eMedicine Specialties > Ophthalmology > Optic Nerve

Optic Neuritis, Adult

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: Jan 4, 2007

Introduction

Background

Optic neuritis (ON) is a demyelinating inflammation of the optic nerve. Many cases of ON are associated with multiple sclerosis (MS), but ON can occur in isolation. In cases associated with MS, ON is commonly the first manifestation of the chronic demyelinating process. Long-term follow-up studies have indicated that up to 75% of female patients initially presenting with ON ultimately develop MS. Occasionally, ON can be due to an infectious process involving the orbits or paranasal sinuses or occur in the course of a systemic viral infection. Certain optic neuropathies, such as anterior ischemic optic neuropathy (AION) and compressive and hereditary optic neuropathies, can resemble ON. This article reviews ON as a primary demyelinating inflammation of the nerve occurring either in isolation or in association with MS.

Much information has been gleaned from the Optic Neuritis Treatment Trial (ONTT), and the reader is encouraged to review the follow-up data from this study.

Pathophysiology

In both MS-associated and isolated monosymptomatic ON, the cause is presumed to be an autoimmune reaction, resulting in a demyelinating inflammation of the nerve. Pathological studies in patients with ON associated with MS have shown that the demyelinative lesions in the optic nerve are similar to the MS plaques seen in the brain, with an inflammatory response marked by perivascular cuffing, T cells, and plasma cells. However, little is known about the pathology of isolated ON.

Biopsy performed in a single case of chronic isolated ON showed the presence of perivascular lymphocytic infiltration, multifocal demyelination, and reactive astrocytosis in the retrobulbar portion of the optic nerve. Abnormal intrathecal immunoglobulin G (IgG) synthesis, reflected as the presence of oligoclonal bands in the cerebrospinal fluid (CSF), is found in 60-70% of patients with isolated ON, suggesting an immunologic pathophysiology similar to MS.

Frequency

International

Studies from Sweden and Denmark have reported an annual incidence of 4-5 per 100,000 for new onset ON cases.

Mortality/Morbidity

Decreased visual acuity secondary to optic neuritis may be permanent.

Race

ON appears to affect Caucasians more commonly than other races. (Patients living in temperate climates seem to be predisposed to ON.)

Sex

Women are affected twice as often as men.

Age

Typically, patients with first time acute ON are young adults aged 20-45 years. Atypical cases of ON occasionally are seen in elderly patients. Bilateral ON in childhood is not uncommon, and it is believed there is less risk of progression to MS.

Clinical

History

  • Patients with ON experience rapidly developing impairment of vision in one eye or less commonly in both eyes during an acute attack.
  • Dyschromatopsia (change in color perception) in the affected eye occasionally may be more prominent than the decreased vision. In nearly all cases, the visual changes are associated with a retroorbital or ocular pain, usually exacerbated by eye movement. The pain may precede the visual loss.
  • Patients may complain of vision loss exacerbated by heat or exercise (Uhthoff phenomenon). Objects moving in a straight line may appear to have a curved trajectory (Pulfrich phenomenon), presumably due to asymmetric conduction between the optic nerves.
  • Typically, patients with first time acute ON are otherwise healthy young adults.
  • A history of preceding viral illness may be present.
  • Patients with MS may have recurrent attacks of ON. Therefore, a history of previous episodes of decreased vision in the same or the fellow eye may be elicited. A previous history of neurologic problems, such as transient episodes of extremity/facial numbness or weakness, suggests a diagnosis of MS. A family history of MS may exist.
  • In patients, especially males with bilateral sequential optic neuropathy with little recovery of vision and optic neuropathy, exclude Leber hereditary optic neuropathy (LHON). Patients with LHON may have a history of vision loss in maternal uncles.

Physical

In a typical first time acute ON case, general physical examination is normal.

  • Pupillary light reaction is decreased in the affected eye and a relative afferent pupillary defect (RAPD) or Marcus Gunn pupil commonly is found. In bilateral cases, the RAPD may not be apparent.
  • Measurement of visual acuity (VA) reveals varying degrees of reduction in vision from a mildly decreased VA to complete visual loss. However, VA may be normal with only a limited, mild visual field defect. Almost all patients with decreased VA also have abnormal contrast sensitivity and color vision revealed by examination using a Pelli-Robson chart and Ishihara color plates, respectively.
  • Classic dictum states that a central scotoma most commonly is seen in ON. However, the ONTT suggested that altitudinal field defects, arcuate defects, and nasal steps were more common than central scotomas and cecocentral scotomas. Visual field examination typically shows a central scotoma. Peripheral extension of the scotoma in any direction and even a generalized depression of the entire visual field may be encountered.
  • In acute ON, the fundus appears normal because two thirds of cases of ON are retrobulbar. With time, the optic nerve may become pale.
  • One third of patients with ON have a swollen disc (papillitis). The disc edema of ON often is diffuse. The presence of segmental changes, altitudinal swelling, pallor, arterial attenuation, and splinter hemorrhages suggest other diagnoses (eg, AION).
  • If a dilated fundus examination is not performed, retinal problems, such as central serous retinopathy and retinal detachment, may be mistaken for ON.

Causes

As noted, most cases of ON are associated with MS even though ON can occur in isolation. In both MS-associated and isolated monosymptomatic ON, the cause is presumed to be an autoimmune reaction, resulting in a demyelinating inflammation of the nerve.

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
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Further Reading

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Keywords

adult optic neuritis, ON, optic nerve, multiple sclerosis, MS

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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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