Open Access Highly Accessed Research article

Quality of life in purely ocular myasthenia in Japan

Shigeaki Suzuki1*, Hiroyuki Murai2, Tomihiro Imai3, Yuriko Nagane4, Masayuki Masuda5, Emiko Tsuda3, Shingo Konno6, Satoru Oji7, Shunya Nakane8, Masakatsu Motomura9, Norihiro Suzuki1 and Kimiaki Utsugisawa4

Author Affiliations

1 Department of Neurology, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo 160-8582, Japan

2 Department of Neurology, Iizuka Hospital, Fukuoka, Japan

3 Department of Neurology, Sapporo Medical University, Hokkaido, Japan

4 Department of Neurology, Hanamaki General Hospital, Iwate, Japan

5 Department of Neurology, Tokyo Medical University, Tokyo, Japan

6 Department of Neurology, Toho University Ohashi Medical Center, Tokyo, Japan

7 Department of Neurology, Saitama Medical Center, Saitama Medical University, Saitama, Japan

8 Department of Neurology, Nagasaki Kawatana Medical Center, Nagasaki, Japan

9 Department of Clinical Neuroscience and Neurology, Graduate School of Biomedical Sciences, Nagasaki University, Nagasaki, Japan

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BMC Neurology 2014, 14:142  doi:10.1186/1471-2377-14-142

Published: 5 July 2014

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.

Conclusion

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.

Keywords:
Ocular myasthenia; Ocular-quantitative myasthenia gravis score; Quality-of-life; Therapeutic outcome