eMedicine Specialties > Ophthalmology > Optic Nerve

Optic Neuritis, Adult: Differential Diagnoses & Workup

Author: Erhan Ergene, MD, Clinical Assistant Professor, Department of Neurology, University of Illinois College of Medicine at Peoria; Medical Director, Comprehensive Epilepsy Program and Clinical Neurophysiology, Illinois Neurological Institute at OSF Saint Francis Medical Center
Coauthor(s): Nancy A Machens, APN, CNP, Professor of Nursing, Bradley University; Advanced Practice Nurse, Nurse Practitioner, Department of Neurology, Illinois Neurological Institute at OSF Saint Francis Medical Center
Contributor Information and Disclosures

Updated: Jul 30, 2009

Differential Diagnoses

Branch Retinal Artery Occlusion
Optic Neuropathy, Anterior Ischemic
Central Retinal Artery Occlusion
Optic Neuropathy, Compressive
Glaucoma, Angle Closure, Acute
Sarcoidosis
Herpes Simplex
Sudden Visual Loss
Meningioma, Optic Nerve Sheath
Thyroid Ophthalmopathy
Ocular Manifestations of Syphilis
Toxic/Nutritional Optic Neuropathy

Other Problems to Be Considered

Neuromyelitis optica
Hereditary optic neuropathies
Nutritional optic neuropathies
Wegener granulomatosis
Necrotizing herpetic retinopathy in persons who are immunocompromised

Workup

Laboratory Studies

  • Blood tests, such as erythrocyte sedimentation rate, thyroid function tests, antinuclear antibodies, angiotensin-converting enzyme, rapid plasma reagin, and mitochondrial DNA mutation studies can be considered to exclude causes of optic neuropathy other than demyelinating optic neuritis (ON). However, in a typical case of ON without any clinical signs and symptoms of a systemic disease, the yield from these tests is extremely low.
  • CSF analysis often is noncontributory to diagnosis. However, the presence of myelin basic protein, oligoclonal bands, and elevated IgG index and synthesis rate in the CSF supports the diagnosis of MS, and, even in the absence of other signs of MS during the initial presentation, patients with positive findings of demyelination in the CSF are more likely to develop MS in the long term.25
  • Neuromyelitis optica-IgG is a specific autoantibody marker for neuromyelitis optica, which is a severe form of a demyelinating disease, likely distinct from MS, that affects the optic nerves and the spinal cord, causing recurrent attacks of blindness and paralysis.26,27

Imaging Studies

  • Magnetic resonance imaging (MRI) is a highly sensitive and specific tool in assessing inflammatory changes in the optic nerves (see Media file 1) and helps to rule out structural lesions. In addition, MRI may have a value in predicting future development of MS in patients presenting with first time acute ON.28,29,30,31,32,33,34 MRI performed at the initial presentation reveals that 10-20% of these patients may have clinically silent demyelinative lesions elsewhere in the brain. MRI at 3.0 T is more sensitive to hyperintense lesions than MRI at 1.5 T.35 These patients are more likely to develop definite MS in the long term than patients with isolated ON. The Optic Neuritis Treatment Trial (ONTT) reported the 10-year risk of MS to be 56% with at least one MR T2 lesion.16
  • Utilization of fat saturation techniques helps to visualize gadolinium enhancement of the optic nerve and is the best imaging technique to visualize inflammation of the optic nerve.
  • In addition to the MRI of the optic nerves and brain/brainstem, an MRI of the spinal cord is indicated in patients with suspected neuromyelitis optica (NMO). An MRI of the spinal cord characteristically shows cord swelling, signal changes, and enhancement extending over several levels consistent with longitudinally extensive myelitis.36

A case of acute optic neuritis. A. 1.5 Tesla, con...

A case of acute optic neuritis. A. 1.5 Tesla, contrast-enhanced spin echo T1-weighted, fat-suppressed coronal MRI through the orbits shows enlargement and contrast enhancement of the left optic nerve in the retrobulbar portion (arrow). B. Coronal spin echo T1-weighted, fat-suppressed MRI of the same patient shows enlargement and contrast enhancement of the nerve in a parasagittal oblique section (arrow).

A case of acute optic neuritis. A. 1.5 Tesla, con...

