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A 5-Year Follow-Up of Triple-Seronegative Myasthenia Gravis Successfully Treated with Tacrolimus Therapy.
Tozawa T1,2, Nishimura A3, Ueno T1,4, Kaneda D1,2, Miyanomae Y5, Chiyonobu T2, Morimoto M2, Hosoi H2.
Neuropediatrics. 2018 Jan 4. doi: 10.1055/s-0037-1618591.
Abstract
Seronegative myasthenia gravis (MG) is a generalized form of MG that is diagnosed on the basis of clinical symptoms, electrophysiological testing, and pharmacological responses, in the absence of a seropositive status for anti-acetylcholine receptor (AChR) antibodies.
Generalized MG that is seronegative for anti-AChR, anti-muscle-specific kinase (MuSK), and anti-low density lipoprotein receptor related protein 4 (Lrp4) antibodies is known as triple-seronegative MG.
We here describe a case of triple-seronegative MG in an 8-year-old boy.
His first symptom was dysphagia, at 3 years of age, and he subsequently developed ptosis, rhinolalia, and a waddling gait.
A genetic analysis was conducted to exclude the possibility of congenital myasthenia syndrome due to the patient's resistance to steroid therapy.
His condition was successfully managed with tacrolimus therapy over a 5-year follow-up period.
Recently, several studies have reported the therapeutic utility of tacrolimus in juvenile seropositive MG; in contrast, a few reports have described tacrolimus treatment in cases of seronegative MG.
Our findings suggest that tacrolimus therapy is a safe and effective option for the treatment of juvenile seronegative MG.
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