You are in: eMedicine Specialties >
Ophthalmology > OPTIC NERVE
Optic Neuritis, Childhood
Article Last Updated: Aug 29, 2008
AUTHOR AND EDITOR INFORMATION
Section 1 of 11  
Author: John E Carter, MD, Associate Professor, Department of Medicine, Assistant Professor, Department of Ophthalmology, University of Texas Health Science Center at San Antonio
John E Carter is a member of the following medical societies: American Academy of Neurology, American Academy of Ophthalmology, and North American Neuro-Ophthalmology Society
Editors: Gerhard W Cibis, MD, Clinical Professor, Director of Pediatric Ophthalmology Service, Department of Ophthalmology, University of Kansas, Kansas City; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine; Brian R Younge, MD, Professor of Ophthalmology, Mayo Clinic School of Medicine; Ralph Garzia, OD, Assistant Dean for Clinical Programs, Associate Professor, School of Optometry, University of Missouri at St Louis; Hampton Roy Sr, MD, Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences
Author and Editor Disclosure
Synonyms and related keywords:
childhood optic neuritis, optic neuritis, optic nerve inflammation, optic nerve, acute vision loss, papillitis, retrobulbar optic neuritis, bilateral simultaneous optic neuritis, bilateral sequential optic neuritis, neuroretinitis, multiple sclerosis, MS, acute disseminated encephalomyelitis, neuromyelitis optica, Devic disease
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
| 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 |
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
Other Problems to be Considered
Neuromyelitis optica
- Formal diagnostic criteria for neuromyelitis optica are as follows: absolute criteria and one major supportive criterion or two minor supportive criteria.
- Absolute criteria
- Optic neuritis
- Acute myelitis
- No clinical disease outside the optic nerves and spinal cord
- Major supportive criteria
- Negative brain MRI at disease onset
- Spinal cord MRI with T2 signal abnormality extending over 3 or more vertebral segments
- Cerebrospinal fluid (CSF) pleocytosis greater than 50 WBC/mm3 or greater than 5 neutrophils/mm3
- Minor supportive criteria
- Bilateral optic neuritis
- Severe optic neuritis with fixed visual acuity worse than 20/200 in at least one eye
- Severe, fixed, attack-related weakness in one or more limbs
- Since there may be a delay between the optic neuritis and the myelitis, and since the treatment will be different, suspicion for neuromyelitis optica should prompt laboratory study for NMO (neuromyelitis optica) antibody.
Leber hereditary optic neuropathy
Papilledema from intracranial hypertension
Optic nerve glioma
Craniopharyngioma or pituitary adenoma
Lab Studies
- Lumbar puncture with measurement of opening pressure excludes papilledema secondary to intracranial hypertension.
- Cerebrospinal fluid (CSF) studies may indicate the presence of a simultaneous meningitis or encephalitis, but a mild lymphocytic pleocytosis may be present with optic neuritis.
- NMO (neuromyelitis optica) antibody in serum helps establish a diagnosis of neuromyelitis optica.
- Systemic lab studies can be performed directed by features in the history and physical examination consistent with other non–immune-mediated causes of optic neuritis.
Imaging Studies
- An MRI of the brain and orbits with contrast should be performed.
- Enhancement of the optic nerve in the orbit (see Media files 3-4) or the intracranial segment of the optic nerve or of the chiasm (see Media files 5-6) is helpful in confirming the diagnosis. Some enlargement of the optic nerve is present in optic neuritis, and a diagnosis of optic nerve glioma should not be made unless the clinical course dictates reconsideration of the diagnosis of optic neuritis.
- An MRI should exclude extrinsic compressive lesions.
- Meningeal enhancement suggests some form of infectious or noninfectious meningitis and may merit additional workup and different therapy.
- Changes in the CNS white matter may confirm other neurologic involvement found on physical examination, may affect the prognosis of MS in the future, or may indicate the presence of acute disseminated encephalomyelitis. One third of children with optic neuritis will have asymptomatic white matter lesions of the brain as compared to one half of adults with optic neuritis (see Media file 7).
- To either diagnose or exclude neuromyelitis optica (Devic disease), an MRI of the spinal cord with contrast is necessary if symptoms and signs consistent with a spinal cord process are present.
Medical Care
- The prognosis for visual recovery is excellent in adults with or without medical therapy, as found in the Optic Neuritis Treatment Trial (ONTT).
- No prospective study of the prognosis for visual recovery in children is available; most, but not all, studies of children have found that visual recovery is good. However, some authors, including those of Walsh & Hoyt's Clinical Neuro-ophthalmology, believe that a greater prevalence of steroid-responsive and steroid-dependent optic neuritis exists in children and routinely treat all their patients with high-dose corticosteroids.
