Quantitative assessment of optic nerve in patients with Leber’s hereditary optic neuropathy using reduced field-of-view diffusion tensor imaging

Fan, Ke; Zhang, Yanqiu; Chen, Yanzi; Tian, Qing; Shi, Dapeng

doi:10.1016/j.ejrad.2017.05.025

Cited by 18 publications

(22 citation statements)

References 28 publications

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“…The underlying pathological changes of the optic nerves’ microstructure, reflected by our observed differences in DTI metrics, agreed with the immunohistochemical and ultrastructural findings of the specimens. This observation is identical to those of optic nerve studies regarding glaucoma ( 35 ), Leber’s hereditary optic neuropathy ( 36 ), and optic neuritis ( 37 ). Berna et al ( 18 ) also demonstrated increased MD and decreased FA in TAO patients, but their finding was limited to the midpoint of the optic nerve, and the comparison was performed between non- DON and healthy controls.…”

Section: Discussionsupporting

confidence: 86%

Multi-Parametric Diffusion Tensor Imaging of The Optic Nerve for Detection of Dysthyroid Optic Neuropathy in Patients With Thyroid-Associated Ophthalmopathy

et al. 2022

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ObjectiveTo evaluate the microstructural changes of the orbital optic nerve in thyroid-associated ophthalmopathy (TAO) patients with or without dysthyroid optic neuropathy (DON) using diffusion tensor imaging (DTI) and investigate whether DTI can be used to detect DON.Materials and Methods59 bilateral TAO patients with (n= 23) and without DON (non-DON, n= 36) who underwent pretreatment DTI were included and 118 orbits were analyzed. The clinical features of all patients were collected. DTI parameters, including mean, axial, and radial diffusivity (MD, AD, and RD, respectively) and fractional anisotropy (FA) of the intra-orbital optic nerve for each orbit were calculated and compared between the DON and non-DON groups. ROC curves were generated to evaluate the diagnostic performance of single or combined DTI parameters. Correlations between DTI parameters and ophthalmological characteristics were analyzed using correlation analysis.ResultsCompared with non-DON, the DON group showed decreased FA and increased MD, RD, and AD (P < 0.01). In the differentiation of DON from non-DON, the MD was optimal individually, and the combination of the four parameters had the best diagnostic performance. There were significant correlations between the optic nerve’s four DTI metrics and the visual acuity and clinical active score (P < 0.05). In addition, optic nerve FA was significantly associated with the amplitude of visual evoked potentials (P = 0.022).ConclusionsDTI is a promising technique in assessing microstructural changes of optic nerve in patients with DON, and it facilitates differentiation of DON from non-DON eyes in patients with TAO.

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Section: Discussionsupporting

confidence: 86%

Multi-Parametric Diffusion Tensor Imaging of The Optic Nerve for Detection of Dysthyroid Optic Neuropathy in Patients With Thyroid-Associated Ophthalmopathy

et al. 2022

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“…Nevertheless, we have also observed a correlation between the reduction of right hemisphere BA 19 source signal in the alpha frequency band and the average thickness of the right RNFL in the LHON participants, which suggest that there is a link between the changes in the retinal nerve fiber layer thickness and the reduction of EEG activity in the occipital cortex. Another study showed a unique process from thickening to thinning of the RNFL [ 42 ] and also that the optic tract fractional anisotropy value was significantly correlated with the thickness of the RNFL [ 7 ] in LHON participants. A comparison of previous findings and our results suggests that decreased alpha activity could progress faster with the degeneration of RNFL, but to confirm this thesis, a longitudinal study should be conducted.…”

Section: Discussionmentioning

confidence: 99%

“…The classic 19th-century picture of LHON is used to describe mainly the ophthalmic dysfunctions; however, the development of a wide range of magnetic resonance imaging techniques (MRI), such as diffusion (DTI—diffusion tensor imaging) or functional (fMRI—functional magnetic resonance imaging) protocols, has expanded the current knowledge about the impact of mtDNA diseases on the human central nervous system (CNS) [ 6 , 7 ]. Researchers reported that in LHON participants, CNS abnormalities occurred in different parts of the visual tract, such as the optic chiasm or optic radiations [ 8 , 9 ].…”

Section: Introductionmentioning

confidence: 99%

Widespread Reductions of Spontaneous Neurophysiological Activity in Leber’s Disease—An Application of EEG Source Current Density Reconstruction

