eMedicine Specialties > Ophthalmology > Optic Nerve

Optic Neuritis, Childhood

Author: John E Carter, MD, Associate Professor, Department of Medicine, Assistant Professor, Department of Ophthalmology, University of Texas Health Science Center at San Antonio
Contributor Information and Disclosures

Updated: Aug 29, 2008

Introduction

Background

Optic neuritis implies an inflammatory process involving the optic nerve.

In children, most cases of optic neuritis are due to an immune-mediated process. These cases of optic neuritis may be associated with a viral or other infection or with immunization. Less commonly, optic neuritis may be the first manifestation of multiple sclerosis (MS) or part of a more diffuse demyelinating disorder, including acute disseminated encephalomyelitis or neuromyelitis optica (Devic disease). Optic neuritis may be related to specific infections, diseases of the adjacent sinuses or orbital structures, and infectious and infiltrative diseases of the brain or meninges that involve the optic nerves.

The following definitions aid in further understanding optic neuritis:

• Papillitis - Optic neuritis involving the optic disc with disc edema
• Retrobulbar optic neuritis - Optic neuritis involving the optic nerve behind the globe. The optic disc appearance should be normal in first-time episodes of retrobulbar optic neuritis.
• Bilateral simultaneous optic neuritis - Optic neuritis in both eyes occurring within 3 weeks of each other
• Bilateral sequential optic neuritis - Optic neuritis occurring in both optic nerves but separated by a period of more than 3 weeks
• Neuroretinitis - Inflammatory process involving the optic discs with exudative changes in the nerve fiber layer of the retina producing a partial or complete macular star. In the past, this condition was called Leber idiopathic stellate neuroretinitis, but now a number of underlying causes for this condition are known. Because this condition is not associated with demyelinating disease and does not imply a future risk of MS, the distinction is important.

Pathophysiology

Possible mechanisms of inflammation in immune-mediated optic neuritis are the cross-reaction of viral epitopes and host epitopes and the persistence of a virus in central nervous system (CNS) glial cells.

Frequency

United States

Optic neuritis is much less common in children than in adults but is not rare. In one combined series, children comprised 5% of cases.

Mortality/Morbidity

Patients with optic neuritis have a good prognosis, but a minority of patients experience persistent visual loss. Patients with neuromyelitis optica generally have a poorer recovery. When optic neuritis is associated with other CNS diseases, the morbidity and mortality of those disorders contribute substantially to the final outcome.

Race

Optic neuritis is more common in whites than in other races.

Sex

In both children and adults, a female predominance exists. Females comprise 60-75% of patients.

Age

• Optic neuritis may occur at any age, including in infants younger than 1 year.
• A comparison of adult optic neuritis and childhood optic neuritis is presented in Table 1. These features are generally true but are not absolute, and they do overlap.

Table 1. Comparison of Features of Optic Neuritis in Adults and Children 

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Table

Adult Optic Neuritis	Pediatric Optic Neuritis
Unilateral	Bilateral
Retrobulbar optic neuritis	Papillitis
Commonly associated with pain on eye movements	Commonly associated with headache
Most often idiopathic	Most often postinfectious or postimmunization
High probability of recurrent inflammatory demyelinating events in the CNS and a diagnosis of MS	Low probability of recurrent demyelinating events and a diagnosis of MS

Adult Optic Neuritis	Pediatric Optic Neuritis
Unilateral	Bilateral
Retrobulbar optic neuritis	Papillitis
Commonly associated with pain on eye movements	Commonly associated with headache
Most often idiopathic	Most often postinfectious or postimmunization
High probability of recurrent inflammatory demyelinating events in the CNS and a diagnosis of MS	Low probability of recurrent demyelinating events and a diagnosis of MS

Clinical

History

• In general, obtaining an accurate history from children may be difficult. Young children may not notice unilateral visual loss and may not report bilateral visual loss until their behavior indicates visual loss to parents or teachers.
• Optic neuritis produces a subacute loss of vision, reaching its maximum deficit in a few days to 2-3 weeks. In many cases, recovery is already underway at 2-3 weeks.
• Headache is common in children with optic neuritis. Periorbital pain, especially if it worsens with eye movements, supports a diagnosis of optic neuritis.
• Visual symptoms reflect the expected deficit observed in any optic neuropathy, including loss of visual acuity, change in color perception, change in brightness sense, and loss of portions of the visual field.
• In reviewing neurologic symptoms, prior resolved neurologic symptoms imply a recurrent process, such as MS, whereas ongoing neurologic symptoms may indicate MS, acute disseminated encephalomyelitis, or neuromyelitis optica.
• A review of systemic symptoms should be aimed at detecting recent vaccinations, infections, or vasculitis.

Physical

• Decreased visual acuity: Visual acuity is not the most sensitive indicator of optic nerve injury and may be normal; however, most children with optic neuritis have some loss of visual acuity. Occasionally, patients with optic neuritis have severe loss of vision that includes no light perception.
• Decreased color acuity: A deficit in color vision is a more sensitive indicator of optic nerve injury, and, when checked, a deficit is expected that is out of proportion to any loss of visual acuity.
• Afferent pupil defect: In unilateral cases of optic neuritis, an afferent pupil defect should be present. In bilateral cases of optic neuritis, this sign is less reliable unless the 2 nerves are asymmetrically affected.
• Subjective light brightness difference between the 2 eyes is also common in asymmetric or unilateral cases of optic neuritis.
• Fundus examination
  • In children, most cases (60-70%) of optic neuritis involve the optic disc with disc edema, as compared to 35% in adults (see Media file 1). 
  • In retrobulbar optic neuritis, the optic disc should be normal. Atrophy of the disc implies a prior episode of optic neuritis or another more chronic process, such as an optic nerve glioma, a craniopharyngioma, or other compressive process.
  • If macular edema or a macular star is associated, a diagnosis of neuroretinitis rather than optic neuritis should be made (see Media file 2).

Causes

• Immune mediated
  • In children, as many as 85% of cases of optic neuritis are associated with a recent immunization or an infection, usually a viral infection.
  • Optic neuritis can be associated with a preceding nonviral infection, such as pertussis, infectious mononucleosis, toxoplasmosis, or brucella.
• Multiple sclerosis
• Neuromyelitis optica (Devic disease)
• Idiopathic
• Lyme disease in endemic areas
• Specific meningeal infections and infiltrations involving the optic nerves, including cryptococcus, tuberculosis, and sarcoidosis
• Vasculitis, such as systemic lupus erythematosus
• Syphilis
• Leukemia
• Associated with bee and wasp stings
• Several cases of optic neuritis have been seen in patients on anti-tumor necrosis factor (anti-TNF) drugs.
• Causes of neuroretinitis
  • Following a viral syndrome
  • Cat scratch disease
  • Toxoplasmosis
  • Toxocariasis and helminths (The finding of a discrete white inflammatory mass overlying the optic disc is suggestive of toxocariasis and helminths.)
  • Lyme disease, usually stage 2
  • Syphilis, especially secondary syphilis as part of a meningitis

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