- In the ONTT, high-dose steroids consisted of methylprednisolone 250 mg administered intravenously every 6 hours for 3 days, followed by oral prednisone 1 mg/kg daily for 11 days.
- No systematic study defining high-dose corticosteroids in childhood optic neuritis has been conducted. Walsh & Hoyt's Clinical Neuro-ophthalmology recommends methylprednisolone 1-2 mg/kg for 3-5 days, followed by a longer taper. Farris and Pickard used doses of methylprednisolone, ranging from 0.25-6.26 mg/kg, with one half of patients receiving doses of 125 mg or 250 mg every 6 hours for 5 days, followed by a taper.
- The author favors methylprednisolone 125-250 mg/kg administered intravenously every 6 hours for initial therapy.
- Treatment of the initial event in neuromyelitis optica, whether it is optic neuritis or myelitis, also uses high-dose intravenous steroids, but refractory cases are common and other therapies may be needed. Currently, plasma exchange is favored in those cases, but intravenous immune globulin (IVIg) has also been used.
- The equivalent doses of 4 commonly used drugs to treat optic neuritis are provided in Table 2.
Table 2. Equivalent Doses of Commonly Used Corticosteroid Medications
| Corticosteroid Drug | Approximate Equivalent Dose | | Prednisone | 5 mg | | Prednisolone | 5 mg | | Methylprednisolone | 4 mg | | Dexamethasone | 0.75 mg |
Consultations
The management of a child with optic neuritis is a combined effort by the ophthalmologist and the neurologist. Ophthalmologic input is required to distinguish between optic neuritis and neuroretinitis and to monitor visual response. Neurologic input is required to evaluate possible generalized CNS involvement and to make appropriate decisions and recommendations based on the future risk of MS. Children taking etanercept or other tumor necrosis factor (TNF) inhibitors should be reevaluated by their rheumatologists.
The goals of pharmacotherapy are to reduce morbidity and to prevent complications.
Drug Category: Corticosteroids
A widely used group of drugs. They have anti-inflammatory properties and cause profound and varied metabolic effects. They modify the body's immune response to diverse stimuli. GI tract absorption is approximately 85% of intravenously administered doses. Corticosteroids are well tolerated. A brief course of steroids is not expected to produce significant adrenal complications. Many physicians prescribe an H2 blocker to prevent GI tract distress.
| Drug Name | Methylprednisolone (Medrol, Adlone, Medralone injection) |
|---|
| Description | Decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reversing increased capillary permeability. |
|---|
| Pediatric Dose | 125-250 mg IV q6h for 3 d, followed by prednisone 1 mg/kg/d PO for 11 d |
|---|
| Contraindications | Documented hypersensitivity; viral, fungal, or tubercular skin infections |
|---|
| Interactions | Coadministration with digoxin may increase digitalis toxicity secondary to hypokalemia; estrogens may increase levels of methylprednisolone; phenobarbital, phenytoin, and rifampin may decrease levels of methylprednisolone (adjust dose); monitor patients for hypokalemia when taking medication concurrently with diuretics |
|---|
| Pregnancy | C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
|
|---|
| Precautions | Hyperglycemia, edema, osteonecrosis, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, growth suppression, myopathy, and infections are possible complications |
|---|
| Drug Name | Prednisone (Meticorten, Orasone, Deltasone, Sterapred) |
|---|
| Description | May decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. |
|---|
| Pediatric Dose | 1 mg/kg/d PO for 11 d; take with food or milk |
|---|
| Contraindications | Documented hypersensitivity; viral infection; peptic ulcer disease; hepatic dysfunction; connective tissue infections; fungal or tubercular skin infections; GI tract disease |
|---|
| Interactions | Coadministration with estrogens may decrease prednisone clearance; concurrent use with digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics |
|---|
| Pregnancy | B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
|
|---|
| Precautions | Abrupt discontinuation of glucocorticoids may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur |
|---|
Further Outpatient Care
- Outpatient follow-up care should include monitoring both visual recovery and recovery from neurologic or systemic disorders that were associated with optic neuritis.
- Children who do develop MS may be more likely to develop disability at a younger age. Monitoring patients who had no associated neurologic signs or symptoms at the time of their optic neuritis will allow early diagnosis. Treatment of those patients for early onset MS with the disease modifying agents that are used in adults may be an option.
- Recurrence of optic neuritis during or shortly after the discontinuation of steroids indicates a steroid-dependent optic neuritis and requires reevaluation and a more prolonged taper of corticosteroids.
