Amaurosis fugax (transient monocular blindness; a TIA of the retina) usually occurs from a retinal embolus often arising from ipsilateral carotid stenosis or the heart. Prolonged occlusion of the central retinal artery results in classic fundus appearance of a milky, infarcted retina with cherry red fovea. Any pt with compromise of the retinal circulation should be evaluated promptly for stroke risk factors (e.g., carotid atheroma, heart disease, atrial fibrillation). Occipital cortex lesions can be confused with amaurosis fugax because many pts mistakenly ascribe symptoms to their left or right eye, when in fact they are occurring in the left or right hemifield of both eyes. Interruption of blood flow to the visual cortex causes sudden graying of vision, occasionally with flashing lights or other symptoms that mimic migraine. The history may be the only guide to the correct diagnosis. Pts should be questioned about the precise pattern and duration of visual loss and other neurologic symptoms, especially those of posterior circulation dysfunction such as diplopia, vertigo, numbness, or weakness.
Marked systemic hypertension can cause visual loss from exudates, hemorrhages, cotton-wool spots (focal nerve fiber layer infarcts), and optic disc edema.
In central or branch retinal vein occlusion, the fundus examination reveals engorged, phlebitic veins with extensive retinal hemorrhages.
In age-related macular degeneration, characterized by extensive drusen and scarring of the pigment epithelium, leakage of blood or fluid from subretinal neovascular membranes can produce sudden central visual loss.
Flashing lights and floaters may indicate a fresh vitreous detachment. Separation of the vitreous from the retina is a frequent involutional event in the elderly. It is not harmful unless it creates sufficient traction to produce a retinal detachment. Vitreous hemorrhage may occur in diabetic pts from retinal neovascularization.
Papilledema refers to optic disc edema from raised intracranial pressure. Transient visual obscurations are common, but visual acuity is not affected unless the papilledema is severe, long-standing, or accompanied by macular exudates or hemorrhage. Enlarged blind spots and peripheral constriction are typical. Neuroimaging should be obtained to exclude an intracranial mass. If negative, a LP is required to confirm elevation of the intracranial pressure. Pseudotumor cerebri (idiopathic intracranial hypertension) is a diagnosis of exclusion. Most pts are young, female, and obese; some are found to have occult cerebral venous sinus thrombosis. Treatment is with acetazolamide, repeated LPs, and weight loss (via bariatric surgery if necessary); some pts require lumboperitoneal shunting to prevent blindness.
Optic neuritis is a common cause of monocular optic disc swelling and visual loss. If site of inflammation is retrobulbar, fundus will appear normal on initial examination. The typical pt is female, age 15-45, with pain provoked by eye movements. Glucocorticoids, typically IV methylprednisolone (1 g daily for 3 days) followed by oral prednisone (1 mg/kg daily for 11 days), may hasten recovery in severely affected pts but make no difference in final acuity (measured 6 months after the attack). If an MRI shows multiple demyelinating lesions, treatment for multiple sclerosis (Chap. 190. Multiple Sclerosis) should be considered. Optic neuritis involving both eyes simultaneously or sequentially suggests neuromyelitis optica.
Anterior ischemic optic neuropathy (AION) is an infarction of the optic nerve head due to inadequate perfusion via the posterior ciliary arteries. Pts have sudden visual loss, often noted on awakening, and painless swelling of the optic disc. It is important to differentiate between nonarteritic (idiopathic) AION and arteritic AION. There is no treatment for nonarteritic AION. In contrast, arteritic AION is caused by giant cell (temporal) arteritis and requires immediate glucocorticoid therapy to prevent blindness; temporal artery biopsy establishes the diagnosis. The ESR and C-reactive protein should be checked in any elderly pt with acute optic disc swelling or symptoms suggestive of polymyalgia rheumatica (associated with arteritic AION).
Section 3. Common Patient Presentations