Abstract：
OBJECTIVE: 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.
METHODS: 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.
CONCLUSIONS: 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.
METHODS: 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.
CONCLUSIONS: 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.
摘要：
目的：为了客观评估Leber遗传性视神经病变（LHON）患者的视觉功能；这项研究使用功能磁共振成像（fMRI）评估了艾地苯醌治疗前后原发性视皮层（V1）反应的变化，考虑到视网膜中央神经节细胞损伤导致的主观测试挑战。
方法：一项涉及4名确诊LHON患者的描述性研究。
方法：4例患者口服艾地苯醌900mg/天，共24周。基线和治疗后视力，视野,将被动观察漂移对比模式视觉刺激时的BOLDfMRI反应与自我报告的症状进行比较。
结果：后艾地苯醌，一名患者在主观测试中显示出积极的趋势，报告的症状，和fMRI。两名患者症状稳定，功能磁共振成像反应稳定；一名患者主观检查有所改善，另一个稍微恶化。尽管症状和功能磁共振成像趋势恶化，但另一名患者的视野检查有所改善。
结论：功能磁共振成像可能为LHON的视觉功能提供有价值的客观测量，并且在评估症状方面似乎更相关。需要对更多参与者进行进一步研究，以确定功能磁共振成像在开发客观视觉评估和治疗评估中的作用。
方法：一项涉及4名确诊LHON患者的描述性研究。
方法：4例患者口服艾地苯醌900mg/天，共24周。基线和治疗后视力，视野,将被动观察漂移对比模式视觉刺激时的BOLDfMRI反应与自我报告的症状进行比较。
结果：后艾地苯醌，一名患者在主观测试中显示出积极的趋势，报告的症状，和fMRI。两名患者症状稳定，功能磁共振成像反应稳定；一名患者主观检查有所改善，另一个稍微恶化。尽管症状和功能磁共振成像趋势恶化，但另一名患者的视野检查有所改善。
结论：功能磁共振成像可能为LHON的视觉功能提供有价值的客观测量，并且在评估症状方面似乎更相关。需要对更多参与者进行进一步研究，以确定功能磁共振成像在开发客观视觉评估和治疗评估中的作用。
