What is Retinal Migraine? 2024
The retina is the back part of the eye, where there are blood vessels, the retinal tissue which contains light sensitive rods and cones, the central circular spot where the optic nerve attaches, and the fovea which is the spot for fine central vision, such as needed for reading newsprint.
Migraine is a neurologic, genetic medical problem affecting 12% of the world’s population. 25% of women and 6% of men have migraine. Migraine is one of the top ten disabling conditions. 30% of headaches are migraine.
Colored zigzag image starting at a and ending after minutes at c.
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Migraine attacks last 4-72 hours, are moderate to severe in intensity, may be one sided, are throbbing, and associated with sensitivity to light and sound. Most patients with a migraine attack are in bed and are disabled for working or being up. Migraine with aura is about 30% of migraine attacks.
Retinal Migraine has been also erroneously termed “ocular Migraine or opthalmic migraine” terms not listed in ICHD. But retinal migraine is rare and controversial, the migraine attacks could be regular migraine with aura attacks occurring in relatively poorly observant patients.
This is an article by Britt Talley Daniel MD, member of the American Academy of Neurology, the American Headache Society, migraine textbook author, podcaster, YouTube video producer, and blogger.
Retinal Migraine implies a monocular migraine aura image occurring only in one eye and a typical migraine headache at the same time. Migraine with aura attacks have visual images occurring in both eyes; for example the person would see the image in the temporal part of the right eye and in the nasal part of the left eye.
The person should also have a previous history of migraine with aura, which requires at least 5 events to secure the diagnosis.
Some patients who complain of monocular visual disturbance in fact have hemianopia (visual loss in the same part of both the right and left eye, such as right eye nasal loss, left eye temporal loss.) Hemianopia originates in the back of the brain in the occipital lobe. Some cases without headache have been reported, but migraine as the underlying aetiology in those cases cannot be ascertained.
Retinal migraine is a migraine visual aura occurring just in one eye, or in what may be referred to as a “monocular” distribution rather than the usual bilateral, homonymous pattern.
In a homonymous pattern the carefully observant and carefully instructed patient may notice the visual aura in, for example, the left halves of both eyes. For gazing left the image would be in the right eye nasal field, and the left eye temporal field. The visual aura should be followed by a typical migraine without aura headache.
The International Classification of Headache Disorders (ICHD) 3 describes retinal migraine as consisting of repeated attacks of monocular visual disturbance which may come with scintillations, scotomas, or blindness, and at the same time as a migraine headache.
ICHD 3 diagnostic criteria for Retinal migraine are:
A. At least two attacks fulfilling criteria B and C
B. Aura consisting of fully reversible monocular positive and/or negative visual phenomena (e.g., scintillations, scotomata or blindness) confirmed during an attack by either or both of the following:
1. clinical visual field examination
2. the patient’s drawing (made after clear instruction) of a monocular field defect
C. At least two of the following three characteristics
1. the aura spreads gradually over ≥5 min
2. aura symptoms last 5-60 min
3. the aura is accompanied, or followed within 60 min, by headache
D. Not better accounted for by another ICHD-3 diagnosis, and other causes of amaurosis fugax have been excluded.
Path of visual images from eye to back of brain.
Comments:
Some patients who complain of monocular visual disturbance in fact have hemianopia (visual loss in the same part of both the right and left eye, such as right eye nasal loss, left eye temporal loss.) Hemianopia originates in the back of the brain in the occipital lobe. Some cases without headache have been reported, but migraine as the underlying aetiology in those cases cannot be ascertained.
Retinal migraine is an extremely rare cause of transient monocular visual loss. Cases of permanent monocular visual loss associated with migraine have been described. Appropriate investigations are required to exclude other causes of transient monocular blindness.
This is a migraine visual aura occurring just in one eye, or in what may be referred to as a “monocular” distribution rather than the usual bilateral, homonymous pattern.
According to the theory of Leao, a spreading wave of electrical depolarization moving across one side of the occipital brain is what causes the migraine with aura patient to see the scintillating, zig-zag pattern in both halves of the visual fields. In a homonymous pattern the carefully observant and carefully instructed patient may notice the visual aura in, for example, the right halves of both eyes. This would comprise the right eye nasal field, and the left eye temporal field. The visual aura should be followed by a typical migraine without aura headache.
Every neurologist knows that patients best note the images in the dominant field (in the example given above the dominant field would be the right eye) and only on close questioning may the patient be able to admit seeing the spectral image in the non-dominant field (in the example given the non-dominant field would be the image in the right part of the left eye.)
Sometimes the patient may be asked if they saw the image with both eyes closed and this memory may help them declare the occurrence of the non-dominant field. Examiners who see patients during the attacks commonly have the patient do the alternate-eye-covering test to differentiate a homonymous from a monocular pattern although Spierings has stated that he never found this test to be helpful.
