Abstract
Purpose
To determine the site of the initial field defect in patients with Leber hereditary optic neuropathy (LHON).
Methods
We studied nine eyes of nine consecutive LHON patients with the 11778 mitochondrial DNA mutation who had no visual loss (four eyes) or only minimal visual loss (five eyes). When unilateral visual loss was observed, Humphrey field analysis (HFA) (HFA 30–2 program and sometimes the HFA 10–2 program) was immediately and repeatedly performed on the better eye.
Results
For the 12 centralmost points in the visual field, a loss of sensitivity (P < 0.02) was initially found in the upper temporal field of nine eyes and in the lower temporal field of three eyes. These results indicate that it was possible to detect the initial site of sensitivity loss in the centralmost temporal test points in all nine cases. The HFA 10–2 program confirmed the sensitivity loss in the temporal field in two cases.
Conclusions
The centralmost temporal visual field appears to be the most susceptible site in eyes of LHON patients. This suggests that the most susceptible cells during the early stages of LHON are the retinal ganglion cells located in the corresponding region of the retina.
Similar content being viewed by others
References
Kleiner LB, Sherman J. Leber’s hereditary optic neuropathy. In: Gurwood AS, Muchnick BG, editors. The optic nerve in clinical practice. Boston: Butterworth-Heinemann; 1997. p. 77–101.
Newman NJ, Biousse V, Newman SA, et al. Progression of visual field defects in Leber hereditary optic neuropathy: experience of the LHON treatment trial. Am J Ophthalmol 2006;141:1061–1067.
Wakakura M. Optic nerve diseases. In: Wakakura M, Kiyosawa M, Yamada M, Ishigooka J, editors. Solution of indefinite complaints and unknown causes of complaints of the eye and visual system [in Japanese]. Tokyo: Kanehara-Shuppan; 2006. p. 117–120.
Hotta Y, Fujiki K, Hayakawa M, et al. Clinical features of Japanese Leber’s hereditary optic neuropathy with 11778 mutation of mitochondrial DNA. Jpn J Ophthalmol 1995;39:96–108.
Johns DR, Heher KL, Miller NR, Smith KH. Leber’s hereditary optic neuropathy. Clinical manifestations of the 14484 mutation. Arch Ophthalmol 1993;111:495–498.
Newman NJ, Lott MT, Wallace DC. The clinical characteristics of pedigrees of Leber’s hereditary optic neuropathy with the 11778 mutation. Am J Ophthalmol 1991;111:750–762.
Bose S, Dhillon N, Ross-Cisneros FN, Carelli V. Relative postmortem sparing of afferent pupil fibers in a patient with 3460 Leber’s hereditary optic neuropathy. Graefes Arch Clin Exp Ophthalmol 2005;243:1175–1179.
Bremner FD, Shallo-Hoffmann J, Riordan-Eva P, Smith SE. Comparing pupil function with visual function in patients with Leber’s hereditary optic neuropathy. Invest Ophthalmol Vis Sci 1999;40:2528–2534.
Wakakura M, Yokoe J. Evidence of preserved direct papillary light response in Leber’s hereditary optic neuropathy. Brit J Ophthalmol 1995;79:442–446.
Stone EM, Newman NJ, Miller NR, Johns DR, Lott MT, Wallace DC. Visual recovery in patients with Leber’s hereditary optic neuropathy and the 11778 mutation. J Clin Neuroophthalmol 1992;12:10–14.
Mashima Y, Sato EA, Ohde H, Oguchi Y. Macular nerve fibers temporal to fovea may have a great potential to recover function in patients with Leber’s hereditary optic neuropathy. Jpn J Ophthalmol 2002;46:660–667.
Edwards TL, Buttery RG, Mackey DA. Is second eye involvement in Leber’s hereditary optic neuropathy due to retrochiasmal spread of apoptosis? Neuroophthalmology 2007;31:87–98.
Saadati HG, Hsu HY, Heller KB, Sadun AA. A histopathologic and morphometric differentiation of nerves in optic nerve hypoplasia and Leber hereditary optic neuropathy. Arch Ophthalmol 1998;116:911–916.
Barboni P, Savini G, Valentino ML, et al. Retinal nerve fiber layer evaluation by optical coherence tomography in Leber’s hereditary optic neuropathy. Ophthalmology 2005;112:120–126.
Sadun AA, Win PH, Ross-Cisneros FN, Walker SO, Carelli V. Leber’s hereditary optic neuropathy differentially affects smaller axons in the optic nerve. Trans Am Ophthalmol Soc 2000;98:223–235.
Quiros PA, Torres RJ, Salomao S, et al. Colour vision defects in asymptomatic carriers of the Leber’s hereditary optic neuropathy (LHON) mtDNA 11778 mutation from a large Brazilian LHON pedigree: a case-control study. Br J Ophthalmol 2006;90:150–153.
Savini G, Barboni P, Valentino ML, et al. Retinal nerve fiber layer evaluation by optical coherence tomography in unaffected carriers with Leber’s hereditary optic neuropathy mutations. Ophthalmology 2005;112:127–131.
Author information
Authors and Affiliations
Corresponding author
About this article
Cite this article
Wakakura, M., Fujie, W. & Emoto, Y. Initial temporal field defect in Leber hereditary optic neuropathy. Jpn J Ophthalmol 53, 603–607 (2009). https://doi.org/10.1007/s10384-009-0743-y
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s10384-009-0743-y