A 5-Year Follow-Up of Triple-Seronegative Myasthenia Gravis Successfully Treated with Tacrolimus Therapy

Takenori Tozawa; Akira Nishimura; Tamaki Ueno; Daisuke Kaneda; Yuri Miyanomae; Tomohiro Chiyonobu; Masafumi Morimoto; Hajime Hosoi

doi:10.1055/s-0037-1618591

Neuropediatrics, Inhaltsverzeichnis

Neuropediatrics 2018; 49(03): 200-203
DOI: 10.1055/s-0037-1618591

Short Communication

Georg Thieme Verlag KG Stuttgart · New York

A 5-Year Follow-Up of Triple-Seronegative Myasthenia Gravis Successfully Treated with Tacrolimus Therapy

Authors

Takenori Tozawa

¹Department of Pediatrics, Ayabe City Hospital, Ayabe, Japan

²Department of Pediatrics, Kyoto Prefectural University of Medicine, Graduate School of Medical Science, Kyoto, Japan
Akira Nishimura

³Department of Neonatology, Japanese Red Cross Society Kyoto Daiichi Hospital, Kyoto, Japan
Tamaki Ueno

¹Department of Pediatrics, Ayabe City Hospital, Ayabe, Japan

⁴Department of Pediatrics, Tokai Central Hospital, Kakamigahara, Japan
Daisuke Kaneda

¹Department of Pediatrics, Ayabe City Hospital, Ayabe, Japan

²Department of Pediatrics, Kyoto Prefectural University of Medicine, Graduate School of Medical Science, Kyoto, Japan
Yuri Miyanomae

⁵Clinics of Kyoto City Child Well-being Center, Kyoto, Japan
Tomohiro Chiyonobu

²Department of Pediatrics, Kyoto Prefectural University of Medicine, Graduate School of Medical Science, Kyoto, Japan
Masafumi Morimoto

²Department of Pediatrics, Kyoto Prefectural University of Medicine, Graduate School of Medical Science, Kyoto, Japan
Hajime Hosoi

²Department of Pediatrics, Kyoto Prefectural University of Medicine, Graduate School of Medical Science, Kyoto, Japan

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.

Keywords

juvenile generealized myasthenia gravis - steroid-resistant - tacrolimus - triple-seronegative myasthenia gravis

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