If a properly educated patient can draw what they saw and the image is just in one eye and followed by a migraine headache, then this is what is termed retinal migraine.
Images in just one eye bring up the possibility of some other medical problem causing the symptoms than migraine.
Zigzag migraine aura image
Neurologic Workup:
Exclusion of a possible embolic source should be provided by diagnostic testing with EKG, echocardiogram, carotid duplex scanning, MRA scanning, CAT scan or MRI brain scan, and angiography.
Diagnosing retinal migraine
An ophthalmologic exam with an ophthalmoscope during an attack, may show decreased blood flow to the eye. In this case the ophthalmologist may be able to make a confident diagnosis of retinal migraine.
However, as attacks are usually brief it's more likely the eye exam will be normal, and the patient will be diagnosed based on an account of symptoms.
Common other terms used to describe Retinal Migraine:
This syndrome is most appropriately called "retinal migraine," but has also be referred to as:
"anterior visual pathway migraine"
“opthalmic migraine”
"monocular migraine"
"ocular migraine"
"retinal vasospasm"
"transient monocular visual loss"
"retinal spreading depression"
Retinal Migraine is commonly mislabeled as “ocular migraine” but ocular migraine is not mentioned in ICHD3.
Description of visual images with Retinal Migraine:
1.positive visual phenomena
flashing rays of light
zigzag lightning patterns
perceptions of bright colored streaks
halos or diagonal lines
2.negative visual losses
include blurring
blank areas
black dots or spots in the field of vision.
Most common patient population
Women in their 20’s or 30’s who have migraine with aura.
Differential diagnoses:
amaurosis fugax from embolic carotid disease or optic neuropathy
vasculitis
hypercoagulable states
illicit drug use
rheumatologic disorders
Possible aggravating features:
stress
smoking
high blood pressure
oral contraceptive pill
exercise
bending over
dehydration
high altitude
low blood sugar
excessive heat
Treatment of Retinal Migraine.
There is currently insufficient clinical information to support recommendations for preventative or acute treatment for this entity.
Therapy of the acute attack of retinal migraine should not include triptans or ergots because of their vasconstrictive properties.
Oral contraceptive pills should not be used. All patients should be advised to stop smoking.
Prophylactic medications that have been tried with anecdotal benefit include calcium-channel blockers, antidepressants amitriptyline and nortriptyline, propranolol, and valproic acid or topiramate.
There is currently no data on the use of Gepant acute therapy migraine drugs, or CGRP preventive drugs such as Aimovig, Ajovy, or Emgality for treatment of retinal migraine.
Aspirin should be given because of its antiplatelet, antistroke activity.
Prognosis
Although retinal migraine is considered a benign condition, very rarely permanent symptoms can persist after the acute attacks.
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Case Report
1.Evans and Grosberg writing in Headache in 2008;48:142-145 on “Expert Opinion: Retinal Migraine: Migraine Associated With Monocular Visual Symptoms,” presented a case and discussed pathophysiology, work up, and treatment. Their case summary follows:
This 25-year-old man reports a 12-year history of similar headaches occurring about one or 2 times monthly. He develops a left or right temple throbbing which is mild at first and later becomes a 10/10 associated with nausea, vomiting, light and noise sensitivity. About 30 minutes after the onset of all of the headaches, he develops sudden total darkness where he cannot see in the eye contralateral to the headache lasting about 4 hours. The headache is severe for about 5 hours and then mild for 24 to 36 hours. Aspirin or acetaminophen is of mild help. He tries to go to bed. He had never seen a physician for the headaches before. Past medical history was negative. There was no family history of migraine. Neurological examination was normal.
Evans and Grosberg stated:
The most likely cause of recurrent stereotypical episodes of transient monocular visual loss in association with headaches is retinal migraine. Secondary causes of transient monocular visual loss are less likely to be found in cases that have been recurring for a long period of time.
Evans and Grosberg felt that the true occurrence of retinal migraine was unknown but that it was a rare entity. The type of visual disturbance noted may be positive and/or negative visual disturbances within one eye associated with migraine headache.
Regarding pathophysiology, Evans and Grosberg said:
The underlying pathophysiology of retinal migraine remains largely unknown. In some cases, vasospasm of the retinal or ciliary circulation may have caused retinal or optic nerve ischemia; this may explain the amaurosis and rare funduscopic findings during acute attacks of retinal migraine. An alternative theory is spreading depression of retinal neurons, a phenomenon that has been demonstrated in the chick retina. Similarly, it is possible that those rare cases with prolonged monocular defects associated with migraine headache could have a mechanism similar to that seen in the cerebral cortex of migraineurs who have persistent aura without infarction.
