Childhood Optic Neuritis Differential Diagnoses

Updated: Nov 02, 2021
  • Author: Honey H H Herce, MD; Chief Editor: Edsel B Ing, MD, PhD, MBA, MEd, MPH, MA, FRCSC  more...
  • Print

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