eMedicine Specialties > Ophthalmology > Optic Nerve

Optic Neuritis, Childhood: Differential Diagnoses & Workup

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

Differential Diagnoses

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

Workup

Laboratory 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.

More on Optic Neuritis, Childhood

Overview: Optic Neuritis, Childhood
Differential Diagnoses & Workup: Optic Neuritis, Childhood
Treatment & Medication: Optic Neuritis, Childhood
Follow-up: Optic Neuritis, Childhood
Multimedia: Optic Neuritis, Childhood
References
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References

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  2. 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].

  3. 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].

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

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  6. 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.

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

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

  22. 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].

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Further Reading

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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

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Contributor Information and Disclosures

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, 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.

Medical Editor

Gerhard W Cibis, MD, Clinical Professor, Director of Pediatric Ophthalmology Service, Department of Ophthalmology, University of Kansas, Kansas City
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.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
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

Ralph Garzia, OD, Assistant Dean for Clinical Programs, Associate Professor, School 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.

 
 
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