Jonak

2020

Brain Sciences

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Leber’s hereditary optic neuropathy (LHON) is a rare, maternally inherited genetic disease caused by a mutation of mitochondrial DNA. Classical descriptions have highlighted structural abnormalities in various parts of patients’ optic tracts; however, current studies have proved that changes also affect many cortical and subcortical structures, not only these belonging to the visual system. This study aimed at improving our understanding of neurophysiological impairments in LHON. First of all, we wanted to know if there were any differences between the health control and LHON subjects in the whole-brain source electroencephalography (EEG) analysis. Second, we wanted to investigate the associations between the observed results and some selected aspects of Leber’s disease’s clinical picture. To meet these goals, 20 LHON patients and 20 age-matched healthy control (HC) subjects were examined. To investigate the electrophysiological differences between the HC and LHON groups, a quantitative analysis of the whole-brain current source density was performed. The signal analysis method was based on scalp EEG data and an inverse solution method called low-resolution brain electromagnetic tomography (eLORETA). In comparison with the healthy subjects, LHON participants showed significantly decreased neuronal activity in the alpha and gamma bands; more specifically, in the alpha band, the decrease was mainly found in the occipital lobes and secondary visual cortex, whereas, in the gamma band, the reduced activity occurred in multiple cortical areas. Additionally, a correlation was found between the alpha band activity of the right secondary visual cortex and the averaged thickness of the right retinal nerve fiber layer in the LHON participants. Our study suggests that LHON is associated with widespread cortical de-activation, rather than simply abnormalities of structures constituting the visual system.

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“…Some previous studies confirmed that the duration of illness in the LHON group was correlated with the macular choroidal thickness [ 45 ]. Nevertheless, DTI studies focused on optic nerve quantitative assessment between chronic LHON patients and normal control subjects showed no correlation between illness duration and DTI parameters and suggest that damage along the visual pathways may occur relatively early from the disease’s clinical onset, or could be already present when the first symptoms are reported by the patient [ 46 ]. Ophthalmologic studies focused on the progressive changes in LHON patients showed structural changes in the ganglion cell layer and inner plexiform layer [ 47 ], as well as RNFL thickening [ 27 ].…”

Section: Discussionmentioning

confidence: 99%

Neuroanatomical Changes in Leber’s Hereditary Optic Neuropathy: Clinical Application of 7T MRI Submillimeter Morphometry

et al. 2020

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Leber’s hereditary optic neuropathy (LHON) is one of the mitochondrial diseases that causes loss of central vision, progressive impairment and subsequent degeneration of retinal ganglion cells (RGCs). In recent years, diffusion tensor imaging (DTI) studies have revealed structural abnormalities in visual white matter tracts, such as the optic tract, and optic radiation. However, it is still unclear if the disease alters only some parts of the white matter architecture or whether the changes also affect other subcortical areas of the brain. This study aimed to improve our understanding of morphometric changes in subcortical brain areas and their associations with the clinical picture in LHON by the application of a submillimeter surface-based analysis approach to the ultra-high-field 7T magnetic resonance imaging data. To meet these goals, fifteen LHON patients and fifteen age-matched healthy subjects were examined. For all individuals, quantitative analysis of the morphometric results was performed. Furthermore, morphometric characteristics which differentiated the groups were correlated with variables covering selected aspects of the LHON clinical picture. Compared to healthy controls (HC), LHON carriers showed significantly lower volume of both palladiums (left p = 0.023; right p = 0.018), the right accumbens area (p = 0.007) and the optic chiasm (p = 0.014). Additionally, LHON patients have significantly higher volume of both lateral ventricles (left p = 0.034; right p = 0.02), both temporal horns of the lateral ventricles (left p = 0.016; right p = 0.034), 3rd ventricle (p = 0.012) and 4th ventricle (p = 0.002). Correlation between volumetric results and clinical data showed that volume of both right and left lateral ventricles significantly and positively correlated with the duration of the illness (left R = 0.841, p = 0.002; right R = 0.755, p = 0.001) and the age of the LHON participants (left R = 0.656, p = 0.007; right R = 0.691, p = 0.004). The abnormalities in volume of the LHON patients’ subcortical structures indicate that the disease can cause changes not only in the white matter areas constituting visual tracts, but also in the other subcortical brain structures. Furthermore, the correlation between those results and the illness duration suggests that the disease might have a neurodegenerative nature; however, to fully confirm this observation, longitudinal studies should be conducted.

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Quantitative assessment of optic nerve in patients with Leber’s hereditary optic neuropathy using reduced field-of-view diffusion tensor imaging

Cited by 18 publications

References 28 publications

Multi-Parametric Diffusion Tensor Imaging of The Optic Nerve for Detection of Dysthyroid Optic Neuropathy in Patients With Thyroid-Associated Ophthalmopathy

Multi-Parametric Diffusion Tensor Imaging of The Optic Nerve for Detection of Dysthyroid Optic Neuropathy in Patients With Thyroid-Associated Ophthalmopathy

Widespread Reductions of Spontaneous Neurophysiological Activity in Leber’s Disease—An Application of EEG Source Current Density Reconstruction

Neuroanatomical Changes in Leber’s Hereditary Optic Neuropathy: Clinical Application of 7T MRI Submillimeter Morphometry

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