Amaurosis fugax

Amaurosis fugax (Latin fugax meaning fleeting,

Greek amaurosis meaning darkening, dark, or
obscure) is a painless temporary loss of vision in
one or both eyes.[1]
Signs and symptoms[edit]
The experience of amaurosis fugax is classically
described as a temporary loss of vision in one or
both eyes that appears as a black "curtain
coming down vertically into the field of vision in
one eye;" however, this altitudinal visual loss is
relatively uncommon. In one study, only 23.8
percent of patients with transient monocular vision
loss experienced the classic "curtain" or "shade"
descending over their vision.[2] Other descriptions of
this experience include a monocular blindness,
dimming, fogging, or blurring.[3] Total or sectorial
vision loss typically lasts only a few seconds, but
may last minutes or even hours. Duration depends
on the etiology of the vision loss. Obscured vision
due to papilledema may last only seconds, while a
severely atherosclerotic carotid artery may be
associated with a duration of one to ten minutes.[4]
Certainly, additional symptoms may be present with
the amaurosis fugax, and those findings will depend

on the etiology of the transient monocular vision

loss.
Cause[edit]
Prior to 1990, amaurosis fugax could, "clinically, be
divided into four identifiable symptom complexes,
each with its underlying pathoetiology: embolic,
hypoperfusion, angiospasm, and unknown".[5] In
1990, the causes of amaurosis fugax were better
refined by the Amaurosis Fugax Study Group, which
has defined five distinct classes of transient
monocular blindness based on their supposed
cause: embolic, hemodynamic, ocular, neurologic,
and idiopathic (or "no cause identified") [6]
Concerning the pathology underlying these causes
(except idiopathic), "some of the more frequent
causes include atheromatous disease of the
internal carotid or ophthalmic artery, vasospasm,
optic neuropathies, giant cell arteritis, angleclosure glaucoma, increased intracranial
pressure, orbital compressive disease, a steal
phenomenon, and blood hyperviscosity or
hypercoagulability."[7]
Embolic and hemodynamic origin[edit]

With respect to embolic and hemodynamic causes,

this transient monocular visual loss ultimately
occurs due to a temporary reduction in retinal
artery, ophthalmic artery, or ciliary artery blood
flow, leading to a decrease in retinal circulation
which, in turn, causes retinal hypoxia.[8] While,
most commonly, emboli causing amaurosis fugax
are described as coming from an atherosclerotic
carotid artery, any emboli arising from vasculature
preceding the retinal artery, ophthalmic artery, or
ciliary arteries may cause this transient monocular
blindness.
Atherosclerotic carotid artery: Amaurosis fugax may
present as a type of transient ischemic attack (TIA),
during which an embolus unilaterally obstructs the
lumen of the retinal artery or ophthalmic artery,
causing a decrease in blood flow to the ipsilateral
retina. The most common source of these atheroemboli is an atherosclerotic carotid artery.[9]
However, a severely atherosclerotic carotid
artery may also cause amaurosis fugax due to its
stenosis of blood flow, leading to ischemia when
the retina is exposed to bright light.[10]
"Unilateral visual loss in bright light may
indicate ipsilateral carotid artery occlusive
disease and may reflect the inability of

borderline circulation to sustain the increased

retinal metabolic activity associated with
exposure to bright light."[11]
Atherosclerotic ophthalmic artery: Will present
similarly to an atherosclerotic internal carotid
artery.
Cardiac emboli: Thrombotic emboli arising from the
heart may also cause luminal obstruction of the
retinal, ophthalmic, and/or ciliary arteries,
causing decreased blood flow to the ipsilateral
retina; examples being those arising due to (1) atrial
fibrillation, (2) valvular abnormalities including postrheumatic valvular disease, mitral valve prolapse,
and a bicuspid aortic valve, and (3) atrial myxomas.
Temporary vasospasm leading to decreased blood
flow can be a cause of amaurosis fugax.[12][13]
Generally, these episodes are brief, lasting no longer
than five minutes,[14] and have been associated
with exercise.[8][15] These vasospastic episodes are
not restricted to young and healthy individuals.
"Observations suggest that a systemic hemodynamic
challenge provoke[s] the release of vasospastic
substance in the retinal vasculature of one eye."[14]
Giant cell arteritis: Giant cell arteritis can result
in granulomatous inflammation within the
central retinal artery and posterior ciliary
arteries of eye, resulting in partial or complete

occlusion, leading to decreased blood flow

manifesting as amaurosis fugax. Commonly,
amaurosis fugax caused by giant cell arteritis may
be associated with jaw claudication and headache.
However, it is also not uncommon for these patients
to have no other symptoms.[16] One comprehensive
review found a two to nineteen percent incidence of
amaurosis fugax among these patients.[17]
Systemic lupus erythematosus[18][19]
Periarteritis nodosa[20]
Eosinophilic vasculitis[21]
Hyperviscosity syndrome[22]
Polycythemia[23]
Hypercoagulability[24]
Protein C deficiency[25]
Antiphospholipid antibodies[26]
Anticardiolipin antibodies[27]
Lupus anticoagulant[28][29]
Thrombocytosis[27]
Subclavian steal syndrome
Malignant hypertension can cause ischemia of
the optic nerve head leading to transient
monocular visual loss.[30]

