INDUCIBLE TRANSIENT CENTRAL

RETINAL ARTERY VASOSPASM:

A CASE REPORT
Aleksey Mishulin, MD, Sachin Ghandi, BA, Daniel Apple, MD, Xihui Lin, MD,
Jonathan Hu, MD, Gary W. Abrams, MD

Purpose: To report a case of inducible transient central retinal artery vasospasm with
associated imaging.
Methods: Observational case report.
Results: A 51-year-old man presented for outpatient follow-up for recurrent inducible
transient vision loss in his right eye. He experienced an episode during examination and was
found to have central retinal artery vasospasm. Fundus photography and fluorescein
angiography obtained during his vasospastic attack confirmed retinal arterial vasospasm.
Treatment with a calcium-channel blocker (nifedipine) has been effective in preventing recurrent
attacks.
Conclusion: Idiopathic primary vasospasm is a rare cause of transient vision loss that is
difficult to confirm because of the transient nature. We obtained imaging showing the
initiation and resolution of the vasospastic event. The patient was then successfully treated
with a calcium-channel blocker.
RETINAL CASES & BRIEF REPORTS 0:1–3, 2017

From the Department of Ophthalmology, Kresge Eye unilateral retinal arterial vasospasm induced by phys-
Institute, Wayne State University, Detroit, Michigan. ical and emotional stimuli, in which we obtained
confirmatory fundus photos and fluorescein angiogra-

P rimary retinal arterial vasospasm is a rare condi-

tion that is difficult to confirm because of its tran-
sient nature. The concept of vasospasm to explain
phy (FA) during an attack.

temporary blindness was coined as “retinal epilepsy” by Case Report

Hughlings Jackson in 1864.1 Primary vasospasm occurs
without an underlying systemic disease, whereas sec-
ondary vasospasm is associated most commonly with
multiple sclerosis, lupus erythematosus, and anti-
phospholipid syndrome.2
There have been previous reports of retinal arterial
spasm associated with eosinophilic vasculitis, mi-
graines, and atheromatous plaques of the carotid
artery. However, imaging of the vasospastic events
has very rarely been captured secondary to the
transient nature of the event. We describe a case of
G. Abrams was a consultant to and received stock options from
RetroSense Therapeutics, LLC before its purchase by Allergan,
LLC. The remaining authors have no any financial/conflicting
interests to disclose.
Reprint requests: Gary W. Abrams, MD, 4717 Street, Antoine,
Detroit, MI 48201; e-mail: gabrams@med.wayne.edu
A 51-year-old African American male presented for outpatient
ophthalmology follow-up after an inpatient admission for recurrent
inducible amaurosis fugax in his right eye. His medical history was
significant for hypertension. He had no known history of any
cardiovascular event.
He complained of multiple episodes of monocular blindness in
his right eye induced by periods of emotional stress (embarrass-
ment, excitement, and anxiety), physical exertion, and brief
changes in head position. The episodes were described as a gradual
“whiting out” of his vision over several seconds with only an island
of vision remaining in his superior temporal periphery. The
episodes lasted for a variable period of time with his vision
gradually returning over the span of several seconds. Most episodes
resolved within 10 minutes; the longest lasted 45 minutes. He had
an inpatient workup 1 week before presentation with unremarkable
computerized tomography of the head, computerized tomographic
angiography of the head and neck, carotid duplex scan, and
echocardiogram. Magnetic resonance imaging of his brain
showed evidence of an old small thalamic hemorrhage and
several old lacunar infarcts but no acute events. After his