Prognosis
- The prognosis for visual recovery generally is considered excellent (see Medical Care).
- Multiple sclerosis
- Adults with isolated optic neuritis have a substantial risk of developing MS. Approximately 15% of patients with normal MRIs at the time of their optic neuritis develop MS in the next 4 years. However, MRI findings are strongly correlated with risk of recurrence of demyelinating events, and 50% of patients whose MRIs demonstrate white matter lesions characteristic of MS at the time of their optic neuritis develop clinically definite MS in the next 4 years.
- Children with optic neuritis are less likely than adults to develop MS, but the risk is still present. A large study from the Mayo Clinic with a mean follow-up of 20 years produced a life-table analysis showing 13% of children with optic neuritis had progressed to clinically or laboratory-supported definite MS at 10 years (see Table 3 below). As in adult studies, those patients converting to MS were more likely to do so early; however, the longer the follow-up interval, the more patients there were who developed MS.
- A smaller study by Wilejto et al found that 36% of children with optic neuritis developed MS.1 All who did had abnormalities on the initial MRI, and bilateral cases were more likely to go on to develop MS.
Table 3. Life-Table Analysis of the Risk for Development of MS in Children With an Isolated Attack of Optic Neuritis2
| Age | Risk for Development of MS | | 10 years | 13% | | 20 years | 19% | | 30 years | 22% | | 40 years | 26% |
- MRI abnormalities in children are associated with the likelihood of developing MS.
- Both adults and children with more severe optic disc swelling are less likely to develop MS, and those with both severe optic disc swelling and retinal exudates rarely develop MS.
Patient Education
- With Uhthoff symptom, patients who have had demyelinating lesions with recovery often have symptoms return with exercise, exposure to heat (eg, hot baths), or febrile illnesses; a rapid return to baseline occurs when body temperature returns to normal. Warning patients about Uhthoff symptom is important so that they do not think they are having a recurrence.
- For excellent patient education resources, visit eMedicine's Muscle Disorders Center. Also, see eMedicine's patient education article, Multiple Sclerosis.
Medical/Legal Pitfalls
- Children with optic neuritis need to be evaluated by both an ophthalmologist and a neurologist. Failure to diagnose and treat patients with optic neuritis who have additional CNS involvement could be a potential source of liability. Efforts to identify those patients who are at high risk of developing future episodes of demyelination and MS are motivated by the belief that early treatment with immunomodulator therapy is important. Although currently less of an issue with children, such decisions are in the purview of the pediatric neurologist who will be aware of the latest literature and treatment practices.
| Media file 1:
Optic disc swelling, right eye (Media file 1a) and left eye (Media file 1b), in a child with bilateral optic neuritis. |
 |  View Full Size Image | |
Media type: Image
|
| Media file 2:
Neuroretinitis in the right eye of an adolescent with cat scratch disease. The optic nerve is swollen, and a deposition of yellowish exudate in the nerve fiber layer of the macula produces a macular star. |
 | View Full Size Image | |
Media type: Image
|
| Media file 3:
T1 contrast enhanced axial section of an MRI of the orbital optic nerve of a child with optic neuritis on the left side. The arrows point to the left optic nerve that enhances along its entire orbital course. |
 | View Full Size Image | |
Media type: MRI
|
| Media file 4:
T1 contrast enhanced coronal section of the MRI of the orbital optic nerve, as seen in Media file 3. The arrow points to the enhancing left optic nerve. |
 | View Full Size Image | |
Media type: MRI
|
| Media file 5:
T1 contrast enhanced axial section of an MRI of the intracranial optic nerves. Enhancement of both optic nerves is seen. The arrow indicates the left optic nerve. |
 | View Full Size Image | |
Media type: MRI
|
| Media file 6:
T1 contrast enhanced coronal section of the MRI, as seen in Media file 5, showing the optic nerves that both enhance. The arrow points to the left optic nerve. |
 | View Full Size Image | |
Media type: MRI
|
| Media file 7:
T2 axial section of an MRI through the cerebral hemisphere of a boy with bilateral optic neuritis. Note high-signal abnormalities in the cerebral white matter that are most prominent in the posterior hemispheres. This is suspicious for mild acute disseminated leukoencephalitis. |
 | View Full Size Image | |
Media type: MRI
|
| Media file 8:
T2 axial sections of MRI of a man with optic neuritis showing scattered white matter lesions. The arrows point to 2 examples of the numerous lesions. |
 | View Full Size Image | |
Media type: MRI
|
- Wilejto M, Shroff M, Buncic JR, et al. The clinical features, MRI findings, and outcome of optic neuritis in children. Neurology. Jul 25 2006;67(2):258-62. [Medline].
