open access

Vol 3, No 1 (2018)
CASE REPORTS
Published online: 2018-07-20
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Visual rehabilitation training in a patient with large central scotoma due to Leber’s hereditary optic neuropathy

Agnieszka Kiszka, Dominika Nowakowska, Katarzyna Nowomiejska, Katarzyna Tońska, Robert Rejdak
DOI: 10.5603/OJ.2018.0029
·
Ophthalmology J 2018;3(1):21-27.

open access

Vol 3, No 1 (2018)
CASE REPORTS
Published online: 2018-07-20

Abstract

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°˛ before and 14.2°˛ after training, respectively.

Conclusions. Visual training via microperimetry could potentially be a method that improves the fixation stability in patients with LHON.  

Abstract

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°˛ before and 14.2°˛ 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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Keywords

Leber’s hereditary optic neuropathy, eccentric viewing, eye-fixation training, microperimetry

About this article
Title

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

Journal

Ophthalmology Journal

Issue

Vol 3, No 1 (2018)

Pages

21-27

Published online

2018-07-20

DOI

10.5603/OJ.2018.0029

Bibliographic record

Ophthalmology J 2018;3(1):21-27.

Keywords

Leber’s hereditary optic neuropathy
eccentric viewing
eye-fixation training
microperimetry

Authors

Agnieszka Kiszka
Dominika Nowakowska
Katarzyna Nowomiejska
Katarzyna Tońska
Robert Rejdak

