RYOTARO IKEGUCHI
   Department   School of Medicine(Tokyo Women's Medical University Hospital), School of Medicine
   Position   Assistant Professor
Article types Original article
Language English
Peer review Peer reviewed
Title Japanese cases of neuromyelitis optica spectrum disorder associated with myasthenia gravis and a review of the literature.
Journal Formal name:Clinical neurology and neurosurgery
Abbreviation:Clin Neurol Neurosurg
ISSN code:18726968/03038467
Domestic / ForeginForegin
Volume, Issue, Page 125,pp.217-221
Author and coauthor Ikeguchi Ryotaro†, Shimizu Yuko, Suzuki Shigeaki, Shimizu Satoru, Kabasawa Chiaki, Hashimoto Shiori, Masuda Masayuki, Nagane Yuriko, Utsugisawa Kimiaki, Suzuki Yasushi, Takahashi Toshiyuki, Utsumi Hiroya, Fujihara Kazuo, Suzuki Norihiro, Uchiyama Shinichiro
Authorship Lead author
Publication date 2014/10
Summary 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.
DOI 10.1016/j.clineuro.2014.07.036
PMID 25178916