A case of acute optic neuritis. A. 1.5 Tesla, contrast-enhanced spin echo T1-weighted, fat-suppressed coronal MRI through the orbits shows enlargement and contrast enhancement of the left optic nerve in the retrobulbar portion (arrow). B. Coronal spin echo T1-weighted, fat-suppressed MRI of the same patient shows enlargement and contrast enhancement of the nerve in a parasagittal oblique section (arrow).

Other Tests

  • Visually-evoked potentials (VEPs) are an important means of evaluating patients with suspected ON and may be abnormal, even when MRI of the optic nerve is normal.
    • VEP often shows a loss of P100 response in the acute phase. P100 recovers with time, but it usually shows a markedly prolonged latency that persists indefinitely even after clinical recovery.
    • VEP may be abnormal in patients without a past history of ON, thereby providing evidence of subclinical involvement of the optic nerve. For this reason, VEP often is performed in patients with a suspected diagnosis of MS.

More on Optic Neuritis, Adult

Overview: Optic Neuritis, Adult
Differential Diagnoses & Workup: Optic Neuritis, Adult
Treatment & Medication: Optic Neuritis, Adult
Follow-up: Optic Neuritis, Adult
Multimedia: Optic Neuritis, Adult
References

References

  1. Beck RW. Optic neuritis: Clinical considerations and the relationship to multiple sclerosis. Neuro-ophthalmology. 1998;20.1-20.3.

  2. Sorensen TL, Frederiksen JL, Bronnum-Hansen H, Petersen HC. Optic neuritis as onset manifestation of multiple sclerosis: a nationwide, long-term survey. Neurology. Aug 11 1999;53(3):473-8. [Medline].

  3. Kountakis SE, Maillard AA, Stiernberg CM. Optic neuritis secondary to sphenoethmoiditis: surgical treatment. Am J Otolaryngol. Nov-Dec 1995;16(6):422-7. [Medline].

  4. Monteiro ML, Borges WI, do Val Ferreira Ramos C, Lucato LT, Leite CC. Bilateral optic neuritis in wegener granulomatosis. J Neuroophthalmol. Mar 2005;25(1):25-8. [Medline].

  5. Nakamoto BK, Dorotheo EU, Biousse V, Tang RA, Schiffman JS, Newman NJ. Progressive outer retinal necrosis presenting with isolated optic neuropathy. Neurology. Dec 28 2004;63(12):2423-5. [Medline].

  6. Rush JA, Kennerdell JS, Martinez AJ. Primary idiopathic inflammation of the optic nerve. Am J Ophthalmol. Mar 1982;93(3):312-6. [Medline].

  7. Sanborn GE, Kivlin JD, Stevens M. Optic neuritis secondary to sinus disease. Arch Otolaryngol. Dec 1984;110(12):816-9. [Medline].

  8. Selbst RG, Selhorst JB, Harbison JW, Myer EC. Parainfectious optic neuritis. Report and review following varicella. Arch Neurol. Jun 1983;40(6):347-50. [Medline].

  9. Lee SB, Lee EK, Kim JY. Bilateral optic neuritis in leprosy. Can J Ophthalmol. Apr 2009;44(2):219-20. [Medline].

  10. Siddiqui J, Rouleau J, Lee AG, Sato Y, Voigt MD. Bilateral optic neuritis in acute hepatitis C. J Neuroophthalmol. Jun 2009;29(2):128-33. [Medline].

  11. Lee HS, Choi KD, Lee JE, Park HK. Optic neuritis after Klebsiella pneumonitis and liver abscess. J Neuroophthalmol. Jun 2009;29(2):134-5. [Medline].

  12. Rizzo JF 3rd, Lessell S. Optic neuritis and ischemic optic neuropathy. Overlapping clinical profiles. Arch Ophthalmol. Dec 1991;109(12):1668-72. [Medline].

  13. Pittock SJ, Weinshenker BG, Lucchinetti CF, Wingerchuk DM, Corboy JR, Lennon VA. Neuromyelitis optica brain lesions localized at sites of high aquaporin 4 expression. Arch Neurol. Jul 2006;63(7):964-8. [Medline].

  14. Matsushita T, Isobe N, Matsuoka T, et al. Aquaporin-4 autoimmune syndrome and anti-aquaporin-4 antibody-negative opticospinal multiple sclerosis in Japanese. Mult Scler. Jul 2009;15(7):834-47. [Medline].

  15. Koch-Henriksen N, Hyllested K. Epidemiology of multiple sclerosis: incidence and prevalence rates in Denmark 1948-64 based on the Danish Multiple Sclerosis Registry. Acta Neurol Scand. Nov 1988;78(5):369-80. [Medline].