References (43)
  1. Sunness JS, Schuchard RA, Shen N, et al. Landmark-driven fundus perimetry using the scanning laser ophthalmoscope. Invest Ophthalmol Vis Sci. 1995; 36(9): 1863–1874.
  2. Hwang JC, Tari SR, Seiple W, et al. Reproducibility of Eye Movement Compensated Fundus Perimetry. Investigative Ophthalmology & Visual Science. 2005; 46: 1561.
  3. Springer C, Bültmann S, Völcker HE, et al. Fundus perimetry with the Micro Perimeter 1 in normal individuals: comparison with conventional threshold perimetry. Ophthalmology. 2005; 112(5): 848–854.
  4. Mendoza-Santiesteban CE, Lopez-Felipe D, Fernández-Cherkasova L, et al. Microperimetry in the study of neuro-ophthalmic diseases. Semin Ophthalmol. 2010; 25(4): 136–143.
  5. Morales MU, Saker S, Amoaku WM. Bilateral eccentric vision training on pseudovitelliform dystrophy with microperimetry biofeedback. BMJ Case Rep. 2015; 2015.
  6. Giacomelli G, Virgili G, Giansanti F, et al. Clinical and microperimetric predictors of reading speed in low vision patients: a structural equation modeling approach. Invest Ophthalmol Vis Sci. 2013; 54(6): 4403–4408.
  7. Ueda-Consolvo T, Otsuka M, Hayashi Y, et al. Microperimetric Biofeedback Training Improved Visual Acuity after Successful Macular Hole Surgery. J Ophthalmol. 2015; 2015: 572942.
  8. Ramírez Estudillo JA, León Higuera MI, Rojas Juárez S, et al. Visual rehabilitation via microperimetry in patients with geographic atrophy: a pilot study. Int J Retina Vitreous. 2017; 3: 21.
  9. Tarita-Nistor L, González EG, Markowitz SN, et al. Plasticity of fixation in patients with central vision loss. Vis Neurosci. 2009; 26(5-6): 487–494.
  10. Vingolo EM, Salvatore S, Cavarretta S. Low-vision rehabilitation by means of MP-1 biofeedback examination in patients with different macular diseases: a pilot study. Appl Psychophysiol Biofeedback. 2009; 34(2): 127–133.
  11. Pacella E, Pacella F, Mazzeo F, et al. Effectiveness of vision rehabilitation treatment through MP-1 microperimeter in patients with visual loss due to macular disease. Clin Ter. 2012; 163(6): e423–e428.
  12. Yohannan J, Bittencourt M, Sepah YJ, et al. Association of retinal sensitivity to integrity of photoreceptor inner/outer segment junction in patients with diabetic macular edema. Ophthalmology. 2013; 120(6): 1254–1261.
  13. Hatef E, Hanout M, Moradi A, et al. Longitudinal comparison of visual acuity as measured by the ETDRS chart and by the potential acuity meter in eyes with macular edema, and its relationship with retinal thickness and sensitivity. Eye (Lond). 2014; 28(10): 1239–1245.
  14. Hatef E, Colantuoni E, Wang J, et al. The relationship between macular sensitivity and retinal thickness in eyes with diabetic macular edema. Am J Ophthalmol. 2011; 152(3): 400–405.e2.
  15. Okada K, Yamamoto S, Mizunoya S, et al. Correlation of retinal sensitivity measured with fundus-related microperimetry to visual acuity and retinal thickness in eyes with diabetic macular edema. Eye (Lond). 2006; 20(7): 805–809.
  16. Ozdemir H, Karacorlu SA, Senturk F, et al. Assessment of macular function by microperimetry in unilateral resolved central serous chorioretinopathy. Eye (Lond). 2008; 22(2): 204–208.
  17. Roisman L, Ribeiro JC, Fechine FV, et al. Does microperimetry have a prognostic value in central serous chorioretinopathy? Retina. 2014; 34(4): 713–718.
  18. Sepah YJ, Hatef E, Colantuoni E, et al. Macular sensitivity and fixation patterns in normal eyes and eyes with uveitis with and without macular edema. J Ophthalmic Inflamm Infect. 2012; 2(2): 65–73.
  19. Verdina T, Giacomelli G, Sodi A, et al. Biofeedback rehabilitation of eccentric fixation in patients with Stargardt disease. Eur J Ophthalmol. 2013; 23(5): 723–731.
  20. Meleth AD, Mettu P, Agrón E, et al. Changes in retinal sensitivity in geographic atrophy progression as measured by microperimetry. Invest Ophthalmol Vis Sci. 2011; 52(2): 1119–1126.
  21. National Operative Group. Epidemic Neuropathy in Cuba. Havana: Ministry of Public Health, 1993; 27: 7–30.
  22. Santiesteban R, Márquez M. Neuro-ophthalmological and neurophysiological characteristics of the epidemic neuropathy. Epidemic Neuropathy in Cuba 1992-1994. Havana: Editorial Medical Sciences. ; 1995: 35–45.
  23. Hirano M, Cleary JM, Stewart AM, et al. 'Outbreak' of optic and peripheral neuropathy in Cuba? JAMA. 1993; 270(4): 511–518.
  24. Torroni TO, Brown MD, Lott MT, et al. Cuba neuropathy field investigation. African, Native American, and European mitochondrial DNA in Cubans from Pinegrove of the River province and implications for the recent epidemic neuropathy in Cuba. Hum Mutat. 1995; 5: 310–317.
  25. Hedges TR, Hirano M, Tucker K, et al. Epidemic optic and peripheral neuropathy in Cuba: a unique geopolitical public health problem. Surv Ophthalmol. 1997; 41(4): 341–353.
  26. Barnouin J, Verdura Barrios T, Chassagne M, et al. Nutritional and food protection against epidemic emerging neuropathy. Epidemiological findings in the unique disease-free urban area of Cuba. Int J Vitam Nutr Res. 2001; 71(5): 274–285.
  27. American Academy of Ophthalmology. Neuro Ophthalmology. Section 5. The sensory visual system. Basic and Clinical Science course 03-2004.
  28. American Academy of Ophthalmology. Optic Nerve Disorders. Ophthalmology monographs 10. Basic and Clinical Science course 03-2004:139–158.
  29. Yu-Wai-Man P, Griffiths PG, Brown DT, et al. The epidemiology of Leber hereditary optic neuropathy in the North East of England. Am J Hum Genet. 2003; 72(2): 333–339.
  30. Santiesteban R, et al. Rodríguez M Mendoza C M Clinical manifestations and molecular identification of patient with hereditary optic neuropatía of Leber in center of national reference for the neuroftalmología in Cuba. Magazine of Neurol. 1999; 29(5): 408–415.
  31. Pérez Moreira JV. Pathology would orbit, exploration diagnosis and surgery. TomoII España. 2002; 41: 949–988.
  32. Poser CHM. An Atlas of Multiple Sclerosis. Boston: Department of Neurology, Harvard Medical School. 1998; 38: 16–19.
  33. Leo-Kottler B, Christ-Adler M. [Leber optic neuropathy in women and children]. Ophthalmologe. 1999; 96(11): 698–701.
  34. Altpeter EK, Blanke BR, Leo-Kottler B, et al. Evaluation of fixation pattern and reading ability in patients with Leber hereditary optic neuropathy. J Neuroophthalmol. 2013; 33(4): 344–348.
  35. VON NOORDEN GK, MACKENSEN G. Phenomenology of eccentric fixation. Am J Ophthalmol. 1962; 53: 642–660.
  36. Holocomb JG, Goodrich GL. Eccentric viewing training. J Am Optom Assoc. 1976; 47(11): 1438–1443.
  37. Timberlake GT, Mainster MA, Peli E, et al. Reading with a macular scotoma. I. Retinal location of scotoma and fixation area. Invest Ophthalmol Vis Sci. 1986; 27(7): 1137–1147.
  38. Timberlake GT, Peli E, Essock EA, et al. Reading with a macular scotoma. II. Retinal locus for scanning text. Invest Ophthalmol Vis Sci. 1987; 28(8): 1268–1274.
  39. Whittaker SG, Budd J, Cummings RW. Eccentric fixation with macular scotoma. Invest Ophthalmol Vis Sci. 1988; 29(2): 268–278.
  40. Fletcher DC, Schuchard RA. Preferred retinal loci relationship to macular scotomas in a low-vision population. Ophthalmology. 1997; 104(4): 632–638.
  41. Sunness JS, Applegate CA, Haselwood D, et al. Fixation patterns and reading rates in eyes with central scotomas from advanced atrophic age-related macular degeneration and Stargardt disease. Ophthalmology. 1996; 103(9): 1458–1466.
  42. Crossland MD, Culham LE, Kabanarou SA, et al. Fixation stability and reading speed in patients with newly developed macular disease. Ophthalmic Physiol Opt. 2004; 24(4): 327–333.
  43. Limol P, Vingolo EM, et al. D’Amato LM, Microperimetry and Fixation in the Low Vision Patient. Investigative Ophthalmology & Visual Science. 2005; 46: 4330.

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