Neuromyelitis Optica Complicated by Ornithine Transcarbamylase Deficiency Treated Safely with Pulse Steroid Therapy

Ryuto Yoshida; Takuya Sasaki; Takashi Sunouchi; Masayuki Ueda; Mizuki Kitamura; Takahiro Nakayama; Jun Saito; Ichiro Imafuku

doi:10.2169/internalmedicine.6885-20

CASE REPORTS

Neuromyelitis Optica Complicated by Ornithine Transcarbamylase Deficiency Treated Safely with Pulse Steroid Therapy

Ryuto Yoshida, Takuya Sasaki, Takashi Sunouchi, Masayuki Ueda, Mizuki Kitamura, Takahiro Nakayama, Jun Saito, Ichiro Imafuku

Author information

Keywords: ornithine transcarbamylase deficiency, neuromyelitis optica, pulse steroid therapy, urea cycle disorders

JOURNAL OPEN ACCESS

2021 Volume 60 Issue 20 Pages 3321-3324

DOI https://doi.org/10.2169/internalmedicine.6885-20

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Abstract

Steroid administration to patients with urea cycle disorders can cause hyperammonemia. We encountered a 36-year-old woman with neuromyelitis optica (NMO) complicated by ornithine transcarbamylase (OTC) deficiency. By reducing the doses of steroids and adequate infusion management, we were able to administer pulse steroid therapy without any severe complications. This case indicates the safety of steroid treatment in patients with urea cycle disorders.

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