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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
Author

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.

Coauthor(s)

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.

Acknowledgements

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.

References
  1. Lenaers G, Hamel CP, Delettre C, Amati-Bonneau P, Procaccio V, Bonneau D, et al. Dominant optic atrophy. Orphanet J Rare Dis. 2012 Jul 9. 7(1):46. [Medline].

  2. Nakaso K, Adachi Y, Fusayasu E, Doi K, Imamura K, Yasui K, et al. Leber's Hereditary Optic Neuropathy with Olivocerebellar Degeneration due to G11778A and T3394C Mutations in the Mitochondrial DNA. J Clin Neurol. 2012 Sep. 8(3):230-4. [Medline]. [Full Text].

  3. Tielsch JM, Javitt JC, Coleman A, et al. The prevalence of blindness and visual impairment among nursing home residents in Baltimore. N Engl J Med. 1995 May 4. 332(18):1205-9. [Medline].

  4. Munoz B, West SK, Rubin GS, et al. Causes of blindness and visual impairment in a population of older Americans: The Salisbury Eye Evaluation Study. Arch Ophthalmol. 2000 Jun. 118(6):819-25. [Medline].

  5. Klopstock T, Yu-Wai-Man P, Dimitriadis K, et al. A randomized placebo-controlled trial of idebenone in Leber's hereditary optic neuropathy. Brain. 2011 Sep. 134:2677-86. [Medline]. [Full Text].

  6. de Lima S, Koriyama Y, Kurimoto T, Oliveira JT, et al. Full-length axon regeneration in the adult mouse optic nerve and partial recovery of simple visual behaviors. Proc Natl Acad Sci U S A. 2012 Jun 5. 109(23):9149-54. [Medline]. [Full Text].

  7. Keirstead SA, Rasminsky M, Fukuda Y, Carter DA, Aguayo AJ, Vidal-Sanz M. Electrophysiologic responses in hamster superior colliculus evoked by regenerating retinal axons. Science. 1989 Oct 13. 246(4927):255-7. [Medline].

  8. Fischer D, Heiduschka P, Thanos S. Lens-injury-stimulated axonal regeneration throughout the optic pathway of adult rats. Exp Neurol. 2001 Dec. 172(2):257-72. [Medline].

  9. Albert DM, Jakobeic FA. Optic atrophy. Principles and Practice of Ophthalmology. 2nd ed. Philadelphia: WB Saunders; 2000. 4108- 4113.

  10. Anderson DR. Ascending and descending optic atrophy produced experimentally in squirrel monkeys. Am J Ophthalmol. 1973 Nov. 76(5):693-711. [Medline].

  11. Glaser JS. Neuro-Ophthalmology. 2nd ed. Philadelphia: JB Lippincott; 1990. 115-117.

  12. Hoyt WF, Schlicke B, Eckelhoff RJ. Fundoscopic appearance of a nerve-fibre-bundle defect. Br J Ophthalmol. 1972 Aug. 56(8):577-83. [Medline].

  13. Kline LB, Bajandas FJ. Neuro-ophthalmology Review Manual. 5th ed. New Jersey: Slack; 2004. 153-164.

  14. Kline LB, ed. Optic nerve disorders. Ophthalmology Monographs. San Francisco, Calif: American Academy of Ophthalmology; 1996. Vol 10:

  15. Kuppersmith MJ, Krohn D. Cupping of the optic disc with compressive lesions of the anterior visual pathway. Ann Ophthalmol. 1984. 16:948-953.

  16. Miller NR, Newman NJ. Walsh & Hoyt's Clinical Neuro-ophthalmology. 6th ed. Philadelphia: JB Lippincott; 208- 218.

  17. Miller NR, Newman SA. Transsynaptic degeneration. Arch Ophthalmol. 1981 Sep. 99(9):1654. [Medline].

  18. Newman NJ. Optic disc pallor: a false localizing sign. Surv Ophthalmol. 1993 Jan-Feb. 37(4):273-82. [Medline].

  19. Patel DA, Hove MW. Focal Points. No 2. San Francisco, Calif: American Academy of Ophthalmology; March 2006. Vol XXIV:

  20. Schwartz B. Cupping and pallor of the optic disc. Arch Ophthalmol. 1973 Apr. 89(4):272-7. [Medline].

  21. Tasman W, Jaeger EA. Topical diagnosis of optic nerve lesions. Duane's Ophthalmology. Philadelphia: JB Lippincott; 2007.

  22. Thompson HS. Pupillary signs in the diagnosis of optic nerve disease. Trans Ophthalmol Soc U K. 1976 Sep. 96(3):377-81. [Medline].

  23. Trobe JD, Glaser JS, Cassady J, et al. Nonglaucomatous excavation of the optic disc. Arch Ophthalmol. 1980 Jun. 98(6):1046-50. [Medline].

  24. Trobe JD, Glaser JS, Cassady JC. Optic atrophy. Differential diagnosis by fundus observation alone. Arch Ophthalmol. 1980 Jun. 98(6):1040-5. [Medline].

 
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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
   



Postneuritis



Ischemic



Arteritic



Ischemic



Nonarteritic



Compressive
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
Neuroimaging



(CT, MRI)



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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