title: Quality of life in purely ocular myasthenia in Japan.
abstract: BACKGROUND
  Since there has been no conclusive evidence regarding the treatment of ocular myasthenia, treatment guidelines were recently issued by the European Federation of Neurological Societies/European Neurological Society (EFNS/ENS). However, the therapeutic outcomes concerning the quality-of-life (QOL) of patients with ocular myasthenia are not yet fully understood.
  
  
  METHODS
  We investigated the therapeutic outcomes of patients with purely ocular myasthenia in a multicenter cross-sectional survey in Japan. To evaluate the severity of ocular symptoms, we used the ocular-quantitative MG (QMG) score advocated by Myasthenia Gravis Foundation of America. We used the Japanese translated version of the MG-QOL15, a self-appraised scoring system.
  
  
  RESULTS
  Of 607 myasthenia gravis (MG) patients with an observation-duration of illness ≥ 2 years, the cases of 123 patients (20%) were limited to ocular muscles (purely ocular myasthenia). During the entire clinical course, 81 patients experienced both ptosis and diplopia, 36 had ptosis alone, and six had diplopia alone. Acetyl-cholinesterase inhibitors and prednisolone were used in 98 and 52 patients, respectively. Treatment improved ocular symptoms, with the mean reduction in ocular-QMG score of 2.3 ± 1.8 points. However, 47 patients (38%) failed to gain minimal manifestation or a better status. Patients with unfavorable outcomes also self-reported severe QOL impairment. Multivariate analyses showed that the pretreatment ocular-QMG score was associated with unfavorable outcomes, but not associated with the patient's QOL.
  
  
  CONCLUSIONS
  A treatment strategy designed in accord with a patient's ocular presentation must be considered in order to improve ocular symptoms and the patient's QOL.
authors.affiliation: Department of Neurology, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo 160-8582, Japan. sgsuzuki@z3.keio.jp.
authors.forename: Shigeaki
authors.initials: S
authors.lastname: Suzuki
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Murai
authors.forename: Tomihiro
authors.initials: T
authors.lastname: Imai
authors.forename: Yuriko
authors.initials: Y
authors.lastname: Nagane
authors.forename: Masayuki
authors.initials: M
authors.lastname: Masuda
authors.forename: Emiko
authors.initials: E
authors.lastname: Tsuda
authors.forename: Shingo
authors.initials: S
authors.lastname: Konno
authors.forename: Satoru
authors.initials: S
authors.lastname: Oji
authors.forename: Shunya
authors.initials: S
authors.lastname: Nakane
authors.forename: Masakatsu
authors.initials: M
authors.lastname: Motomura
authors.forename: Norihiro
authors.initials: N
authors.lastname: Suzuki
authors.forename: Kimiaki
authors.initials: K
authors.lastname: Utsugisawa
mesh_terms: D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D002800:Cholinesterase Inhibitors / Q000627:therapeutic use; D003430:Cross-Sectional Studies; D005260:Female; D006801:Humans; D007564:Japan; D008297:Male; D008875:Middle Aged; D009157:Myasthenia Gravis / Q000150:complications* / Q000188:drug therapy*; D011239:Prednisolone / Q000627:therapeutic use; D011788:Quality of Life
chemical_list: D000893:Anti-Inflammatory Agents; D002800:Cholinesterase Inhibitors; D011239:Prednisolone