July 2018
Volume 59, Issue 9
Open Access
ARVO Annual Meeting Abstract  |   July 2018
Therapeutic effects of Idebenone in Japanese patients with Leber hereditary optic neuropathy: A multicenter prospective study
Author Affiliations & Notes
  • Hiroto Ishikawa
    Ophthalmology, Hyogo College of Medicine, Nishinomiya, Hyogo, Japan
  • Yoichiro Masuda
    Ophthalmology, The Jikei University School of Medicine, Minato-ku, Tokyo, Jersey
  • Fumi Gomi
    Ophthalmology, Hyogo College of Medicine, Nishinomiya, Hyogo, Japan
  • Keigo Shikishima
    Ophthalmology, The Jikei University School of Medicine, Minato-ku, Tokyo, Jersey
  • Toshiaki Goseki
    Ophthalmology, Kitasato University School of Medicine, Sagamihara, Kanagawa, Japan
  • Hitoshi Ishikawa
    Ophthalmology, Kitasato University School of Medicine, Sagamihara, Kanagawa, Japan
  • Takeshi Kezuka
    Ophthalmology, Tokyo Medical University, Shinjuku-ku, Tokyo, Japan
  • Masahiko Terao
    Research Institute for Time Studies, Yamaguchi University, Yamaguchi, Japan
  • Atsushi Miyazaki
    Tamagawa University Brain Science Institute, Tokyo, Japan
  • Kenji Matsumoto
    Tamagawa University Brain Science Institute, Tokyo, Japan
  • Hiroki Nishikawa
    Center for Clinical Research and Education, Hyogo College of Medicine, Nishinomiya, Hyogo, Japan
  • Rika Okamoto
    Center for Clinical Research and Education, Hyogo College of Medicine, Nishinomiya, Hyogo, Japan
  • Osamu Mimura
    Ophthalmology, Hyogo College of Medicine, Nishinomiya, Hyogo, Japan
  • Footnotes
    Commercial Relationships   Hiroto Ishikawa, None; Yoichiro Masuda, None; Fumi Gomi, None; Keigo Shikishima, None; Toshiaki Goseki, None; Hitoshi Ishikawa, None; Takeshi Kezuka, None; Masahiko Terao, None; Atsushi Miyazaki, None; Kenji Matsumoto, None; Hiroki Nishikawa, None; Rika Okamoto, None; Osamu Mimura, None
  • Footnotes
    Support  None
Investigative Ophthalmology & Visual Science July 2018, Vol.59, 3343. doi:
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      Hiroto Ishikawa, Yoichiro Masuda, Fumi Gomi, Keigo Shikishima, Toshiaki Goseki, Hitoshi Ishikawa, Takeshi Kezuka, Masahiko Terao, Atsushi Miyazaki, Kenji Matsumoto, Hiroki Nishikawa, Rika Okamoto, Osamu Mimura; Therapeutic effects of Idebenone in Japanese patients with Leber hereditary optic neuropathy: A multicenter prospective study. Invest. Ophthalmol. Vis. Sci. 2018;59(9):3343.

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      © ARVO (1962-2015); The Authors (2016-present)

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Abstract

Purpose : To verify the therapeutic effects of Idebenone in Japanese LHON patients.

Methods : This was a multicenter, prospective, single arm, non-randomized and open-label confirmatory study. Between October 2013 and May 2016, a total of 57 LHON patients (52 males, 5 females; mean age: 28.9 ± 17.7 years) were enrolled (4 patients dropped out during the follow-up period). Study participants received a supplement containing Idebenone at 900mg/day for 24 weeks. Ophthalmological examinations including best collected visual acuity (BCVA), visual field (VF), critical fusion frequency (CFF), and retinal nerve fiber layer thickness (RNFLT), were performed every 2 months, and blood analysis was performed every 6 months. In 4 patients, we performed exploratory examinations by fMRI to assess brain activities before and after Idebenone therapy.

Results : Mitochondrial mutation 11778 G>A was found in 94.7% of the patients. No patients showed any clinically significant adverse events. Among the patients who showed improvements in BCVA at 12M, significant improvement was observed in 32.1%. Also, for VF and CFF, significant improvements were observed in 24.5% and 34.0% respectively. In all eyes, RNFLT showed significant decreases at 12M. In fMRI, 1 of 4 patients who had clinical efficacy for Idebenone showed improvements.

Conclusions : The therapeutic effects of Idebenone with a favorable safety profile were confirmed in Japanese LHON patients in this study.

This is an abstract that was submitted for the 2018 ARVO Annual Meeting, held in Honolulu, Hawaii, April 29 - May 3, 2018.

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