for 18 months after thymectomy. Early thymectomy following induction methylprednisolone pulse therapy might be a treatment option for prepubertal juvenile MG with severe respiratory crisis.
Department of General Thoracic Surgery, Osaka University Graduate School of Medicine, Osaka, Japan.
Yusuke
Y
Hirata
Department of General Thoracic Surgery, Osaka University Graduate School of Medicine, Osaka, Japan.
Masayoshi
M
Inoue
Department of Pediatrics, Osaka University Graduate School of Medicine, Osaka, Japan.
Shin
S
Nabatame
Department of General Thoracic Surgery, Osaka University Graduate School of Medicine, Osaka, Japan.
Meinoshin
M
Okumura
Department of Pediatrics, Osaka University Graduate School of Medicine, Osaka, Japan.
Keiichi
K
Ozono
D001706:Biopsy; D002648:Child; D005260:Female; D005500:Follow-Up Studies; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D007155:Immunologic Factors / Q000627:therapeutic use; D009157:Myasthenia Gravis / Q000175:diagnosis / Q000628:therapy*; D011239:Prednisolone / Q000627:therapeutic use*; D012121:Respiration, Artificial / Q000379:methods*; D013934:Thymectomy / Q000379:methods*; D013950:Thymus Gland / Q000000981:diagnostic imaging / Q000601:surgery
D005938:Glucocorticoids; D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors; D011239:Prednisolone
crisis; juvenile; myasthenia gravis; steroid pulse therapy; thymectomy