Childhood Optic Neuritis Workup

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

Approach Considerations

Visual acuity, color vision, afferent pupillary defect (if visual loss is asymmetrical), and confrontation fields should be documented. Perimetry and optical coherence tomography (OCT) of the optic nerve should be performed whenever feasible. Visual evoked potentials may be helpful.

Exclude infectious, genetic, or neoplastic processes.

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Laboratory Studies

Systemic laboratory studies can be performed directed by features in the history and physical examination consistent with other non–immune-mediated causes of optic neuritis.

Neuromyelitis optica (NMO) antibody in serum (anti aquaporin-4 immunoglobulin) helps establish a diagnosis of neuromyelitis optica, although a spectrum of diseases may have this antibody. Testing for myelin oligodendrocyte glycoprotein may be helpful, as the disease course in patients with this spectrum of NMO may be relatively benign.

Myelin Oligodendrocyte Glycoprotein (MOG-IgG+) antibody testing in the serum helps establish a diagnosis of Myelin Oligodendrocyte Glycoprotein (MOG-IgG+) Seropositive Demyelinating Disease.  This can be helpful in both the diagnosis and prognosis of the patient. [36, 37]

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.

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Imaging Studies

MRI of the brain and orbits with contrast should be performed. The MRI should exclude extrinsic compressive lesions.

Enhancement of the optic nerve in the orbit or the intracranial segment of the optic nerve or of the chiasm 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.

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.

The images below depict characteristics relevant to a diagnosis of optic neuritis.

T1 contrast enhanced axial section of an MRI of th 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 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 th 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 sh 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 he 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.

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.

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Other Tests

Optical coherence tomography (OCT) is often used to assess neuronal injury, including retinal nerve fiber layer (RNFL) thickness. Pediatric patients with MS have been reported to have a 10%-20% thinner RNFL layer. [1]

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