Copyright ª by Ophthalmic Communications Society, Inc. Unauthorized reproduction of this article is prohibited.
 2 RETINAL CASES & BRIEF REPORTS´  2017  VOLUME 0  NUMBER 0
hospital stay, the episodes became less frequent and with shorter
duration, occurring 4-5 times per week and lasting no more than
5 minutes.
Both anterior segment and fundus examination were normal. He
also had normal macular optical coherence tomography and 81-2
Humphrey visual field. In the course of the examination, the patient
experienced an episode of transient vision loss (TVL) in the right eye
that lasted approximately 2 minutes. Before vision loss, he had an
uncorrected visual acuity of 20/20 bilaterally, and during the episode,
his uncorrected visual acuity decreased to hand motion in the right
eye’s remaining temporal island of vision. During the episode, the
central retinal artery in the right eye appeared markedly constricted,
with associated ischemic retinal whitening and a mild cherry-red spot.
The vasculature was observed to gradually refill before the patient
regaining his vision. After this event, the patient was then asked to
perform push-ups, an exercise that he reported would consistently elicit
TVL. Immediate examination after exercise was noteworthy for
spontaneous attenuation and blanching of his retinal vessels just before
vision loss (Figure 1D). Intraocular pressure and blood pressure were
measured before, during, and after the induced vasospastic events with
no significant correlation at any point of measurement. Intraocular
pressure remained between 19 and 21 mmHg, and blood pressure was
found to fluctuate from baseline of 138/85 before push-ups and
symptoms, to 161/97 immediately after the onset of vision loss, and
decreased to 148/92 after resolution of the episode. The patient
denied any systemic symptom during each event aside from the
vision loss. Subsequent episodes were captured on serial fundus
photography and FA, each time using push-ups to elicit his TVL
(Figures 1 and 2). Of note was constriction of the retinal arteries
with stasis and segmentation of flow and irregular hypofluor-
escence of the inferior choroid, possibly indicating vasospasm
of the ophthalmic artery and/or ciliary arteries.
We started the patient on nifedipine, a calcium-channel blocker,
60 mg daily for 2 weeks, but this dose was unsuccessful in
managing his vision loss. The dose was increased to 120 mg daily,
but he experienced dizziness and orthostatic hypotension. Ulti-
mately, a dose of 80 mg daily was successful in preventing vision
loss while avoiding negative side effects.
Discussion
Amaurosis fugax has been reported as a presenting
symptom in many conditions including thromboembolic
events with embolization or thrombosis of the ophthalmic
or central retinal artery, obstruction of the carotid artery,
transient ischemic attacks (TIAs), and inflammatory
conditions such as giant-cell arteritis, migraine head-
Fig. 1. Fundus photographs of the right eye. A. Normal-appearing fundus photograph before induced vasospasm. B. Immediately after induction of
vasospasm: note the constriction of the central retinal artery and the arteries in the vascular arcades. C. Severe attenuation of the arteries and arterioles
continues with marked segmentation of blood flow and mild whitening of the nerve fiber layer. D. Relaxation of central retinal artery and the vascular
arcades 1 minute after onset of symptoms. E. Relaxation of the macular arteries with re-establishment of normal blood flow creating a normal-appearing
fundus.
Copyright ª by Ophthalmic Communications Society, Inc. Unauthorized reproduction of this article is prohibited.
aches, and idiopathic vasospasm. Initial workup for
patients presenting with transient monocular vision loss
should include bilateral carotid ultrasound, echocardio-
gram, and laboratory evaluation that includes erythrocyte
sedimentation rate (ESR) and C-reactive protein (CRP).
Vasospasm is a possible mechanism for TVL in any of
the above diseases. Vasospasm can be triggered by
inflammation or platelet aggregation, potentially giving
a mixed mechanism for TVL in patients with atheroscle-
rotic and vasculitic disease.3,4 Because some of the
conditions in the differential diagnosis of TVL require
emergent management, migraine headaches, and idio-
pathic vasospasm should be considered diagnoses of
exclusion.5 Emotional stress, cold, and exercise have all
been shown to precipitate vasospasm-induced blindness.6
Without confirmation by imaging or ophthalmologic
examination during an episode of TVL, idiopathic
vasospasm is a presumptive diagnosis of exclusion.
Logistically, it has proven very difficult to have
patients undergo such imaging, and there have been
only rare examples of successful imaging by FA
confirming the diagnosis of central retinal artery
vasospasm.7,8 Our patient’s imaging was similar to
previous reports: there was constriction of the arteries
to the point where they appeared nearly empty, with
stasis and segmentation of dye in the veins during the
vasospastic event (Figures 1 and 2).
As in our patient, calcium-channel blockers (CCBs)
have shown efficacy in relieving idiopathic central
retinal artery vasospasm.9 A dose-related response has
been observed, with successful management reported
by starting with a low dose and titrating up until
symptoms resolve. Attacks of monocular vision loss
should become less frequent and eventually subside as
the appropriate dose is reached.9
Primary retinal artery vasospasm is generally con-
sidered a benign condition; however, there have been
reported cases with serious complications. Tomsak
et al reported 24 patients with recurrent transient
monocular blindness secondary to vasospastic events
where one patient developed permanent monocular
 INDUCIBLE CRA VASOSPASM 3