  16. Beck RW, Trobe JD, Moke PS, et al. High- and low-risk profiles for the development of multiple sclerosis within 10 years after optic neuritis: experience of the optic neuritis treatment trial. Arch Ophthalmol. Jul 2003;121(7):944-9. [Medline].

  17. Atkins EJ, Biousse V, Newman NJ. The natural history of optic neuritis. Rev Neurol Dis. Spring 2006;3(2):45-56. [Medline].

  18. Flanagan P, Zele AJ. Chromatic and luminance losses with multiple sclerosis and optic neuritis measured using dynamic random luminance contrast noise. Ophthalmic Physiol Opt. May 2004;24(3):225-33. [Medline].

  19. de Stoppelaar JD. [Bacteria as cause of dental caries]. Tijdschr Ziekenverpl. Nov 18 1975;28(23):1094-7. [Medline].

  20. Wingerchuk DM, Lennon VA, Lucchinetti CF, et al. The spectrum of neuromyelitis optica. Lancet Neurol. 2007;6(9):810-815.

  21. Wingerchuk DM, Lennon VA, Pittock SJ, Lucchinetti CF, Weinshenker BG. Revised diagnostic criteria for neuromyelitis optica. Neurology. May 23 2006;66(10):1485-9. [Medline].

  22. Cree B. Neuromyelitis optica: diagnosis, pathogenesis, and treatment. Current Neurology & Neuroscience Reports. Sep/2008;8(5):427-33.

  23. Kattah JC, Gujrati M. Neuromyelitis optica and autoimmune diseases. Arch Neurol. Jul 2008;65(7):995-6; author reply 996. [Medline].

  24. Warner JE, Lessell S, Rizzo JF 3rd, Newman NJ. Does optic disc appearance distinguish ischemic optic neuropathy from optic neuritis?. Arch Ophthalmol. Nov 1997;115(11):1408-10. [Medline].

  25. Söderström M, Ya-Ping J, Hillert J, Link H. Optic neuritis: prognosis for multiple sclerosis from MRI, CSF, and HLA findings. Neurology. Mar 1998;50(3):708-14. [Medline].

  26. Lennon VA, Kryzer TJ, Pittock SJ, Verkman AS, Hinson SR. IgG marker of optic-spinal multiple sclerosis binds to the aquaporin-4 water channel. J Exp Med. Aug 15 2005;202(4):473-7. [Medline].

  27. Lennon VA, Wingerchuk DM, Kryzer TJ, et al. A serum autoantibody marker of neuromyelitis optica: distinction from multiple sclerosis. Lancet. Dec 11-17 2004;364(9451):2106-12. [Medline].

  28. Cornblath WT, Quint DJ. MRI of optic nerve enlargement in optic neuritis. Neurology. Apr 1997;48(4):821-5. [Medline].

  29. Frederiksen JL, Larsson HB, Henriksen O, Olesen J. Magnetic resonance imaging of the brain in patients with acute monosymptomatic optic neuritis. Acta Neurol Scand. Dec 1989;80(6):512-7. [Medline].

  30. Frederiksen JL, Larsson HB, Olesen J. Correlation of magnetic resonance imaging and CSF findings in patients with acute monosymptomatic optic neuritis. Acta Neurol Scand. Sep 1992;86(3):317-22. [Medline].

  31. Hickman SJ, Toosy AT, Jones SJ, et al. A serial MRI study following optic nerve mean area in acute optic neuritis. Brain. Nov 2004;127:2498-505. [Medline].

  32. CHAMPS Study Group. MRI predictors of early conversion to clinically definite MS in the CHAMPS placebo group. Neurology. Oct 8 2002;59(7):998-1005. [Medline].

  33. Ergene E, Rupp FW Jr, Qualls CR, Ford CC. Acute optic neuritis: association with paranasal sinus inflammatory changes on magnetic resonance imaging. J Neuroimaging. Oct 2000;10(4):209-15. [Medline].

  34. Ormerod IE, McDonald WI, du Boulay GH, et al. Disseminated lesions at presentation in patients with optic neuritis. J Neurol Neurosurg Psychiatry. Feb 1986;49(2):124-7. [Medline].

  35. Nielsen K, Rostrup E, Frederiksen JL, et al. Magnetic resonance imaging at 3.0 tesla detects more lesions in acute optic neuritis than at 1.5 tesla. Invest Radiol. Feb 2006;41(2):76-82. [Medline].

