Diagnostic Considerations

Childhood optic neuritis is marked by heterogeneity. It may occur as a monophasic illness, recurrent isolated optic neuritis, and recurrent optic neuritis in the context of multifocal inflammatory CNS disease.

Neuromyelitis optica (Devic disease)

Formal diagnostic criteria for neuromyelitis optica are as follows: absolute criteria and one major supportive criterion or two minor supportive criteria.

Absolute criteria include the following:

Optic neuritis
Acute myelitis
No clinical disease outside the optic nerves and spinal cord

Major supportive criteria include the following:

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 include the following:

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.

Myelin Oligodendrocyte Glycoprotein (MOG-IgG+) Seropositive Demyelinating Disease

Myelin Oligodendrocyte Glycoprotein Serepositive Demyelinating disease (MOG IgG+) is a recently discovered cause of optic neuritis. The disease phenotype associated with MOG seropositivity is still being studied. Patient presentation for optic neuritis has been found to have a bimodal distribution with younger patients less than 9 years of age exhibiting MOG-IgG in association with ADEM and older children with clinically isolated optic neuritis or NMO-SD phenotype. ^{[36, 37]}

Differential Diagnoses

Chronic Relapsing Inflammatory Optic Neuropathy
Craniopharyngioma
Diabetic Papillopathy
Leber Hereditary Optic Neuropathy
Medication Related

eg, nivolumab
Multiple Sclerosis
Neuroretinitis

Due to infectious causes (Bartonella, Syphilis, Lyme, Toxoplasma, Toxocara) or idiopathic causes
NMO Spectrum Disorder
Optic nerve or optic nerve sheath tumor

Due to glioma or meningioma
Papilledema

Due to increased intracranial hypertension (idiopathic intracranial hypertension, meningitis, intracranial mass lesion, hydrocephalus, or venous thrombosis)
Pituitary Adenoma
Systemic Lupus Erythematosus

Workup

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Media Gallery

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.

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

Adult Optic Neuritis	Pediatric Optic Neuritis
Unilateral	Often bilateral in children < 10 years^[6]
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, with the exception of NMO

Table 2. Life-Table Analysis of the Risk for Development of MS in Children With an Isolated Attack of Optic Neuritis ^[9]

Age	Risk for Development of MS
10 years	13%
20 years	19%
30 years	22%
40 years	26%

Table 3. Visual Acuity on Examination in Children with Optic Neuritis

Percentage (%)	Visual Acuity
20	20/40 or better at presentation
20	20/50 - 20/190
60	20/200 or worse

Table 4. 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

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Author

Honey H H Herce, MD Assistant Professor, Departments of Ophthalmology and Pediatrics, Program Director for Pediatric Ophthalmology and Strabismus Fellowship, Baylor College of Medicine, Texas Children's Hospital

Honey H H Herce, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Ophthalmology, American Academy of Pediatrics, American Association for Pediatric Ophthalmology and Strabismus, American Medical Association, Houston Ophthalmological Society, Houston Pediatric Society, Texas Association of Pediatric Ophthalmology & Strabismus, Texas Ophthalmological Association, Women in Ophthalmology, Inc

Disclosure: Nothing to disclose.

Coauthor(s)

Varsha Sivagami Sathappan MD Candidate, Baylor College of Medicine

Disclosure: Nothing to disclose.

Specialty Editor Board

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

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Edsel B Ing, MD, PhD, MBA, MEd, MPH, MA, FRCSC Professor, Department of Ophthalmology and Vision Sciences, Sunnybrook Hospital, University of Toronto Faculty of Medicine; Incoming Chair of Ophthalmology, University of Alberta Faculty of Medicine and Dentistry, Canada

Edsel B Ing, MD, PhD, MBA, MEd, MPH, MA, 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 Medical Association, Canadian Ophthalmological Society, Canadian Society of Oculoplastic Surgery, Chinese Canadian Medical Society, European Society of Ophthalmic Plastic and Reconstructive Surgery, North American Neuro-Ophthalmology Society, Ontario Medical Association, Royal College of Physicians and Surgeons of Canada, Statistical Society of Canada

Disclosure: Nothing to disclose.

Additional Contributors

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, American Ophthalmological Society

Disclosure: Nothing to disclose.

John E Carter, MD Professor, Departments of Neurology and 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, North American Neuro-Ophthalmology Society

Disclosure: Nothing to disclose.

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 for Pediatric Ophthalmology and Strabismus, North American Neuro-Ophthalmology Society, Texas Medical Association

Disclosure: Nothing to disclose.

Acknowledgements

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.