Physical
When examining a patient with a pale disc, nonpathologic causes of optic atrophy must be ruled out (pseudo atrophy), as follows:
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Axial myopia: The optic disc has a segmental whitish appearance due to an oblique angle of insertion of the optic nerve and nasal displacement of the optic nerve contents.
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Myelinated nerve fibers: Feathery margins are due to the superficial location, usually adjacent to the disc.
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Optic nerve pit: Small colobomas are most often located in the inferotemporal portion of the disc.
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Tilted disc leads to confusion.
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Optic nerve hypoplasia is characterized by a small disc and peripapillary double ring sign, and the inner ring is actually the optic disc margin.
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Scleral crescent areas - devoid of retinal pigment epithelium.
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Optic disc drusen
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Fundus viewing through an intraocular lens implant
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Brighter-than-normal luminosity: The luminosity of an indirect ophthalmoscope is approximately 2000 lux and that of a direct ophthalmoscope is up to 900 lux. A disc appears pale if the luminosity of the instrument is brighter than normal.
Optic atrophy in young individuals
Hereditary and congenital optic atrophy generally presents in the first or second decade of life. They can be broadly classified into the following three major groups:
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Optic atrophy with generalized white matter disease (eg, adrenoleukodystrophy)
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Optic atrophy with seemingly unrelated systemic features (generally associated with OPA1 gene mutation)
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Isolated optic atrophy (may be autosomal dominant or recessive mitochondrial inheritance; eg, Leber hereditary optic neuropathy)
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Normal optic nerve histopathology.
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Glaucomatous optic atrophy histopathology.
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Healthy optic disc.
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Nonarteritic anterior ischemic optic neuropathy.
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Arteritic anterior ischemic optic neuropathy, cilioretinal artery occlusion.
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Primary optic atrophy.
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Optic atrophy following papilledema (secondary).
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Glaucomatous optic atrophy.
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Juvenile open-angle glaucoma (JOAG) with optic pallor.
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Consecutive optic atrophy following panretinal photocoagulation (PRP).
Tables
|
Postneuritis |
Ischemic Arteritic |
Ischemic Nonarteritic |
Compressive |
Age |
15-50 y |
Approximately 70 y |
Sixth decade |
Varies based on cause |
Sex |
Multiple sclerosis F>M |
F>M |
F=M |
Varies based on cause |
Visual acuity |
Varies from mild blurring (34%) and moderate loss of acuity (12%) to severe or total loss of light perception (complete blindness) in 54% of cases, to no light perception. The loss of vision is acute and progressive.--Vision usually recovers within 2 mo |
< 20/200 (6/60) |
>20/200 (6/60) |
Varies from mild blurring to no light perception |
Color vision |
Color vision > vision loss |
Color vision loss = vision loss |
Color vision loss = vision loss |
Color vision = vision loss |
RAPD* |
+ |
+ |
+ |
+ |
Motility |
Painful movement in cases of retrobulbar neuritis |
Normal |
Normal |
Depends on the site of compression |
Nystagmus |
In multiple sclerosis, vertical nystagmus (upbeating or downbeating) may be seen |
No |
No |
See-saw nystagmus in optic chiasm compression |
Optic disc |
Temporal pallor |
Pallid disc edema |
Segmental disc edema |
Bow-tie pallor seen in optic chiasm compression; varies in other instances |
Electrophysiologic study |
VEP-increased latency < †> |
VEP-reduced amplitude |
VEP-reduced amplitude |
Reduced VEP amplitude |
Neuroimaging (CT, MRI) |
In multiple sclerosis, hyperechoic lesions are seen in the brain on MRI |
- |
- |
For exact location of compression |
Other associations |
|
Headache, scalp tenderness, jaw claudication |
Hypertension and diabetes |
Headache, vomiting, and focal neurologic deficits |
*RAPD - Relative afferent pupil defect < †>VEP - Visual-evoked potential |
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