Childhood Optic Neuritis Clinical Presentation

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.

• 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 shown below), as compared to 35% in adults. Optic disc swelling in the right eye and left eye in a child with bilateral optic neuritis.
  • 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 (as shown below) is associated, a diagnosis of neuroretinitis rather than optic neuritis should be made. 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.

• 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