Current Neurology and Neuroscience Reports (2021) 21: 35

https://doi.org/10.1007/s11910-021-01122-1

HEADACHE (R.B. HALKER SINGH AND J. VANDERPLUYM, SECTION EDITORS)

Retinal Migraine: Evaluation and Management

Monica E. Maher 1 & William Kingston 1,2,3

Accepted: 22 April 2021 / Published online: 11 May 2021

# The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature 2021

Abstract
Purpose of Review This review aims to summarize the last 15 years of literature and case reports detailing retinal migraine—an
uncommon and somewhat poorly understood migraine variant.
Recent Findings In the last 15 years, only 12 cases of retinal migraine have been outlined. Similar to other migraine statistics,
retinal migraine appears to affect women more so than men and presents with unilateral headache which tends to be ipsilateral to
the side of vision loss. The pathophysiology may relate to vasoconstriction of retinal vessels, as evidenced by ictal fundus
photography in the past few years.
Summary Retinal migraine is a rare entity, with a paucity of described cases in the literature. Retinal migraine is a diagnosis of
exclusion, as monocular vision loss might be ascribed to several concerning disorders requiring urgent diagnosis and treatment,
and any patient presenting as such should be thoroughly investigated. Patients suffering from retinal migraine appear to respond
to typical migraine abortive therapies including NSAIDs and migraine prophylactic medications.

Keywords Retinal migraine . Ophthalmologic migraine . Migraine aura . Visual aura . Monocular vision loss

Introduction migraine is best differentiated from migraine aura by its mon-

A rather contentious clinical entity, retinal migraine was first
described in 1882 by Galezowski [1]. Detailed in the ICHD-3
[2], it primarily involves recurrent, transient attacks of mon-
ocular vision loss associated with or preceding headaches that
otherwise meet criteria for migraine (Fig. 1). As inferred by
the ICHD, retinal migraine is a rather rare cause of monocular
vision loss—and within the literature, there are assertions that
the visual symptoms detailed in case reports of retinal mi-
graine may be better attributed to other etiologies [3, 4], such
as central or branch retinal artery occlusion, ischemic optic
neuropathy, or optic atrophy. Given the number of potentially
emergent and sinister causes of monocular visual loss, retinal
migraine should be considered a diagnosis of exclusion and a
careful evaluation should never be overlooked. Retinal
ocular nature—the underlying pathophysiology of migraine
aura has been demonstrated by functional neuroimaging to
be related to cortical spreading depression of the occipital
lobe, which would instead manifest as binocular visual symp-
toms [3].
Indeed, retinal migraine is a rare enough migraine syn-
drome that there is an appreciable paucity of literature on the
topic. As such, we endeavor here to summarize the last 15
years of primary publications on the topic with the aim of
better characterizing the constellation of symptoms involved
in a presentation of retinal migraine.
Methods

This article is part of the Topical Collection on Headache PubMed and Ovid/Embase databases were searched using the
combination of the following terms: “retinal migraine,”
* William Kingston “retin*” AND “migraine,” “ophthalmologic*” AND “mi-
William.Kingston@sunnybrook.ca graine,” “ophthalmologic migraine.” Initially, only publica-
tions within the last 3 years were included; however, given
1
Division of Neurology, University of Toronto, Toronto, Canada how few results that date range produced, the search was
2
Sunnybrook Health Sciences Centre, A 455 - 2075 Bayview Ave, expanded to include those pieces published within the past
Toronto, ON M4N 3M5, Canada 15 years to present. Review papers and review papers that
3
Centre for Headache, Women’s College Hospital, Toronto, Canada did not contain original case reports were excluded.
35 Page 2 of 4
ICHD-3 Criteria
- Recurrent episodes of monocular vision
disturbances associated with headache
meeting criteria for migraine with aura
o Can include scintillations, scotoma
or blindness
o Must be fully reversible; vision
loss should be confirmed with
clinical examination
o Spread gradually over >5 min and
lasts 5-60 min
o Headache onset at the same time
or within 60 min
- Rare cause of transient monocular vision
loss, therefore necessary to complete
investigations to rule out other potential
etiologies
Fig. 1 ICHD-3 criteria for retinal migraine [2]
Ultimately, 4 case reports and 1 combined review and case
report resulted from this search strategy and were included
below.
Results
A total of 12 cases of retinal migraine were reviewed and are
summarized in Table 1. Between the 5 papers included here, a
total of 12 patient cases were detailed. In terms of basic patient
characteristics, 10 were female and the average age was 39.5.
Eleven of the 12 had a prior history of migraine headache,
with 4 of those patients reporting a previous history of mi-
graine with aura (though this characteristic/lack thereof was
only commented on in 7 individuals). A positive family his-
tory of migraine is present in 4 of the 8 patients for which that
information is available.
The episodes of retinal migraine themselves were docu-
mented to involve complete monocular vision loss in 10 of
the 12 patients included. The duration of the patients’ loss of
vision ranged from less than 5 min [5•] to as long as 2 weeks
[6] in one case. Ten patients reported onset of a headache that
followed or was concurrent with the visual loss and 9 experi-
enced headaches ipsilateral to the side of their vision loss.
Only one patient did not report a headache at any point during
the initial episode, but months later experienced their usual
migraine headache with new blurring of their vision [7].
Of the nine cases which detailed the work-up the patient
underwent for their vision loss, all had MRI and/or MRA
which were normal. Other investigations completed included
EEG, transcranial Dopplers, fundoscopy or fundus photogra-
phy during the attack, and formal visual fields. The two
Curr Neurol Neurosci Rep (2021) 21: 35
patients evaluated with formal visual fields were found to
have incomplete visual field defects [7]—one with an arcuate
defect, the other with a superior quadrantanopia. Both were
found to be reversible. Interestingly, in the patient who report-
ed a 2-week duration of their vision loss, an MRI of the brain
was completely normal, but they were additionally found to
have an RAPD and red desaturation in the absence of reduced
visual acuity or disk pallor [6]. Three case reports utilized ictal
fundus photography in an attempt to better understand the
underlying pathophysiology of retinal migraine [5•, 8, 9]. In
two of these cases, they noted reversible vasoconstriction of
retinal arteries during an attack and a corresponding reduction
in visual acuity [5•, 8].
Treatment for retinal migraine was quite variable between
all the case reports and only 2 publications detailed the phar-
macological therapies prescribed. Most commonly used was
topiramate in 3 cases [6], verapamil in 3 cases [6, 7], or a
tricyclic antidepressant agent (either amitriptyline or nortrip-
tyline) in 3 cases [6, 7]. Though it should be noted that mul-
tiple agents were utilized in 6 of the 8 patients for whom a
treatment regimen was described.
Case Report and Clinical Observations
In our experience in clinical practices, cases are similar to
those reported above. Here we will detail one such case, in-
volving a 41-year-old woman with a history of migraine head-
aches since adolescence—typically associated with changes
in weather and her menstrual cycle—and a maternal family
history of migraine. She first began experiencing right mon-
ocular vision loss at age 36. These episodes were described as
having a gradual onset of blurry vision, progressing to com-
plete “blackout” of the affected eye lasting 3–7 min before
gradually returning to normal. Often, there is an associated
mild, ipsilateral headache and after the visual episode re-
solves, she will become nauseous for 2–3 h and will need to
lie down or sleep. Prior to referral to our clinic several years
after onset, she had been extensively investigated with multi-
ple MRI brains (with and without contrast), carotid Dopplers,
inflammatory/rheumatological blood panels and had under-
gone assessment by a neuro-ophthalmologist. She had previ-
ously tried verapamil for prophylaxis; however, this resulted
in lightheadedness. Ultimately, we found that a regimen of
magnesium citrate 400 mg twice daily, riboflavin 200 mg dai-
ly and melatonin 5 mg QHS with rizatriptan 10 mg as an
acute, abortive migraine therapy was successful in reducing
the frequency and severity of both her retinal migraines and
typical migraine headaches.
Overall, we have seen success in treating patients with
typical migraine preventive therapies. On at least two occa-
sions where the visual symptoms were the most bothersome
and the headache was a more minor complaint, we have seen
Table 1 Twelve cases of retinal migraine
Year Author ID Presentation
2006 Grosberg and 30F Blurred vision of
Solomon the left eye
41F Scotoma affecting
right eye
2006 Grosberg et al. 42F Recurrent monocular
vision loss, beginning
with black spots and
flashing lights
35M Left monocular vision
loss
33F Monocular vision loss
44F Monocular vision loss
18F Monocular vision loss
59F Gray patch/black spots
in left eye, then
persistent vision loss
59F Monocular vision loss
2013 Ota et al. 29F “Twinkling stars” and
“whiteout” of vision
in right eye
2018 El Youssef 58M Multiple episodes <5 min
of left eye vision loss
2020 Lee et al. 26F Monocular
vision loss
Duration VL Recurrence Headache History of migraine Investigations Findings Treatment
1–2 h Y over 3 weeks None Y MRI, formal visual Reversible arcuate Imipramine, morphine
fields defect note
15 min–4 h Y over 3 weeks Y Y MRI brain, visual Superior temporal Verapamil, amitriptyline,
fields quadrant VF defect tizanidine, oxycodone
for severe; butalbital
for moderate
5 min Y Y ipsilateral Y without aura; MRI brain, bloodwork Cover/uncover Nortriptyline 75 mg
family history confirmed complete
monocular vision loss
Curr Neurol Neurosci Rep (2021) 21: 35
5 min Y (1–2/year) Y ipsilateral Y and family history Imaging and clinical Cover/uncover confirmed Flunarizine 10 mg and
migraine with aura examination complete monocular topiramate 100 mg; later
vision loss switched to magnesium
and riboflavin due to
intolerable weight gain
1h Y Y ipsilateral Y with visual aura MRI/MRA, fundoscopy Normal N/A
all performed during
episode
8 h–3 d (entire Y 1–2/week Y ipsilateral Y without aura; Topiramate 300 mg daily
duration of family history to resolution of VL and
migraine) reduced migraine severity
30 min Y over 3 years Y ipsilateral 10-y history of migraine Normal Topiramate 100 mg
with visual/sensory/
motor auras, no
family history
15 min–3 days Y Y ipsilateral Y since age MRI/MRA brain Normal Acute: dexamethasone,
7 with aura + orbits nitroglycerin, ASA, and
verapamil
2 weeks N Y ipsilateral MRI brain normal R RAPD, red desaturation Nortriptyline, verapamil,
but no disk edema/pallor, ASA 325 mg with
relatively preserved VA gastric protection
(20/30OD, 20/25OS)
2–25 min Y 10 year with aura; Fundus recording: narrowing N/A
maternal family of retinal arteries during
history attack; optic disk pallor,
choroid darkening—all
reversible; reduced visual
acuity to light perception
only during attacks
Y over 2 days Y periorbital N MRI, ictal fundus Reversible N/A
photography vasoconstriction of
central retinal
artery/vein and
branches
10–40 min Y Y ipsilateral 4-year history; MRI, MRA normal, Automated static N/A
no family history VEPs, EEG, perimetry: complete
electronystagometry, monocular blindness;
transcranial Dopplers no vasoconstriction
on fundus
photography; all
resolved
Page 3 of 4 35
35 Page 4 of 4
some benefit with daily magnesium citrate in doses in excess
of 400 mg daily. Ultimately, the symptom burden and overall
patient profile are likely the most important factors in choos-
ing therapeutic agents.
Conclusions
Retinal migraine is a rare headache diagnosis of which there is
a dearth of case reports or primary publications. Of those cases
that have been reported in the last 15 years, there are several
commonly shared characteristics across most of the patients
including monocular vision loss, ipsilateral headache, and a
previous history of migraine headache. Similar to migraine
headache as a whole, retinal migraine also appears to affect
women disproportionately more than it does men. However, it
is crucial to be mindful of alternative diagnoses that should be
considered and rule out prior to settling on a diagnosis of
retinal migraine.
Of the cases of retinal migraine that have been reported, many
have undergone thorough neurological investigations, including
extensive neuroimaging of the brain, orbits and cerebral vascu-
lature, fundoscopic examination, formal visual fields, and—in
one case—VEPs and EEG. These investigations largely exclud-
ed alternative diagnoses, leading the clinicians involved to in-
voke a diagnosis of retinal migraine. Unfortunately, given how
few cases have been reported and the variability in the results of
their investigations, it is difficult to ascribe a particular underly-
ing pathophysiology to explain the presentation and subsequent-
ly rationalize an optimal treatment regimen. Particularly as a
number of patients had completely normal examination and
investigations—though several relied on rather subjective assess-
ments such as the cover/uncover test. In clinical practice, it may
be more appropriate to complete formal visual field assessments
in these patients, to better delineate the nature of their visual loss.
Fundoscopic photography would suggest that retinal vasocon-
striction may be important in retinal migraine, which would
perhaps explain why medications such as verapamil have been
used successfully as a treatment. This could also represent vaso-
constriction seen from oligemia in retinal process akin to cortical
spreading depression, but this has not been proven. It would
appear that retinal migraine may simply respond well to usual
migraine prophylactic medications, as evidenced by the success-
ful use of topiramate, amitriptyline, and nortriptyline.
Ultimately, based on the available literature and our clinical
experience, the treatment of retinal migraine should be tai-
lored to the patient much like is done in typical migraine
treatment. The frequency and severity of episodes as well as
the most bothersome symptom should be taken into account
when choosing an agent. There is insufficient data to suggest
that retinal migraine should be treated any differently from
Curr Neurol Neurosci Rep (2021) 21: 35
migraine with aura. Lastly, to avoid misdiagnosis and poten-
tial patient harm, all new presentations of monocular visual
loss should all have an urgent, thorough ophthalmologic ex-
amination before ascribing the symptoms to migraine [10].
Compliance with Ethical Standards
Conflict of Interest The authors declare that they have no conflict of
interest.
Human and Animal Rights This article does not contain any studies
with human or animal subjects performed by any of the authors.
References
Papers of particular interest, published recently, have been
highlighted as:
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