Before embarking on expensive ancillary testing, it is crucial for the neur
ologist to distinguish visual loss due to optic nerve dysfunction from othe
r causes of visual loss, This can usually be accomplished based on specific
features of the history and bedside examination. Once it has been establis
hed that a patient has some form of optic neuropathy, several clinical feat
ures are helpful in determining the etiology. The most important of these i
s the time course. Other factors include presence or absence of pain, patte
rn of visual loss (particularly visual field defects), and funduscopic appe
arance. In most cases, by using this information it is possible to differen
tiate among the common forms of optic neuropathy: papilledema, ischemic opt
ic neuropathy, optic neuritis, compressive lesions, toxic/nutritional defic
iencies, and hereditary forms. This article also reviews recent information
concerning the evaluation and treatment of optic neuritis, how to recogniz
e conditions that mimic optic neuritis (e.g., neuroretinitis, papillophlebi
tis), distinguishing arteritic from nonarteritic AION, and new developments
in the genetics of Leber's Hereditary Optic Neuropathy. There is also a di
scussion of various forms of toxic/nutritional visual loss including Cuban
Epidemic Optic Neuropathy and visual loss due to commonly prescribed medica
tions.