Evaluation of Fixation Pattern and Reading Ability in Patients With Leber Hereditary Optic Neuropathy

Altpeter, Elke; Blanke, Björn R.; Leo-Kottler, Beate; Nguyen, X. N.; Trauzettel‐Klosinski, Susanne

doi:10.1097/wno.0b013e31829d1f5b

Cited by 14 publications

(8 citation statements)

References 21 publications

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“…It has also been reported that the scotoma or its fenestration in some LHON patients is relieved (Nakamura and Yamamoto 2000 ). A component of the visual improvement in LHON may be the adaptive phenomenon of eccentric fixation (Altpeter et al 2013 ).…”

Section: Discussionmentioning

confidence: 99%

A retrospective analysis of characteristics of visual field damage in patients with Leber’s hereditary optic neuropathy

Ran

Yang

et al. 2016

SpringerPlus

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The objective of this study is to investigate the characteristics and the evolution of visual field damage caused by Leber’s hereditary optic neuropathy (LHON) and to provide clinical data for the diagnosis of LHON. Parameters of visual field in 32 consecutive patients (49 eyes) with LHON who were confirmed by genetic diagnostic tests were retrospectively measured within 1 week, between three to six months, and at six months after onset. Visual field defects revealed central scotoma in 26 eyes (53.1 %), paracentral scotoma in 12 eyes (24.5 %), ceco-central defects in 6 eyes (12.2 %), blind spot enlargenment in 3 eyes (6.1 %), quadrantanopia in 2 eyes (4.1 %) within 1 week after onset. After 3 to 6 months, ceco-central defects were detected in 22 eyes (44.9 %), central isopter constriction in 10 eyes (20.4 %), hemianopia or quadrantanopia in 5 eyes (10.2 %), central scotoma in 4 eyes (8.2 %), and paracentral scotoma in 1 eye (2.0 %). After 6 months, central isopter constriction was observed in 18 eyes (36.7 %), diffuse defects in 21 eyes (42.9 %), ceco-central defects in 3 eyes (6.1 %), hemianopia or quadrantanopia in 5 eyes (10.2 %), and central scotoma in 2 eyes (4.1 %). LHON at different stages was characterized by different focal visual field defects: visual field defects in LHON patients within 1 week after onset were mostly central or paracentral scotoma, which was enlarged around the ceco-central defect, or connected to form a blind spot after 3–6 months. Diffuse and central isopter constriction defects were usually developed after 6 months. Damages firstly appeared in papillomacular bundle and gradually expanded outward. These characteristics of visual field defects reported in this study might provide a clinical basis for better diagnosis of LHON.

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Section: Discussionmentioning

confidence: 99%

A retrospective analysis of characteristics of visual field damage in patients with Leber’s hereditary optic neuropathy

Ran

Yang

et al. 2016

SpringerPlus

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“…Blind spot enlargement Considered ubiquitous in papilledema 7 ; treatment not necessary unless encroaching upon fixation 77,78 Central/ring scotoma Optical assistance with magnification or size scaling 79,80 Microperimetry-assisted identification of preferential retinal locus [81][82][83] Biofeedback and training 84 Cecocentral scotoma Eccentric viewing training 85 Visual restitution therapy 60 Functional training 86 Hemianopia Optical modalities such as monocular or binocular prisms 55,66,67 Saccadic compensation training 87 Restorative training 71…”

Section: Pattern Of Field Defect Treatment Strategiesmentioning

confidence: 99%

Prognosis and Treatment of Visual Field Defects

Agarwal

Kedar

2015

Semin Neurol

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Visual field deficits are common in neurologic disease conditions such as cerebrovascular disease, traumatic brain injury, and brain tumors. Loss of visual fields may lead to impairment of reading skills (hemianopic dyslexia) and limitations of daily activities such as driving, which can have a significant impact on an individual's socioeconomic status and quality of life. Moreover, patients with motor deficits from neurologic diseases have a 20% decreased likelihood of achieving independence in ambulation and self-care activities with coexisting hemianopia. Studies on the natural history of homonymous hemianopia have shown that spontaneous improvement of visual fields may occur in less than 40% of individuals early in the disease process. Improvement is usually incomplete, which implies that a significant number of individuals will be left with a disabling visual deficit. Although several methods of rehabilitation (optical, compensatory, and restitution therapy) are used in practice, none, unfortunately, have shown consistent and significant benefits. In this review, the authors focus on the natural history, impact, prognosis, and treatment modalities for neurologic field defects.

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“…However, central scotoma in LHON lead to difficulties with reading, thus visual rehabilitation seems to be very important for these patients to continue their education and their ability to work [33]. The visual improvement in LHON patients has also been observed during follow-up and it is possibly the adaptive phenomenon of eccentric fixation [34]. Eccentric viewing (EV), a representative method of visual function training, consists of using any non-foveal point on the retina for viewing [35].…”

Section: Discussionmentioning

confidence: 99%

Visual rehabilitation training in a patient with large central scotoma due to Leber’s hereditary optic neuropathy

Kiszka

Nowakowska²,

Nowomiejska

et al. 2018

Ophthalmol J

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BaCKground: Leber's hereditary optic neuropathy (LHON) is a disease associated with pathogenetic mutations of mitochondrial DNA which affects predominantly young adult males and leads to loss of central vision, low visual acuity and unstable fixation. The purpose of this study was to allow the patient to establish the best possible fixation in the best area of retinal sensitivity in LHON patient with central scotoma. Methods: A 17 years old patient with confirmed mitochondrial LHON 11778G > A mutation was included in the study. The patient underwent 16 visual rehabilitation sessions-two for each of 8 weeks using the training module available in the equipment-MAIA microperimeter. Visual acuity and standrad microperimetry examination were performed before and 8 weeks period of training. To measure the fixation stability with MAIA microperimeter, P1, P2 and Bivariate Contour Ellipse Area (BCEA) parameters were used. results: Visual acuity of the trained better eye was 0.08 at the baseline versus 0.063 after 8 weeks training. Fixation stability parameter was P1-21%, P2-64% at the first session versus P1-60%, P2-90% at the last session. BCEA values 29.2°² before and 14.2°² after training, respectively. ConClusions: Visual training via microperimetry could potentially be a method that improves the fixation stability in patients with LHON.

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Evaluation of Fixation Pattern and Reading Ability in Patients With Leber Hereditary Optic Neuropathy

Cited by 14 publications

References 21 publications

A retrospective analysis of characteristics of visual field damage in patients with Leber’s hereditary optic neuropathy

A retrospective analysis of characteristics of visual field damage in patients with Leber’s hereditary optic neuropathy

Prognosis and Treatment of Visual Field Defects

Visual rehabilitation training in a patient with large central scotoma due to Leber’s hereditary optic neuropathy

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