Abstract：
OBJECTIVE: The study aimed to unravel abnormal cerebral blood flow (CBF) in patients with Leber\'s hereditary optic neuropathy (LHON) using arterial spin labeling (ASL) and to investigate the associations among disrupted CBF, disease duration, and neuro-ophthalmological impairment.
METHODS: ASL perfusion imaging data was collected from 20 patients with acute LHON, 29 patients with chronic LHON, and 37 healthy controls. We used a one-way analysis of covariance to test the intergroup differences in CBF. Linear and nonlinear curve fit models were applied to explore the associations among CBF, disease duration, and neuro-ophthalmological metrics.
RESULTS: Brain regions differed in LHON patients, including the left sensorimotor and bilateral visual areas (p < 0.05, cluster-wise family-wise error correction). Acute and chronic LHON patients demonstrated lower CBF in bilateral calcarine than the healthy controls. Chronic LHON had lower CBF in the left middle frontal gyrus and sensorimotor cortex, and temporal-partial junction than the healthy controls and acute LHON. A significant logarithmic negative correlation was shown between CBF of left middle frontal gyrus and disease duration. A significant linear positive correlation was found between retinal nerve fiber layer thickness and CBF in left middle frontal gyrus, and negative correlations between loss of variance and CBF in left middle frontal gyrus and sensorimotor cortex (p < 0.05, Bonferroni correction).
CONCLUSIONS: LHON patients exhibited reduced CBF in the visual pathway, sensorimotor and higher-tier cognitive areas. Disease duration and neuro-ophthalmological impairments can influence the metabolism of non-visual areas.
METHODS: ASL perfusion imaging data was collected from 20 patients with acute LHON, 29 patients with chronic LHON, and 37 healthy controls. We used a one-way analysis of covariance to test the intergroup differences in CBF. Linear and nonlinear curve fit models were applied to explore the associations among CBF, disease duration, and neuro-ophthalmological metrics.
RESULTS: Brain regions differed in LHON patients, including the left sensorimotor and bilateral visual areas (p < 0.05, cluster-wise family-wise error correction). Acute and chronic LHON patients demonstrated lower CBF in bilateral calcarine than the healthy controls. Chronic LHON had lower CBF in the left middle frontal gyrus and sensorimotor cortex, and temporal-partial junction than the healthy controls and acute LHON. A significant logarithmic negative correlation was shown between CBF of left middle frontal gyrus and disease duration. A significant linear positive correlation was found between retinal nerve fiber layer thickness and CBF in left middle frontal gyrus, and negative correlations between loss of variance and CBF in left middle frontal gyrus and sensorimotor cortex (p < 0.05, Bonferroni correction).
CONCLUSIONS: LHON patients exhibited reduced CBF in the visual pathway, sensorimotor and higher-tier cognitive areas. Disease duration and neuro-ophthalmological impairments can influence the metabolism of non-visual areas.
摘要：
目的：该研究旨在使用动脉自旋标记（ASL）揭示Leber遗传性视神经病变（LHON）患者的异常脑血流量（CBF），并研究CBF之间的关系。疾病持续时间,和神经眼科损害。
方法：收集了20例急性LHON患者的ASL灌注成像数据，29例慢性LHON患者，和37个健康对照。我们使用单向协方差分析来检验CBF的组间差异。线性和非线性曲线拟合模型被用来探索CBF，疾病持续时间,和神经眼科指标。
结果：LHON患者的大脑区域不同，包括左侧感觉运动和双侧视觉区域（p<0.05，逐簇家庭误差校正）。急性和慢性LHON患者的双侧calcarineCBF低于健康对照组。慢性LHON左额中回和感觉运动皮层CBF较低，和颞部部分交界处比健康对照组和急性LHON。左额中回CBF与病程呈显著的对数负相关。视网膜神经纤维层厚度与左额中回CBF呈线性正相关,左额中回和感觉运动皮质的方差损失与CBF呈负相关（p<0.05，Bonferroni校正）。
结论：LHON患者在视觉通路中表现出CBF降低，感觉运动和更高层次的认知领域。疾病持续时间和神经眼科损伤可影响非视觉区域的代谢。
方法：收集了20例急性LHON患者的ASL灌注成像数据，29例慢性LHON患者，和37个健康对照。我们使用单向协方差分析来检验CBF的组间差异。线性和非线性曲线拟合模型被用来探索CBF，疾病持续时间,和神经眼科指标。
结果：LHON患者的大脑区域不同，包括左侧感觉运动和双侧视觉区域（p<0.05，逐簇家庭误差校正）。急性和慢性LHON患者的双侧calcarineCBF低于健康对照组。慢性LHON左额中回和感觉运动皮层CBF较低，和颞部部分交界处比健康对照组和急性LHON。左额中回CBF与病程呈显著的对数负相关。视网膜神经纤维层厚度与左额中回CBF呈线性正相关,左额中回和感觉运动皮质的方差损失与CBF呈负相关（p<0.05，Bonferroni校正）。
结论：LHON患者在视觉通路中表现出CBF降低，感觉运动和更高层次的认知领域。疾病持续时间和神经眼科损伤可影响非视觉区域的代谢。
