Japanese cases of neuromyelitis optica spectrum disorder associated with myasthenia gravis and a review of the literature

Ryotaro Ikeguchi, Yuko Shimizu, Shigeaki Suzuki, Satoru Shimizu, Chiaki Kabasawa, Shiori Hashimoto, Masayuki Masuda, Yuriko Nagane, Kimiaki Utsugisawa, Yasushi Suzuki, Toshiyuki Takahashi, Hiroya Utsumi, Kazuo Fujihara, Norihiro Suzuki, Shinichiro Uchiyama

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7 Citations (Scopus)

Abstract

Background The incidence of concurrent myasthenia gravis (MG) and neuromyelitis optica spectrum disorder (NMOSD) is higher than what chance predicts, yet it remains unclear why MG and NMOSD appear concurrently. Objective The purpose of the present study was to examine the clinical features of the concurrence of these diseases. Methods Clinical details were analyzed retrospectively. Results Three (0.5%) out of 631 MG patients had confirmed (n = 2) or suspected (n = 1) NMOSD. Two of these patients were women. All showed early-onset MG (EOMG) that preceded NMOSD and were positive for acetylcholine receptor antibody (AChR-Ab). Two patients were tested for aquaporin 4 antibody (AQP4-Ab) and were positive. Two patients were treated with a thymectomy that preceded NMOSD. Two patients had decreased frequency of regulatory T (Treg) cells. We identified in the literature 46 patients with both MG and NMOSD. Our results of female predominance, EOMG, MG preceding NMOSD, and positive AChR-Ab are consistent with previous descriptions. Conclusions This is the first report to examine the frequency of NMOSD in Japanese patients with MG. The reduction and/or dysfunction of Treg cells may be one cause of NMOSD development in MG.

Original languageEnglish
Pages (from-to)217-221
Number of pages5
JournalClinical Neurology and Neurosurgery
Volume125
DOIs
Publication statusPublished - 2014 Oct

Keywords

  • Acetylcholine receptor antibody
  • Myasthenia gravis
  • Neuromyelitis optica
  • Regulatory T cell
  • Thymectomy

ASJC Scopus subject areas

  • Surgery
  • Clinical Neurology

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    Ikeguchi, R., Shimizu, Y., Suzuki, S., Shimizu, S., Kabasawa, C., Hashimoto, S., Masuda, M., Nagane, Y., Utsugisawa, K., Suzuki, Y., Takahashi, T., Utsumi, H., Fujihara, K., Suzuki, N., & Uchiyama, S. (2014). Japanese cases of neuromyelitis optica spectrum disorder associated with myasthenia gravis and a review of the literature. Clinical Neurology and Neurosurgery, 125, 217-221. https://doi.org/10.1016/j.clineuro.2014.07.036