Dissociation of damage to spatial and luminance channels in early Leber's hereditary optic neuropathy manifested by the visual evoked potential

Mashima, Yukihiko; Imamura, Yutaka; Oguchi, Yoshihisa

doi:10.1038/eye.1997.181

Cited by 8 publications

(7 citation statements)

References 22 publications

(2 reference statements)

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“…Flash VEPs in that patient were normal. Although it has been suggested that luminance-related fibres may be less affected than contrast-related fibres in patients with early LHON [11], flash VEPs are generally less sensitive to disease than pattern VEPs. Further detailed electrophysiological investigation of patients exhibiting visual improvement may provide additional insight into the mechanisms underlying the visual loss and delayed recovery, currently speculative.…”

Section: Discussionmentioning

confidence: 95%

Clinical and electrophysiological recovery in Leber hereditary optic neuropathy with G3460A mutation

et al. 2012

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To report a case of clinical and electrophysiological recovery in Leber hereditary optic neuropathy (LHON) with G3460A Mutation. A 10-year-old boy with a three-month history of painless bilateral sequential visual loss upon presentation underwent visual acuity (diminished), anterior and posterior segment examination (normal), fluorescein angiography (normal), Goldman kinetic perimetry (bilateral central scotomata), genetic (a point G3460A mutation) and electrophysiological investigation (undetectable pattern visual evoked potentials (VEP); low amplitude, broadened and reduced flash VEPs and loss of the N95 component in the pattern electroretinograms). Diagnosis of LHON was made. Eighteen months later vision and electrophysiological tests results began spontaneously improving. Kinetic perimetry revealed reduced density and size of scotomata. Two years later, there had been further electrophysiological improvement. This report describes both clinical and electrophysiological improvement in LHON with G3460A mutation.

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Section: Discussionmentioning

confidence: 95%

Clinical and electrophysiological recovery in Leber hereditary optic neuropathy with G3460A mutation

et al. 2012

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“…3,16 An absence of waveforms in PRVEP was found in all nine patients of our study, which is more severe than the findings of Mashima and colleagues. 23 They reported that the amplitudes were reduced and the latencies prolonged in PRVEP in the early stage of LHON. This discrepancy might be due to the relatively late stage and worse visual acuity of our patients.…”

Section: Discussionmentioning

confidence: 99%

Clinical phenotype and the G11778A mutation of mitochondrial DNA in patients with Leber’s hereditary optic neuropathy in Taiwan

Chu¹,

Huang²,

Kao

et al. 2001

Neuro-Ophthalmology

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We report the clinical manifestations and mitochondrial DNA (mtDNA) mutations of nine Chinese patients with Leber's hereditary optic neuropathy from seven families in Taiwan. The nine probands were all males, aged 19 to 43 years at the time of the study and with ages of onset ranging between 13 and 30 years. They were characterized by an acute or subacute onset of visual loss in one eye, which progressed to the other eye within a few weeks. Their visual outcome was poor, although improvement was noted in four patients. A muscle biopsy from one patient showed a decreased activity of cytochrome c oxidase and subsarcolemmal mitochondrial aggregation. All patients and unaffected maternal family members had a homoplasmic G11778A mtDNA mutation regardless of variations in clinical manifestation. High frequencies of environmental factors such as smoking, alcohol consumption, trauma, and/or military training were found in these patients. We conclude that even though a homoplasmic G11778A mtDNA mutation was found in all patients and their maternal family members, this mutation could not account for the presentation of the clinical features. Additional genetic, epigenetic, and/or environmental factors may be important trigger factors in the clinical expression. LHON in Taiwan 207 Original paperNeuroophthalmology Downloaded from informahealthcare.com by The University of Manchester on 11/03/14For personal use only.Neuroophthalmology Downloaded from informahealthcare.com by The University of Manchester on 11/03/14For personal use only.

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“…In a further study on 5 patients with LHON, VEPs showed reduced mean amplitude and increased mean latency in all of them. Applying flash VEPs, decreased mean amplitude, but normal mean latency were found (7). In 7 patients with multiple systemic lipomatosis, regarded to be a RCD, VEPs were abnormal in 4, the amplitude was decreased in 4 and the latency was prolonged in 2 (12).…”

Section: Discussionmentioning

confidence: 99%

“…Visual function anomalies can be confirmed clinically, by flourescein angiography (shows retinal morphological anomalies), reduced amplitudes on retinography and abnormal visual evoked potentials (VEPs). Though VEPs are frequently abnormal in adult patients with RCD, no reliable data are available about the frequency of abnormal VEPs in a bigger group of patients (2–9). This study was thus carried out to investigate 1) how frequent VEPs are abnormal in patients with RCD, 2) how often VEPs are abnormal despite normal visual acuity and 3) which of the VEP variables are most often abnormal.…”

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confidence: 99%

Visually evoked potentials in respiratory chain disorders

Finsterer

2001

Acta Neurologica Scandinavica

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Dissociation of damage to spatial and luminance channels in early Leber's hereditary optic neuropathy manifested by the visual evoked potential

Abstract: Luminance-related fibres may be less affected than are the spatial-vision-related fibres in patients with early LHON.

Cited by 8 publications

References 22 publications

Clinical and electrophysiological recovery in Leber hereditary optic neuropathy with G3460A mutation

Clinical and electrophysiological recovery in Leber hereditary optic neuropathy with G3460A mutation

Clinical phenotype and the G11778A mutation of mitochondrial DNA in patients with Leber’s hereditary optic neuropathy in Taiwan

Visually evoked potentials in respiratory chain disorders

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