Drug abuse-related intravascular emboli[6]

Iatrogenic: Amaurosis fugax can present as a
complication following carotid endarterectomy,
carotid angiography, cardiac catheterization, and
cardiac bypass.[27]
Ocular origin[edit]
Ocular causes include:
Iritis[31]
Keratitis[22]
Blepharitis[22]
Optic disc drusen[27]
Posterior vitreous detachment[22]
Closed-angle glaucoma[32]
Transient elevation of intraocular pressure[6][31]
Intraocular hemorrhage[6]
Coloboma[27]
Myopia[27]
Orbital hemangioma[33]
Orbital osteoma[34]
Keratoconjunctivitis sicca[27]
Neurologic origin[edit]

Neurological causes include:

Optic neuritis[6]
Compressive optic neuropathies[6][27]
Papilledema: "The underlying mechanism for
visual obscurations in all of these patients appear
to be transient ischemia of the optic nerve head
consequent to increased tissue pressure. Axonal
swelling, intraneural masses, and increased influx of
interstitial fluid may all contribute to increases in
tissue pressure in the optic nerve head. The
consequent reduction in perfusion pressure renders
the small, low-pressure vessels that supply the
optic nerve head vulnerable to compromise. Brief
fluctuations in intracranial or systemic blood
pressure may then result in transient loss of
function in the eyes."[35] Generally, this transient
visual loss is also associated with a headache and
optic disk swelling.
Multiple Sclerosis can cause amaurosis fugax due
to a unilateral conduction block, which is a
result of demyelination and inflammation of the
optic nerve, and "...possibly by defects in
synaptic transmission and putative circulating
blocking factors."[36]
Migraine[37][38][39][40][41][42][43][44]

Pseudotumor cerebri[45]
Intracranial tumor[45]
Psychogenic[22]
Diagnosis[edit]
Despite the temporary nature of the vision loss,
those experiencing amaurosis fugax are usually
advised to consult a physician immediately as it is a
symptom that usually heralds serious vascular
events, including stroke.[46][47] Restated, because
of the brief interval between the transient event and
a stroke or blindness from temporal arteritis, the
workup for transient monocular blindness should be
undertaken without delay. If the patient has no
history of giant cell arteritis, the probability of
vision preservation is high; however, the chance of
a stroke reaches that for a hemispheric TIA.
Therefore, investigation of cardiac disease is
justified.[6]
A diagnostic evaluation should begin with the
patient's history, followed by a physical exam, with
particular importance being paid to the ophthalmic
examination with regards to signs of ocular
ischemia. When investigating amaurosis fugax, an
ophthalmologic consult is absolutely warranted if
available. Several concomitant laboratory tests

should also be ordered to investigate some of the

more common, systemic causes listed above,
including a complete blood count, erythrocyte
sedimentation rate, lipid panel, and blood glucose
level. If a particular etiology is suspected based on
the history and physical, additional relevant labs
should be ordered.[6]
If laboratory tests are abnormal, a systemic disease
process is likely, and, if the ophthalmologic
examination is abnormal, ocular disease is likely.
However, in the event that both of these routes of
investigation yield normal findings or an inadequate
explanation, noninvasive duplex ultrasound
studies are recommended to identify carotid artery
disease. Most episodes of amaurosis fugax are the
result of stenosis of the ipsilateral carotid artery.[48]
With that being the case, researchers investigated
how best to evaluate these episodes of vision loss,
and concluded that for patients ranging from 3674
years old, "...carotid artery duplex scanning
should be performed...as this investigation is more
likely to provide useful information than an
extensive cardiac screening (ECG, Holter 24-hour
monitoring, and precordial echocardiography)."[48]
Additionally, concomitant head CT or MRI imaging

is also recommended to investigate the presence of a

clinically silent cerebral embolism.[6]
If the results of the ultrasound and intracranial
imaging are normal, renewed diagnostic efforts may
be made, during which fluorescein angiography is
an appropriate consideration. However, carotid
angiography is not advisable in the presence of a
normal ultrasound and CT.[49]
Treatment[edit]
If the diagnostic workup reveals a systemic disease
process, directed therapies to treat that underlying
etiology should be initiated. If the amaurosis fugax
is caused by an atherosclerotic lesion, aspirin is
indicated, and a carotid endarterectomy
considered based on the location and grade of
the stenosis. Generally, if the carotid artery is still
patent, the greater the stenosis, the greater the
indication for endarterectomy. "Amaurosis fugax
appears to be a particularly favorable indication
for carotid endarterectomy. Left untreated, this
event carries a high risk of stroke; after carotid
endarterectomy, which has a low operative risk,
there is a very low postoperative stroke rate."[50]
However, the rate of subsequent stroke after

amaurosis is significantly less than after a

hemispheric TIA, therefore there remains debate as
to the precise indications for which a carotid
endarterectomy should be performed. If the full
diagnostic workup is completely normal, patient
observation is recommended.[6]