Optic Atrophy Treatment & Management

  • Author: Rashmin Gandhi, MBBS, FRCS(Edin), FRCS(Glasg); Chief Editor: Hampton Roy, Sr, MD  more...
Updated: Sep 12, 2014

Medical Care

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.[5]

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.[6]

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.[7] In other studies, remapping of the retina was noted in the superior colliculus following axon regeneration.[8] These findings have given hope to clinically meaningful regeneration of axons, which may become a reality in the near future.

Contributor Information and Disclosures

Rashmin Gandhi, MBBS, FRCS(Edin), FRCS(Glasg) Faculty, Departments of Ocular Physiology and Neuro-ophthalmology, Elite School of Optometry; Faculty, CU Shah Ophthalmic Postgraduate Center, Consulting Staff, Department of Ophthalmology, Sankara Nethralaya Hospital

Rashmin Gandhi, MBBS, FRCS(Edin), FRCS(Glasg) is a member of the following medical societies: American Academy of Ophthalmology, All India Ophthalmological Society

Disclosure: Nothing to disclose.


Gangaprasad Muthaiah Amula, MBBS, DNB, FRCS(Glasg) FICO, FMRF, Consultant, L V Prasad Eye Institute, India

Gangaprasad Muthaiah Amula, MBBS, DNB, FRCS(Glasg) is a member of the following medical societies: All India Ophthalmological Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Simon K Law, MD, PharmD Clinical Professor of Health Sciences, Department of Ophthalmology, Jules Stein Eye Institute, University of California, Los Angeles, David Geffen School of Medicine

Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, Association for Research in Vision and Ophthalmology, American Glaucoma Society

Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy, Sr, MD Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Hampton Roy, Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

Additional Contributors

Edsel Ing, MD, FRCSC Associate Professor, Department of Ophthalmology and Vision Sciences, University of Toronto Faculty of Medicine; Consulting Staff, Hospital for Sick Children and Sunnybrook Hospital

Edsel Ing, MD, FRCSC is a member of the following medical societies: American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, American Society of Ophthalmic Plastic and Reconstructive Surgery, Royal College of Physicians and Surgeons of Canada, Canadian Ophthalmological Society, North American Neuro-Ophthalmology Society, Canadian Society of Oculoplastic Surgery, European Society of Ophthalmic Plastic and Reconstructive Surgery, Canadian Medical Association, Ontario Medical Association, Statistical Society of Canada, Chinese Canadian Medical Society

Disclosure: Nothing to disclose.


Brian R Younge, MD Professor of Ophthalmology, Mayo Clinic School of Medicine

Brian R Younge, MD is a member of the following medical societies: American Medical Association, American Ophthalmological Society, and North American Neuro-Ophthalmology Society

Disclosure: Nothing to disclose.

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Normal optic nerve histopathology.
Glaucomatous optic atrophy histopathology.
Healthy optic disc.
Nonarteritic anterior ischemic optic neuropathy.
Arteritic anterior ischemic optic neuropathy, cilioretinal artery occlusion.
Primary optic atrophy.
Optic atrophy following papilledema (secondary).
Glaucomatous optic atrophy.
Juvenile open-angle glaucoma (JOAG) with optic pallor.
Consecutive optic atrophy following panretinal photocoagulation (PRP).
Table. Various Common Groups of Disorders Presenting with Optic Atrophy






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
RAPD* + + + +
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

Electrophysiologic study

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

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