Nutritional & Toxic Optic Neuropathies

The usual clinical features of nutritional or toxic optic neuropathy are subacute, progressive,
symmetrical visual loss, with central field defects (Figure 1420), poor color vision, and the
development of temporal disk pallor (Figure 146).

Figure 1420.

Nutritional amblyopia showing centrocecal scotoma. VA = 20/200.

Vitamin Deficiency

Optic nerve involvement is relatively uncommon in vitamin B12 deficiency, but it may be the first
manifestation of pernicious anemia. Thiamin (vitamin B1) deficiency is generally a feature of
severe malnutrition, and, as discussed below, there is an overlap with tobacco-alcohol amblyopia.
Folate deficiency alone is a rare cause of optic neuropathy.

Tobacco-Alcohol Amblyopia

Nutritional amblyopia is probably a more accurate term for this entity. Usually it occurs in
individuals with poor dietary habits, with heavy alcohol consumption and heavy smoking often
being associated. Strict vegetarianism without vitamin supplementation may contribute. Much
consideration has been given to other toxic causes, such as cyanide from tobacco producing low
vitamin stores and low levels of sulfur-containing amino acids, but experimental studies with
cyanide in primates have not confirmed this theory. Drug-induced optic neuropathy must be
excluded. Leber's hereditary optic neuropathy, pernicious anemia, methanol poisoning,
retrobulbar neuritis, or macular disease may cause diagnostic confusion.

There is bilateral loss of central vision, generally reducing visual acuity to less than 20/200, but it
can be asymmetric. Central visual fields reveal scotomas that nearly always include both fixation
and the blind spot (centrocecal scotoma) (Figure 1420).

Adequate diet plus thiamine, folic acid, and vitamin B12 supplements are nearly always effective
if presentation is not delayed. Withdrawal of tobacco and alcohol is advisable and may hasten the
cure, but innumerable cases are known in which adequate nutrition or vitamin B12 supplements
were effective despite continued excessive intake of alcohol or tobacco. Improvement usually
begins within 12 months, although in occasional cases, significant improvement may not occur
for 1 year. Visual function can but may not return to normal; permanent optic atrophy or at least
temporal disk pallor can occur depending on the stage of disease at the time treatment was
started (Figure 146). Loss of the ganglion cells of the macula and destruction of myelinated
fibers of the optic nerveand sometimes of the chiasm as wellare the main histologic
changes.

Heavy Metal Poisoning

Chronic lead exposure, thallium (present in depilatory cream), or arsenic poisoning can produce
a toxic effect on the optic nerve.

Drug-Induced Optic Neuropathy

Ethambutol, isoniazid (INH), rifampin, disulfiram, linezolid, and tamoxifen can all cause optic
neuropathy, which usually improves with prompt cessation of the drug with or without
nutritional supplements. Quinine overdose produces optic neuropathy with narrowed retinal
arterioles and irregular, poorly reactive pupils. Chloramphenicol in high doses causes optic
neuropathy. Amiodarone has been associated with bilateral optic neuropathy with chronic disk
swelling, but the relationship is not necessarily causal. It characteristically also induces a
verticillate keratopathy.

Chemical-Induced Optic Neuropathy: Methanol Poisoning

Methanol is used widely in the chemical industry as antifreeze, solvent varnish, or paint remover;
it is also present in fumes of some industrial solvents such as those used in old photocopier
machines. Significant systemic absorption can occur from fumes inhaled in a room with
inadequate ventilation and (rarely) can be absorbed through the skin.

Clinical Findings

Visual impairment can be the first sign and begins with mild blurring of vision that progresses to
contraction of visual fields and sometimes to complete blindness. The field defects are quite
extensive and nearly always include the centrocecal area.

Hyperemia of the disk is the first ophthalmoscopic finding. Within the first 2 days, a whitish,
striated edema of the disk margins and nearby retina appears. Disk edema can last up to 2 months
and is followed by optic atrophy of mild to severe degree.

Decreased pupillary response to light occurs in proportion to the amount of visual loss. In severe
cases, the pupils become dilated and fixed. Extraocular muscle palsies and ptosis may also occur.

Treatment

Treatment consists of correction of the acidosis with intravenous sodium bicarbonate and oral or
intravenous administration of ethanol to compete with and thus prevent the slower metabolism of
methanol into its byproducts. Hemodialysis is indicated for blood methanol levels over 50 mg/dL