Characterization of macular thickness changes in Leber’s hereditary optic neuropathy by optical coherence tomography

Zhang, Yixin; Huang, Houbin; Wei, Shimin; Gong, Yan; Li, Hongyang; Dai, Yanwei; Zhao, Shilei; Wang, Yingyun; Yan, Hongxin

doi:10.1186/1471-2415-14-105

Cited by 28 publications

(23 citation statements)

References 12 publications

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“…Central field defects and blind spot enlargement were common findings, in keeping with previous investigations [18]. e current study showed that that the thickness of the RNFL around the optic disc and surrounding the macula did not reduce in the acute stage but reduced significantly in the chronic stage of the disease, corroborating previous reports [19,20]. e pattern ERG N95 and approximately 70% of P50 originate in the retinal ganglion cells.…”

Section: Discussionsupporting

confidence: 92%

Electrophysiological and Structural Changes in Chinese Patients with LHON

Wang

Guo

et al. 2020

Journal of Ophthalmology

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Objective. To review retrospectively the electrophysiological and structural changes in 13 Chinese patients with Leber hereditary optic neuropathy (LHON). Methods. 26 eyes of 13 patients with a genetically confirmed diagnosis of LHON were categorized into two groups according to the duration of the disease: group 1 (duration less than 3 months) and group 2 (duration between 3 months and 18 years). Clinical history, comprehensive visual electrophysiology, optical coherence tomography (OCT), and color fundus photography were performed. Results. Fundoscopy showed optic disc hyperemia in group 1 and optic atrophy in group 2. OCT measures of retinal nerve fiber layer (RNFL) thickness around the optic disc and surrounding macula were normal in group 1 but reduced in group 2 (10 of 10 eyes). The thickness of the retinal ganglion cell layer (GCL) plus inner plexiform layer (IPL) surrounding the macula reduced significantly in group 1 and group 2 compared with a healthy control group. Pattern ERG (PERG) P50 amplitude was normal, but the N95/P50 ratio reduced in most of group 1 (4 of 5 eyes) and in all of group 2 (11 eyes). PERG P50 peak time was abnormally short in group 2. Multifocal electroretinography (mfERG) showed subnormal responses associated with ring 1 (the central area) and ring 2 in group 1 and reductions in rings 1, 2, and 3 in group 2. Conclusion. The study highlights differences in retinal structure and function between the acute and chronic stages of LHON in a group of Chinese patients. There is PERG evidence of retinal ganglion cell dysfunction and OCT evidence of GCL + IPL thinning in both groups, but there is additional peripapillary RNFL loss in the chronic stage, associated with more severe RGC dysfunction. There is multifocal ERG evidence of localized macular dysfunction in both acute and chronic groups. The study highlights the importance of comprehensive electrophysiological and structural assessments of the retina in LHON and is pertinent to studies that aim to monitor disease progression or the effects of future therapeutic interventions.

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Section: Discussionsupporting

confidence: 92%

Electrophysiological and Structural Changes in Chinese Patients with LHON

Wang

Guo

et al. 2020

Journal of Ophthalmology

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“…This result confirms a recent study23 evaluating total macular thickness in patients with LHON, stratified in five subgroups based on disease duration. The authors separately analysed the inner and the outer ring in nine different sectors with methodology corresponding to the Early Treatment Diabetic Retinopathy Study.…”

Section: Discussionsupporting

confidence: 91%

Macular nerve fibre and ganglion cell layer changes in acute Leber's hereditary optic neuropathy

Balducci¹,

Savini

Cascavilla

et al. 2015

Br J Ophthalmol

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AimsTo evaluate longitudinal retinal ganglion cell inner plexiform layer (GC-IPL) and macular retinal nerve fibre layer (mRNFL) thickness changes in acute Leber's hereditary optic neuropathy (LHON).MethodsSix eyes of four patients with LHON underwent SD-OCT (optical coherence tomography) at month 1, 3, 6 and 12 after visual loss. In two eyes, the examination was carried out in the presymptomatic stage. The relationship and curves for area under the receiver operator characteristic (AUROC) were generated to assess the ability of each parameter to detect ganglion cell loss.ResultsSignificant longitudinal thinning of GC-IPL and mRNFL was detected in LHON. GC-IPL thinning was detectable in the deviation map during the presymptomatic stage in the inner ring of the nasal sector and then it progressively extended following a centrifugal and spiral pattern. Similarly, mRNFL thinning began in the inferonasal sector and it progressively extended. No further statistically significant changes were detected after month 3. The highest level of AUROC values at 1 month were detected in the nasal sectors and inferonasal mRNFL thickness reached AUROC value=1. All the parameters were equally able to detect ganglion cell loss from month 2 to 12.ConclusionsThe natural history of GC-IPL thinning follows a specific pattern of reduction, reflecting the anatomical course of papillomacular fibres. Month 6 represents the end of GC-IPL loss. GC-IPL and mRNFL thinning is detectable before onset of visual loss. These observations can help future therapeutic approaches for both LHON carriers at high risk of conversion and patients with acute early LHON.

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“…As it has been previously reported, the temporal fibres of the peripapillary retinal nerve fibre layer were the most severely affected in our patient, with relative sparing of the nasal ones . Recent publications have reported changes in the macular ganglion cell layer in patients with LHON during the initial days of visual loss, and have suggested these changes may precede the conversion from carrier to disease status …”

Section: Discussionsupporting

confidence: 81%