评估勒伯遗传性视神经病变患者服用意地美酮后的客观视觉功能。

IF 2.1 3区 医学 Q2 OPHTHALMOLOGY
Japanese Journal of Ophthalmology Pub Date : 2024-09-01 Epub Date: 2024-07-05 DOI:10.1007/s10384-024-01077-z
Yoichiro Masuda, Hiroto Ishikawa, Hitoshi Ishikawa, Takeshi Kezuka, Atsushi Miyazaki, Kenji Matsumoto, Fumi Gomi, Osamu Mimura, Keigo Shikishima, Tadashi Nakano, Masahiko Terao
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引用次数: 0

摘要

目的:为了客观评估勒伯遗传性视神经病变(LHON)患者的视觉功能,本研究使用功能磁共振成像(fMRI)评估了利眠宁治疗前后初级视觉皮层(V1)反应的变化,因为视网膜中央神经节细胞受损给主观测试带来了挑战:研究设计:一项涉及四名确诊 LHON 患者的描述性研究:四名患者连续 24 周每天口服 900 毫克的依地美酮。将基线和治疗后视力、视野以及被动观看漂移对比模式视觉刺激时的 BOLD fMRI 反应与自我报告的症状进行比较:结果:治疗后,一名患者在主观测试、报告症状和 fMRI 方面均表现出积极趋势。两名患者的症状和 fMRI 反应稳定;其中一名患者的主观测试有所改善,另一名患者则略有恶化。结论:fMRI 可为 LHON 患者的视觉功能提供一种有价值的客观测量方法,而且在评估症状方面似乎更具相关性。需要对更多参与者进行进一步研究,以确定 fMRI 在制定客观视觉评估和治疗评估中的作用。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

Assessment of objective visual function following idebenone administration in patients with leber hereditary optic neuropathy.

Assessment of objective visual function following idebenone administration in patients with leber hereditary optic neuropathy.

Purpose: To objectively assess visual function in Leber's Hereditary Optic Neuropathy (LHON) patients; this study evaluated pre- and post-idebenone treatment changes in primary visual cortical (V1) responses using functional magnetic resonance imaging (fMRI), given the challenges in subjective testing due to central retinal ganglion cell damage.

Study design: A descriptive study involving four confirmed LHON patients.

Methods: Four patients received 900 mg/day of oral idebenone for 24 weeks. Baseline and post-treatment visual acuity, visual fields, and BOLD fMRI responses while passively viewed drifting contrast pattern visual stimuli were compared with self-reported symptoms.

Results: Post-idebenone, one patient showed positive trends across subjective tests, reported symptoms, and fMRI. Two patients had stable symptoms and fMRI responses; one improved on subjective tests, and another worsened slightly. Another patient improved in visual field tests despite worsening symptoms and fMRI trends.

Conclusion: fMRI may offer a valuable objective measure of visual functions in LHON and appears to be more relevant in assessing symptoms. Further research with more participants is needed to ascertain fMRI's role in developing objective visual assessments and treatment evaluation.

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来源期刊
CiteScore
4.80
自引率
8.30%
发文量
65
审稿时长
6-12 weeks
期刊介绍: The Japanese Journal of Ophthalmology (JJO) was inaugurated in 1957 as a quarterly journal published in English by the Ophthalmology Department of the University of Tokyo, with the aim of disseminating the achievements of Japanese ophthalmologists worldwide. JJO remains the only Japanese ophthalmology journal published in English. In 1997, the Japanese Ophthalmological Society assumed the responsibility for publishing the Japanese Journal of Ophthalmology as its official English-language publication. Currently the journal is published bimonthly and accepts papers from authors worldwide. JJO has become an international interdisciplinary forum for the publication of basic science and clinical research papers.
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