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首页> 外文期刊>Journal of neurology >Patterns of white matter diffusivity abnormalities in Leber's hereditary optic neuropathy: a tract-based spatial statistics study.
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Patterns of white matter diffusivity abnormalities in Leber's hereditary optic neuropathy: a tract-based spatial statistics study.

机译：Leber遗传性视神经病变中白质扩散性异常的模式：基于区域的空间统计研究。

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Leber's hereditary optic neuropathy (LHON) is a mitochondrial disease characterized by retinal ganglion cell degeneration and optic nerve atrophy, leading to a loss of central vision. The aim of this study was to explore the topographical pattern of damage to the brain white matter (WM) tracts from patients with chronic LHON using diffusion tensor (DT) MRI and tract-based spatial statistics (TBSS). Brain dual-echo and DT MRI scans were acquired from 13 patients with chronic LHON and 25 matched controls using a 3.0?T scanner. TBSS analysis was performed using the FMRIB's Diffusion Toolbox. A complete neuro-ophthalmologic examination, including standardized automated Humphrey perimetry as well as average and temporal peripapillary retinal nerve fiber layer thickness (PRNFL) measurements, was obtained in all patients. Mean average and temporal PRNFL thicknesses were decreased significantly in LHON patients. Compared to controls, TBSS analysis revealed significant diffusivity abnormalities in these patients, which were characterized by a decreased fractional anisotropy (FA) and an increased mean diffusivity and radial diffusivity, affecting exclusively the optic tracts and optic radiations (OR). In patients, a significant correlation was found between optic tract average FA and mean visual acuity (r?=?0.57, p?=?0.04). In LHON patients, DT MRI reveals a microstructural alteration of the WM along the entire visual pathways, with a sparing of the other main WM tracts of the brain. Damage to the OR may be secondary either to trans-synaptic degeneration, which in turn is due to neuroaxonal loss in the retina and optic nerve, or to local mitochondrial dysfunction.
机译：Leber的遗传性视神经病（LHON）是一种线粒体疾病，其特征在于视网膜神经节细胞变性和视神经萎缩，导致中枢视力丧失。这项研究的目的是使用弥散张量（DT）MRI和基于道的空间统计（TBSS）探索慢性LHON患者对脑白质（WM）道的损害的地形图。使用3.0？T扫描仪从13例慢性LHON患儿和25个匹配的对照中进行了脑双回波和DT MRI扫描。使用FMRIB的扩散工具箱进行TBSS分析。所有患者均获得了完整的神经眼科检查，包括标准化的自动化汉弗莱视野检查以及平均和颞上乳头周围视网膜神经纤维层厚度（PRNFL）测量。 LHON患者的平均和时间PRNFL厚度均明显降低。与对照组相比，TBSS分析显示这些患者的弥漫性异常明显，其特征在于分数各向异性（FA）降低​​，平均弥散性和径向弥散性增加，从而仅影响视线和光辐射（OR）。在患者中，发现视线平均FA与平均视力之间存在显着相关性（r = 0.57，p = 0.04）。在LHON患者中，DT MRI显示整个视觉通路中WM的微结构改变，而大脑的其他主要WM道则保留。对OR的损害可能是继突触变性后继发的，后者又是由于视网膜和视神经中的神经轴突丢失或局部线粒体功能障碍所致。

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