Optic Atrophy Treatment & Management

Updated: Apr 01, 2019
  • Author: Rashmin Gandhi, MBBS, FRCS(Edin), FRCS(Glasg); Chief Editor: Edsel Ing, MD, MPH, FRCSC  more...
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Medical Care

No proven treatment reverses 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 and is the only clinically proven drug in the treatment of Leber hereditary optic neuropathy. The drug molecule bypasses the defective mitochondrial complex I, leading to improved energy supply and a functional recovery of retinal ganglion cells during the acute stage of the disease, thereby preventing further vision loss and promoting vision recovery. [5] So far, the results were noted to be modest and the treatment is quite expensive. 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. [6]

Research in stem cell treatment can hold a key in the future treatment of neuronal disorders. Currently, there are no approved treatments for mitochondrial disease, including optic neuropathies caused by primary or secondary mitochondrial dysfunction. [7]

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. [8]

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. [9] In other studies, remapping of the retina was noted in the superior colliculus following axon regeneration. [10] These findings have given hope to clinically meaningful regeneration of axons, which may become a reality in the near future.

At present, the best defense is early diagnosis, because, if the cause can be found and corrected, further damage can be prevented.


Further Outpatient Care

Low-vision aids for patients with some useful vision should be considered for occupational rehabilitation.