Optic Atrophy Treatment & Management
- Author: Rashmin Gandhi, MBBS, FRCS(Edin), FRCS(Glasg); Chief Editor: Hampton Roy, Sr, MD more...
No proven treatment exists for optic atrophy. However, treatment that is initiated before the development of optic atrophy can be helpful in saving useful vision. The role of intravenous steroids is proven in a case of optic neuritis or arteritic anterior ischemic optic neuropathy. Early diagnosis and prompt treatment can help patients with compressive and toxic neuropathies.
Idebenone, a quinone analog, has been used recently in a few cases of Leber hereditary optic neuropathy to ameliorate the net ATP synthesis by providing an alternate pathway, as well as by scavenging free radicals, with the advantage of concentrating readily in the mitochondria. Klopstock et al conducted a 24-week multicenter double-blind, randomized, placebo-controlled trial in 85 patients with Leber hereditary optic neuropathy. They did not find a statistically significant visual recovery in the intention-to-treat population. They did find, however, evidence that patients with discordant visual acuities are the most likely to benefit from idebenone treatment, which is safe and well tolerated.
Stem cell treatment can hold a key in the future treatment of neuronal disorders. Neural progenitor cells delivered to the vitreous can integrate into the ganglion cell layer of the retina, turn on neurofilament genes, and migrate into the host optic nerve.
de Lima et al were able to restore some depth perception in mice with severe optic nerve damage. In addition, they found that the mice regained the ability to detect overall movement of the visual field and were able to perceive light. They found that using adequate stimulus, the fibers (1) are able to find their way to the correct visual centers in the brain, (2) are wrapped in the conducting insulation known as myelin, and (3) can make connections (synapses) with other neurons, allowing visual circuits to re-form. At present, the best defense is an early diagnosis because if the cause can be found and corrected, further damage can be prevented. de Lima et al discovered a molecule called oncomodulin. They achieved neuroregeneration in mice by simultaneously targeting the protein oncomodulin, elevating levels of the small signaling molecule cyclic adenosine monophosphate (cAMP) and deleting the gene that encodes the enzyme PTEN.
The optic nerve fiber is made of axons from the retinal ganglion cells, which usually do not regenerate after injury, resulting in lifelong visual loss. In recent studies using hamster models, anterograde tracing and electrophysiologic responses reveal that a small number of axons can regenerate all the way back to the superior colliculus. In other studies, remapping of the retina was noted in the superior colliculus following axon regeneration. These findings have given hope to clinically meaningful regeneration of axons, which may become a reality in the near future.
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|Age||15-50 y||Approximately 70 y||Sixth decade||Varies based on cause|
|Sex||Multiple sclerosis F>M||F>M||F=M||Varies based on cause|
|Visual acuity||Varies from mild blurring (34%) and moderate loss of acuity (12%) to severe or total loss of light perception (complete blindness) in 54% of cases, to no light perception. The loss of vision is acute and progressive.--Vision usually recovers within 2 mo||< 20/200 (6/60)||>20/200 (6/60)||Varies from mild blurring to no light perception|
|Color vision||Color vision > vision loss||Color vision loss = vision loss||Color vision loss = vision loss||Color vision = vision loss|
|Motility||Painful movement in cases of retrobulbar neuritis||Normal||Normal||Depends on the site of compression|
|Nystagmus||In multiple sclerosis, vertical nystagmus (upbeating or downbeating) may be seen||No||No||See-saw nystagmus in optic chiasm compression|
|Optic disc||Temporal pallor||Pallid disc edema||Segmental disc edema||Bow-tie pallor seen in optic chiasm compression; varies in other instances|
|VEP-increased latency <†>||VEP-reduced amplitude||VEP-reduced amplitude||Reduced VEP amplitude|
|In multiple sclerosis, hyperechoic lesions are seen in the brain on MRI||-||-||For exact location of compression|
|Other associations||Headache, scalp tenderness, jaw claudication||
Hypertension and diabetes
|Headache, vomiting, and focal neurologic deficits|
|*RAPD - Relative afferent pupil defect
<†>VEP - Visual-evoked potential