Childhood Optic Neuritis 

  • Author: Martha P Schatz, MD; Chief Editor: Hampton Roy Sr, MD   more...
 
Updated: Dec 5, 2011
 

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 image below depicts optic disc swelling in a child with bilateral optic neuritis.

Optic disc swelling in the right eye and left eye Optic disc swelling in the right eye and left eye in a child with bilateral optic neuritis.

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.
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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.

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Epidemiology

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.[1]

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 (Open Table in a new window)

Adult Optic NeuritisPediatric Optic Neuritis
UnilateralBilateral
Retrobulbar optic neuritisPapillitis
Commonly associated with pain on eye movementsCommonly associated with headache
Most often idiopathicMost often postinfectious or postimmunization
High probability of recurrent inflammatory demyelinating events in the CNS and a diagnosis of MSLow probability of recurrent demyelinating events and a diagnosis of MS
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Contributor Information and Disclosures
Author

Martha P Schatz, MD  Chief, Pediatric Ophthalmology Service, Clinical Professor of Ophthalmology, University of Texas Health Science Center at San Antonio

Martha P Schatz, MD is a member of the following medical societies: American Academy of Ophthalmology, American Association of Pediatric Ophthalmology & Strabismus (AAPOS), North American Neuro-Ophthalmology Society, and Texas Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

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, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Ophthalmology, and North American Neuro-Ophthalmology Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Gerhard W Cibis, MD  Clinical Professor, Director of Pediatric Ophthalmology Service, Department of Ophthalmology, University of Kansas School of Medicine

Gerhard W Cibis, MD is a member of the following medical societies: American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, and American Ophthalmological Society

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

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.

Ralph Garzia, OD  Assistant Dean for Clinical and Academic Programs, Associate Professor, College of Optometry, University of Missouri at St Louis

Ralph Garzia, OD is a member of the following medical societies: American Academy of Optometry and American Optometric Association

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.

References

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  2. Waldman AT, Stull LB, Galetta SL, Balcer LJ, Liu GT. Pediatric optic neuritis and risk of multiple sclerosis: Meta-analysis of observational studies. J AAPOS. Oct 2011;15(5):441-6. [Medline].

  3. 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].

  4. 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].

  5. 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].

  6. Beck RW. Optic neuritis. In: Miller NR, Newman NJ, ed. Walsh and Hoyt's Clinical Neuro-ophthalmology. 5th ed. Baltimore: Lippincott Williams & Wilkins; 1998.

  7. Beck RW, Trobe JD. What we have learned from the Optic Neuritis Treatment Trial. Ophthalmology. Oct 1995;102(10):1504-8. [Medline].

  8. 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.

  9. Carroll DM, Franklin RM. Leber's idiopathic stellate retinopathy. Am J Ophthalmol. Jan 1982;93(1):96-101. [Medline].

  10. Dreyer RF, Hopen G, Gass JD, et al. Leber's idiopathic stellate neuroretinitis. Arch Ophthalmol. Aug 1984;102(8):1140-5. [Medline].

  11. Farris BK, Pickard DJ. Bilateral postinfectious optic neuritis and intravenous steroid therapy in children. Ophthalmology. Mar 1990;97(3):339-45. [Medline].

  12. 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].

  13. Hierons R, Lyle TK. Bilateral retrobulbar optic neuritis. Brain. 1959;82:56-67.

  14. Keast-Butler J, Taylor D. Optic neuropathies in children. Trans Ophthalmol Soc U K. Apr 1980;100:111-8. [Medline].

  15. Kennedy C, Carroll FD. Optic neuritis in children. Arch Ophthalmol. 1960;73:747-755.

  16. King MH, Cartwright MJ, Carney MD. Leber's idiopathic stellate neuroretinitis. Ann Ophthalmol. Feb 1991;23(2):58-60. [Medline].

  17. Koraszewska-Matuszewska B, Samochowiec-Donocik E, Rynkiewicz E. [Optic neuritis in children and adolescents]. Klin Oczna. Jun 1995;97(6):207-10. [Medline].

  18. 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].

  19. Maitland CG, Miller NR. Neuroretinitis. Arch Ophthalmol. Aug 1984;102(8):1146-50. [Medline].

  20. Meadows SP. Doyne memorial lecture (1969). Retrobulbar and optic neuritis in childhood and adolescence. Trans Ophthalmol Soc U K. 1970;89:603-38. [Medline].

  21. Morales DS, Siatkowski RM, Howard CW, et al. Optic neuritis in children. J Pediatr Ophthalmol Strabismus. Sep-Oct 2000;37(5):254-9. [Medline].

  22. Nakao Y, Omoto T, Shimomura Y. Optic neuritis in children. Folia Ophthalmol Jpn. 1983;34:496-498.

  23. Neetens A, Smets RM. Leber's neurogenic stellate maculopathy. Neuro Ophthalmol. 1987;7:315-328.

  24. 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].

  25. 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].

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Optic disc swelling in the right eye and left eye in a child with bilateral optic neuritis.
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.
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.
T1 contrast enhanced coronal section of the MRI of the orbital optic nerve. The arrow points to the enhancing left optic nerve.
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.
T1 contrast enhanced coronal section of the MRI showing the optic nerves that both enhance. The arrow points to the left optic nerve.
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.
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.
Table 1. Comparison of Features of Optic Neuritis in Adults and Children
Adult Optic NeuritisPediatric Optic Neuritis
UnilateralBilateral
Retrobulbar optic neuritisPapillitis
Commonly associated with pain on eye movementsCommonly associated with headache
Most often idiopathicMost often postinfectious or postimmunization
High probability of recurrent inflammatory demyelinating events in the CNS and a diagnosis of MSLow probability of recurrent demyelinating events and a diagnosis of MS
Table 2. Equivalent Doses of Commonly Used Corticosteroid Medications
Corticosteroid DrugApproximate Equivalent Dose
Prednisone5 mg
Prednisolone5 mg
Methylprednisolone4 mg
Dexamethasone0.75 mg
Table 3. Life-Table Analysis of the Risk for Development of MS in Children With an Isolated Attack of Optic Neuritis[4]
AgeRisk for Development of MS
10 years13%
20 years19%
30 years22%
40 years26%
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