Fig. 2. Fluorescein angiography of the right eye. A. Normal-appearing arteriovenous phase FA before induced vasospasm. B. Immediately after
induction of vasospasm, the central retinal artery becomes constricted, and the inferior choroid is irregularly hypofluorescent possibly representing
constriction of the ophthalmic artery and/or ciliary arteries. C. Stasis and segmentation of dye in the vascular arcades, as the vessels constrict. D.
Relaxation of the central retinal artery. E. Refilling of arteries and veins. Note the re-establishment of laminar flow through the veins. F. Normal-
appearing recirculation phase FA after relaxation of vasospasm.

blindness believed to be secondary to anterior ische-
mic optic neuropathy that occurred during a vasospas-
tic event.5 Vision loss from vasospasm of the
ophthalmic and ciliary arteries has been reported in
one patient with confirmation by cerebral angiography,
however, no FA imaging was obtained and this patient
suffered permanent vision loss from vasospasm refrac-
tory to CCBs.10
In summary, we captured photographic evidence
of idiopathic vasospasm of the central retinal artery
in a patient with monocular TVL, and successfully
treated him with a calcium-channel blocker. Addi-
tional studies are needed to elucidate the etiology of
idiopathic vasospasm and provide more reliable
testing modalities to secure the diagnosis for this
treatable condition.
Key words: amaurosis fugax, fluorescein angiogra-
phy, transient vision loss, vasospasm.
References
1. Humphrey WT. Central retinal artery spasm. Ann Ophthalmol
1979;11:877–881.
2. Abdul-rahman AM, Gilhotra JS, Selva D. Dynamic focal
retinal arteriolar vasospasm in migraine. Indian J Ophthalmol
2011;59:51–53.
3. Faraci FM, Williams JK, Breese KR, et al. Atherosclerosis
potentiates constrictor responses of cerebral and ocular blood
vessels to thromboxane in monkeys. Stroke 1989;20:242–247.
4. Williams JK, Baumbach GL, Armstrong ML, Heistad DD.
Hypothesis: vasoconstriction contributes to amaurosis fugax. J
Cereb Blood Flow Metab 1989;9:111–116.
5. Tomsak RL, Jergens PB. Benign recurrent transient monocular
blindness: a possible variant of acephalgic migraine. Headache
1987;27:66–69.
6. Flammer J, Pache M, Resink T. Vasospasm, its role in the
pathogenesis of diseases with particular reference to the eye.
Prog Retin Eye Res 2001;20:319–349.
7. Weinberg DV, Covert DJ. Amaurosis fugax captured during
fluorescein angiography. Retina 2015;35:2669–2671.
8. Kosmorsky GS. Angiographically documented transient mon-
ocular blindness: retinal migraine? Br J Ophthalmol
2013;97:1604–1606, 1615.
9. Winterkorn JM, Kupersmith MJ, Wirtschafter JD, Forman S.
Brief report: treatment of vasospastic amaurosis fugax with
calcium-channel blockers. N Engl J Med 1993;329:396–398.
10. Mosso M, Jung HH, Baumgartner RW. Recurrent spontaneous
vasospasm of cervical carotid, ophthalmic and retinal arteries
causing repeated retinal infarcts: a case report. Cerebrovasc Dis
2007;24:381–384.

Copyright ª by Ophthalmic Communications Society, Inc. Unauthorized reproduction of this article is prohibited.