  36. Pittock SJ, Lennon VA, Krecke K, Wingerchuk DM, Lucchinetti CF, Weinshenker BG. Brain abnormalities in neuromyelitis optica. Arch Neurol. Mar 2006;63(3):390-6. [Medline].

  37. Optic Neuritis Study Group. Long-term brain magnetic resonance imaging changes after optic neuritis in patients without clinically definite multiple sclerosis. Arch Neurol. Oct 2004;61(10):1538-41. [Medline].

  38. Optic Neuritis Study Group. The clinical profile of optic neuritis. Experience of the Optic Neuritis Treatment Trial. Optic Neuritis Study Group. Arch Ophthalmol. Dec 1991;109(12):1673-8. [Medline].

  39. Arnold AC. Evolving management of optic neuritis and multiple sclerosis. Am J Ophthalmol. Jun 2005;139(6):1101-8. [Medline].

  40. Trobe JD, Sieving PC, Guire KE, Fendrick AM. The impact of the optic neuritis treatment trial on the practices of ophthalmologists and neurologists. Ophthalmology. Nov 1999;106(11):2047-53. [Medline].

  41. Watanabe S, Nakashima I, Misu T, Miyazawa I, Shiga Y, Fujihara K. Therapeutic efficacy of plasma exchange in NMO-IgG-positive patients with neuromyelitis optica. Mult Scler. Jan 2007;13(1):128-32. [Medline].

  42. Ruprecht K, Klinker E, Dintelmann T, Rieckmann P, Gold R. Plasma exchange for severe optic neuritis: treatment of 10 patients. Neurology. Sep 28 2004;63(6):1081-3. [Medline].

  43. Jacobs LD, Beck RW, Simon JH, et al. Intramuscular interferon beta-1a therapy initiated during a first demyelinating event in multiple sclerosis. CHAMPS Study Group. N Engl J Med. Sep 28 2000;343(13):898-904. [Medline].

  44. Beck RW, Smith CH, Gal RL, et al. Neurologic impairment 10 years after optic neuritis. Arch Neurol. Sep 2004;61(9):1386-9. [Medline].

  45. Beck RW, Gal RL, Bhatti MT, et al. Visual function more than 10 years after optic neuritis: experience of the optic neuritis treatment trial. Am J Ophthalmol. Jan 2004;137(1):77-83. [Medline].

  46. Levin LA, Lessell S. Optic neuritis and multiple sclerosis. Arch Ophthalmol. Jul 2003;121(7):1039-40. [Medline].

  47. Levin LA, Lessell S. Risk of multiple sclerosis after optic neuritis. JAMA. Jul 16 2003;290(3):403-4. [Medline].

  48. Osborne BJ, Volpe NJ. Optic neuritis and risk of MS: differential diagnosis and management. Cleve Clin J Med. Mar 2009;76(3):181-90. [Medline].

Further Reading

Keywords

adult optic neuritis, ON, optic nerve, multiple sclerosis, MS, neuromyelitis optica, NMO

Contributor Information and Disclosures

Author

Erhan Ergene, MD, Clinical Assistant Professor, Department of Neurology, University of Illinois College of Medicine at Peoria; Medical Director, Comprehensive Epilepsy Program and Clinical Neurophysiology, Illinois Neurological Institute at OSF Saint Francis Medical Center
Erhan Ergene, MD is a member of the following medical societies: American Academy of Neurology
Disclosure: Nothing to disclose.

Coauthor(s)

Nancy A Machens, APN, CNP, Professor of Nursing, Bradley University; Advanced Practice Nurse, Nurse Practitioner, Department of Neurology, Illinois Neurological Institute at OSF Saint Francis Medical Center
Disclosure: Nothing to disclose.

Medical Editor

Edsel Ing, MD, FRCSC, Assistant Professor, Department of Ophthalmology & Vision Sciences, University of Toronto: Consulting Staff, Toronto East General Hospital
Edsel Ing, MD, FRCSC is a member of the following medical societies: American Academy of Ophthalmology, American College of Physician Executives, American Society of Contemporary Ophthalmology, Canadian Ophthalmological Society, Contact Lens Association of Ophthalmologists, North American Neuro-Ophthalmology Society, and Royal College of Physicians and Surgeons of Canada
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.

Managing Editor

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.

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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