- Lucchinetti CF, Kiers L, O'Duffy A, et al. Risk factors for developing multiple sclerosis after childhood optic neuritis. Neurology. Nov 1997;49(5):1413-8. [Medline].
- Banwell B, Tremlett H. Coming of age: the use of immunomodulatory therapy in children with multiple sclerosis. Neurology. Mar 8 2005;64(5):778-9. [Medline].
- Beck RW. Optic neuritis. In: Miller NR, Newman NJ, ed. Walsh and Hoyt's Clinical Neuro-ophthalmology. 5th ed. Baltimore: Lippincott Williams & Wilkins; 1998.
- Beck RW, Trobe JD. What we have learned from the Optic Neuritis Treatment Trial. Ophthalmology. Oct 1995;102(10):1504-8. [Medline].
- Bonhomme GR, Liu GT, Balcer LJ. Isolated pediatric optic neuritis. Brain MRI abnormalities and risk of multiple sclerosis. Paper presented at: Annual Meeting of the North American Neuro-ophthalmology Society; 2005.
- Carroll DM, Franklin RM. Leber's idiopathic stellate retinopathy. Am J Ophthalmol. Jan 1982;93(1):96-101. [Medline].
- Dreyer RF, Hopen G, Gass JD, et al. Leber's idiopathic stellate neuroretinitis. Arch Ophthalmol. Aug 1984;102(8):1140-5. [Medline].
- Farris BK, Pickard DJ. Bilateral postinfectious optic neuritis and intravenous steroid therapy in children. Ophthalmology. Mar 1990;97(3):339-45. [Medline].
- Gass JD. Diseases of the optic nerve that may simulate macular disease. Trans Sect Ophthalmol Am Acad Ophthalmol Otolaryngol. Sep-Oct 1977;83(5):763-70. [Medline].
- Hierons R, Lyle TK. Bilateral retrobulbar optic neuritis. Brain. 1959;82:56-67.
- Keast-Butler J, Taylor D. Optic neuropathies in children. Trans Ophthalmol Soc U K. Apr 1980;100:111-8. [Medline].
- Kennedy C, Carroll FD. Optic neuritis in children. Arch Ophthalmol. 1960;73:747-755.
- King MH, Cartwright MJ, Carney MD. Leber's idiopathic stellate neuroretinitis. Ann Ophthalmol. Feb 1991;23(2):58-60. [Medline].
- Koraszewska-Matuszewska B, Samochowiec-Donocik E, Rynkiewicz E. [Optic neuritis in children and adolescents]. Klin Oczna. Jun 1995;97(6):207-10. [Medline].
- Kriss A, Francis DA, Cuendet F, et al. Recovery after optic neuritis in childhood. J Neurol Neurosurg Psychiatry. Oct 1988;51(10):1253-8. [Medline].
- Maitland CG, Miller NR. Neuroretinitis. Arch Ophthalmol. Aug 1984;102(8):1146-50. [Medline].
- Meadows SP. Doyne memorial lecture (1969). Retrobulbar and optic neuritis in childhood and adolescence. Trans Ophthalmol Soc U K. 1970;89:603-38. [Medline].
- Morales DS, Siatkowski RM, Howard CW, et al. Optic neuritis in children. J Pediatr Ophthalmol Strabismus. Sep-Oct 2000;37(5):254-9. [Medline].
- Nakao Y, Omoto T, Shimomura Y. Optic neuritis in children. Folia Ophthalmol Jpn. 1983;34:496-498.
- Neetens A, Smets RM. Leber's neurogenic stellate maculopathy. Neuro Ophthalmol. 1987;7:315-328.
- Pohl D, Rostasy K, Gartner J, et al. Treatment of early onset multiple sclerosis with subcutaneous interferon beta-1a. Neurology. Mar 8 2005;64(5):888-90. [Medline].
- Riikonen R, Donner M, Erkkilä H. Optic neuritis in children and its relationship to multiple sclerosis: a clinical study of 21 children. Dev Med Child Neurol. Jun 1988;30(3):349-59. [Medline].
- Rothermel H, Hedges TR 3rd, Steere AC. Optic neuropathy in children with Lyme disease. Pediatrics. Aug 2001;108(2):477-81. [Medline].
- Rush JA. Idiopathic optic neuritis of childhood. J Pediatr Ophthalmol Strabismus. May-Jun 1981;18(3):39-41. [Medline].
Optic Neuritis, Childhood excerpt Article Last Updated: Aug 29, 2008
|