Evans and Grosberg stressed that prolonged and permanent monocular visual loss was more common in retinal migraine than in cases of prolonged aura or migrainous infarction in patients with conventional migraine. Thus, retinal migraine carries a worse prognosis, a situation provoking consideration of pharmacological treatment.
2.Brian M. Grosberg, M.D.
Assistant Professor of Neurology, Albert Einstein College of Medicine, and Director of the Inpatient Headache Program, Montefiore Headache Center, Bronx, NY, From the National Headache Foundation.
THE CASE
A 42-year-old woman with migraine was referred by her eye doctor to a headache clinic because she was having recurrent episodes of visual loss in one eye. Her migraine headaches were severe, pulsating and left-sided. Associated features included nausea, vomiting and increased sensitivity to lights, sounds and odors. Her headaches occurred approximately six times monthly and lasted 24 to 72 hours.
One-third of her headaches began with transient spells of visual loss in the left eye consisting of black spots and flashing lights. The visual phenomena always began in the outer edge of the woman’s left eye and expanded to engulf the entire eye within a few minutes. Alternately covering each eye during an attack and comparing their views confirmed that the visual disturbances were confined to the left eye. Complete visual loss in the left eye lasted for five minutes, then fully resolved, and was followed immediately by a migraine headache.
The woman’s general medical and neurological examinations were normal, as were repeated eye examinations by several ophthalmologists. Other tests, which included an MRI of the brain, ultrasound examination of the carotid arteries, echocardiography and extensive blood testing, were within normal limits. The patient was treated with a gradually escalated dose of nortriptyline, a medication used for headache prevention. The episodes of visual loss completely stopped, and the woman experienced a significant reduction in headaches (down to one per month).
DISCUSSION
The features in this case suggest a diagnosis of retinal migraine, as long as other causes of visual loss involving one eye are excluded. Other terms that have been used for this condition include ophthalmic migraine, ocular migraine and anterior visual pathway migraine.
Retinal migraine is thought be a rare entity, but its true occurrence is unknown. Retinal migraine is most common in women with a history of migraine with aura who are in their twenties and thirties. It is characterized by episodes of fully reversible positive and/or negative visual disturbances within one eye, associated with migraine headache. Typical descriptions of positive visual phenomena include flashing rays of light, zigzag lightning patterns or perceptions of bright colored streaks, halos or diagonal lines. Negative visual losses include blurring, blank areas, and black dots or spots in the field of vision causing partial or complete blindness. Complex patterns of visual impairment, such as the coming together of spots and tunnel vision (not being able to see items in the periphery of one’s visual field) are less common.
The visual disturbance often occurs on the same side as the migraine headache and may precede, accompany or, rarely, follow it. The duration of the visual symptoms may be as short as a few seconds but usually lasts many minutes to one hour. More rarely, prolonged but fully reversible visual loss in one eye may occur, sometimes lasting hours, days or even weeks.
The diagnostic work-up should include a careful medical and ophthalmological examination. It is often difficult to distinguish between a visual aura that affects the right or left half of a person’s visual field in both eyes and the loss of vision in one eye. To make this distinction one must alternately cover each eye and compare the views. If visual loss in one eye is confirmed, causes of visual loss other than migraine must first be excluded. Blood work and imaging studies of the heart, brain, eyes and blood vessels in the neck are recommended.
Once retinal migraine is suspected, the patient should be referred to an ophthalmologist and a neurologist who specializes in the treatment of headache. Although retinal migraine has usually been viewed as a benign condition, it appears that partial or complete permanent visual loss of one eye may occur. No specific factor has been identified to account for this occurrence. Therefore, preventive drug therapy for migraine seems prudent, even if attacks are infrequent.
Literature Review of retinal migraine
Carroll introduced the term “Retinal Migraine” writing in Headache in 1970. He described 15 patients with transient and persistent monocular visual loss but no associated headaches. Criticism has been leveled at Carroll because ICHD II criteria, which were first published in 1988, were not applied to his cases and many of the patients had no headache. Note that ICHD II calls this entity “Retinal Migraine” and not “eye migraine, ocular migraine, or ophthalmic migraine” which are other terms commonly used for this syndrome.
Grosberg, et al, writing in 2006 in Cephalalgia on “Retinal migraine reappraised,” reported that most of their patients were women in the second or third decade of life who had a history of migraine with aura. Grosberg, et al, reported 6 new cases and reviewed 40 from the literature. This is different from ICDH 3 Beta criteria regarding retinal migraine, which states that most patients have migraine without aura. The authors stated, “in the typical attack monocular visual features consist of partial or complete vision loss lasting < 1 hour, ipsilateral to the headache.” