Adult Optic Neuritis 

  • Author: Erhan Ergene, MD; Chief Editor: Hampton Roy Sr, MD   more...
 
Updated: Feb 9, 2012
 

Background

Optic neuritis (ON) is a demyelinating inflammation of the optic nerve that typically first occurs in young adulthood (see the image below). Many cases of ON are associated with multiple sclerosis (MS) or neuromyelitis optica (NMO), but ON can occur in isolation.[1] In cases associated with MS, ON is commonly the first manifestation of the chronic demyelinating process.[2] Long-term follow-up studies have indicated that up to 75% of female patients initially presenting with ON ultimately develop MS. (See Presentation and Prognosis.)

A case of acute optic neuritis. A. 1.5 Tesla, contA 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).

Occasionally, ON can result from an infectious process involving the orbits or paranasal sinuses or occur in the course of a systemic viral infection.[3, 4, 5, 6, 7, 8, 9, 10, 11] Certain optic neuropathies, such as anterior ischemic optic neuropathy (AION) and compressive and hereditary optic neuropathies, can resemble ON.[12]

This article reviews ON as a primary demyelinating inflammation of the nerve occurring either in isolation or in association with MS or NMO. (NMO is a severe form of a demyelinating disease; it affects the optic nerves and the spinal cord, causing recurrent attacks of blindness and paralysis.[13, 14] ) 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. (See Etiology, Treatment, and Medication.)[15, 16, 17, 18]

Patient education

For patient education information, see Multiple Sclerosis.

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Etiology

Most cases of ON are associated with MS, even though ON can occur in isolation. In MS-associated and isolated, monosymptomatic ON, the cause is presumed to be an autoimmune reaction that results in a demyelinating inflammation of the nerve. Pathologic 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.

In a single case of chronic, isolated ON, a biopsy specimen 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 etiology similar to MS.

NMO has been recognized as a distinct inflammatory demyelinating disease consisting of ON in combination with longitudinally extensive transverse myelitis. NMO is associated with the presence of a specific serum, NMO IgG autoantibody, which targets the water channel aquaporin-4.[19, 20, 21]

As previously stated, ON can occasionally result from an infectious process involving the orbits or paranasal sinuses or occur in the course of a systemic viral infection.[3, 4, 5, 6, 7, 8, 9, 10, 11]

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Epidemiology

Studies from Sweden and Denmark have reported an annual incidence of 4-5 cases of new-onset ON per 100,000 persons.[22] Patients living in temperate climates seem to be predisposed to ON.

Race-, sex-, and age-related demographics

ON appears to affect Caucasians more commonly than it does other races. Women are affected twice as often as men.[15]

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

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Prognosis

In contrast to ischemic optic neuropathies and compressive optic neuropathies, a gradual recovery of visual acuity with time is characteristic of ON.[23] For most patients with ON, visual function begins to improve 1 week to several weeks after onset, even without any treatment. However, permanent residual deficits in color vision and contrast and brightness sensitivity are common.[18]

Decreased visual acuity secondary to ON may be permanent. Final visual outcome may be better in patients with an isolated episode of ON, compared with patients who eventually develop MS. Up to 75% of female patients and 35% of male patients initially presenting with ON ultimately develop MS.[24, 25, 26]

Patients with silent demyelinative lesions elsewhere in the brain, observed on magnetic resonance imaging (MRI) performed at the initial presentation, are more likely to develop definite MS in the long term than are patients with isolated ON. In addition, patients who have recurrent episodes of ON may be more likely to develop MS.

In patients with normal findings on MRI, a 16% risk of progression to clinically definite MS exists at 5-year follow-up.

Most patients with relapsing NMO have an aggressive form of the disease that is associated with frequent and severe exacerbations and poor prognosis.

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Proceed to Clinical Presentation
 
 
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.

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.

Additional Contributors

Edsel Ing, MD, FRCSC Associate Professor, Department of Ophthalmology and Vision Sciences, University of Toronto Faculty of Medicine; Consulting Staff, Toronto East General Hospital, Canada

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, Canadian Ophthalmological Society, North American Neuro-Ophthalmology Society, and Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Simon K Law, MD, PharmD Associate Professor 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, American Glaucoma Society, and Association for Research in Vision and Ophthalmology

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.

Acknowledgments

The authors and editors of Medscape Reference gratefully acknowledge the assistance of Ryan I Huffman, MD, with the literature review and referencing for this article.

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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).
 
 
 
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