eMedicine Specialties > Ophthalmology > Optic Nerve

Optic Neuropathy, Anterior Ischemic: Differential Diagnoses & Workup

Author: Brian R Younge, MD, Professor of Ophthalmology, Mayo Clinic School of Medicine
Contributor Information and Disclosures

Updated: Nov 26, 2007

Differential Diagnoses

Central Retinal Vein Occlusion
Ocular Hypotony
Papilledema
Pseudopapilledema

Other Problems to Be Considered

Diabetic papillitis

Workup

Laboratory Studies

  • The most important test in any patient with AION is the erythrocyte sedimentation rate (ESR). In patients with arteritic AION, the ESR is usually elevated, although 10% of patients may have a normal ESR. In NAION, the ESR is more likely to be normal, assuming no comorbid condition is present. The Westergren ESR is thought to be more reliable than the Wintrobe ESR.
  • A hematology group is useful. Mild anemia may be present.
  • Other blood tests, such as the C-reactive protein (CRP), have been found useful in diagnosing giant cell arteritis.

Imaging Studies

  • Ultrasound of the temporal arteries and ocular Doppler ultrasound have been described, but their use in mainstream diagnosis of arteritic AION versus NAION remains to be seen.
  • Ocular plethysmography (OPG) may be advocated. OPG is thought to be abnormal in patients with arteritic AION.
  • MRI is useful in younger individuals who may have demyelinating disease. It is not useful in older age groups, in either the arteritic or nonarteritic form of AION.
  • Computed tomography is not useful in either the arteritic or nonarteritic form of AION.
  • Fluorescein angiography has been suggested as a possible method of distinguishing arteritic AION from NAION. With arteritic AION, a markedly prolonged choroidal filling time is usually present.
  • Angiography of the cerebral circulation has been useful in giant cell arteritis, showing segmental stenosis or even occlusion of the extracranial vessels. However, this invasive study has fallen into less frequent use.

Procedures

  • Temporal artery biopsy is used to diagnose giant cell arteritis. It is especially useful in patients with any of the symptoms of giant cell arteritis or in patients with visual loss and a high ESR. A normal result of the temporal artery biopsy is often used to exclude the diagnosis of giant cell arteritis in older patients with AION.
  • Whenever possible, a biopsy specimen of at least 2-3 centimeters should be obtained to minimize the possibility of skip lesions. Bilateral temporal artery biopsy should be considered if giant cell arteritis is still suspected despite an initial negative result of the temporal artery biopsy. Delaying the second side a few weeks may improve the yield of a positive biopsy result on that second side.
  • Biopsy should generally be performed within 4 weeks of initiation of steroid treatment, although positive biopsy results can be obtained months after steroids have begun.

Histologic Findings

The idiopathic form of ischemic optic neuropathy has no characteristic pathology other than obliterative occlusion of the cilioretinal arteries and ischemic necrosis of the optic nerve head in variable degree.

Giant cell arteritis has a characteristic inflammatory infiltrate that has a granulomatous appearance, sometimes with giant cells. Complete occlusion of the ophthalmic artery within the orbit may result in ischemic changes of the globe in its entirety. The use of frozen section for temporal artery biopsy is very useful in determining arteritis, and it may establish the diagnosis with a single temporal artery biopsy. Rarely, if the initial temporal artery biopsy result is negative, the contralateral biopsy result may be positive due to minimal involvement or skip areas. Inflammatory infiltrate in the adventitia often is considered to be sufficient evidence for diagnosis, even with the elastica intact.

More on Optic Neuropathy, Anterior Ischemic

Overview: Optic Neuropathy, Anterior Ischemic
Differential Diagnoses & Workup: Optic Neuropathy, Anterior Ischemic
Treatment & Medication: Optic Neuropathy, Anterior Ischemic
Follow-up: Optic Neuropathy, Anterior Ischemic
Multimedia: Optic Neuropathy, Anterior Ischemic
References
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References

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  2. Ali Ibn Isa. Memorandum Book of a Tenth-Century Oculist. (Translated by CA Wood). Chicago: Northwestern University; 1936.

  3. Hutchinson J. Diseases of the arteries. Arch Surg (London). 1890;1:323.

  4. Horton BT, Magath TB, Brown GE. An undescribed form of arteritis of the temporal vessels. Proc Staff Meet Mayo Clinic. 1932;7:700.

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  13. Ischemic Optic Neuropathy Decompression Trial. Characteristics of patients with nonarteritic anterior ischemic optic neuropathy eligible for the Ischemic Optic Neuropathy Decompression Trial. Arch Ophthalmol. Nov 1996;114(11):1366-74. [Medline].

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  15. Kuprjanowicz L, Goslawski W, Karczewicz D, Szych Z. [Evaluation of retinal nerve fiber thickness with scanning laser polarimetry in patients with anterior ischemic optic neuropathy]. Klin Oczna. 2004;106(3 Suppl):440-2. [Medline].

  16. Love DC, Rapkin J, Lesser GR, Shmookler BM, Kolsky MP, Jackson B, et al. Temporal arteritis in blacks. Ann Intern Med. Sep 1986;105(3):387-9. [Medline].

  17. Munteanu M, Lehaci C. [Acute anterior ischemic optic neuropathy in association with optic nerve drusen]. Oftalmologia. 2004;48(3):16-9. [Medline].

  18. Purvin V, King R, Kawasaki A, Yee R. Anterior ischemic optic neuropathy in eyes with optic disc drusen. Arch Ophthalmol. Jan 2004;122(1):48-53. [Medline].

  19. Salomon O, Rosenberg N, Steinberg DM, Huna-Baron R, Moisseiev J, Dardik R, et al. Nonarteritic anterior ischemic optic neuropathy is associated with a specific platelet polymorphism located on the glycoprotein Ibalpha gene. Ophthalmology. Jan 2004;111(1):184-8. [Medline].

  20. The Ischemic Optic Neuropathy Decompression Trial Research Group. Optic nerve decompression surgery for nonarteritic anterior ischemic optic neuropathy (NAION) is not effective and may be harmful. The Ischemic Optic Neuropathy Decompression Trial Research Group. JAMA. Feb 22 1995;273(8):625-32. [Medline].

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Further Reading

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Keywords

anterior ischemic optic neuropathy, AION, nonarteritic anterior ischemic optic neuropathy, NAION, arteritic anterior ischemic optic neuropathy, ischemic optic neuropathy, ION, giant cell arteritis, optic atrophy, optic nerve, optic disc

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Contributor Information and Disclosures

Author

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.

Medical Editor

Edsel Ing, MD, FRCSC, Assistant Professor, Department of Ophthalmology & Vision Sciences, University of Toronto, Sunnybrook and Women's Health Sciences Center, Toronto East General Hospital
Edsel Ing, MD, FRCSC is a member of the following medical societies: American Academy of Ophthalmology, Canadian Medical Association, Canadian Ophthalmological Society, and North American Neuro-Ophthalmology Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Simon K Law, MD, PharmD, Assistant Professor of Ophthalmology, Jules Stein Eye Institute; Chief of Section of Ophthalmology Surgical Services, Department of Veterans Affairs Healthcare Center, West Los Angeles
Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, American Glaucoma Society, and Association for Research in Vision and Ophthalmology
Disclosure: Nothing to disclose.

CME Editor

Lance L Brown, OD, MD, Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri
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, and Pan-American Association of Ophthalmology
Disclosure: Nothing to disclose.

 
 
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