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Introduction
The cause of optic nerve degeneration, unlike the ganglion cells, is mainly due to energy
deficiency. Due to its high firing rate of nerve impulse, it requires ATP production for its
respiratory chain (Howell 1997).
Symptoms:
Patients with LHON usually experience vision loss at age 13-28. Vision loss happened
with both eyes separate by a slight lag time. Vision gradually decreases from 1-36 weeks,
most patient will only limited to counting fingers; others may results in perception to
light only or total blindness. LHON is usually painless but pain may occur during eye
movements. Depending on the type of mutation, multiple-sclerosis like symptoms may
occur (Riordan-Eva et al., 1995).
Diagnosis:
The most reliable diagnosis method is genetic counseling, analysis of mtDNA. Diagnosis
by clinical features is often misleading, especially for patient with no affected relatives
(Riordan-Eva et al., 1995). Some patients may show atypical LHON symptoms which is
misled to other optic atrophy. Assessment of visual fields can be done by kinetic
perimetry and fluorescein angiography to check the microvasculature of the retina.
Electrophysiological methods like electroretinograms and visual evoked potentials can
determine optic nerve function (Man et al., 2002).
Treatment:
Reference List
Calerlli, V, Rugolo, M, Sgarbi, G, Ghelli, A, Zanna, C, Baracca, A, Lenaz, G, Napoli, E,
Martinuzzi, A & Solaini, G 2004, ‘ Bioenergetics shapes cellular death pathways in
Leber’s hereditary optic neuropathy: a model of mitochondrial neurodegeneration’,
Biochimica ct Biophysica Acta, vol. 1658, pp. 172-179, viewed 2 October 2010,
<http://www.sciencedirect.com/>.
Riodan-Eva, P, Sanders, MD, Govan, GG, Sweeney, MG, Da Costa, J & Harding, AE
1995, ‘The clinical features of Leber’s hereditary optic neuropathy defined by the
presence of a pathogenic mitochondria DNA mutation’, Brain, vol. 118, pp. 319-337,
viewed 6 October 2010, < http://0-proquest.umi.com.alpha2.latrobe.edu.au/>.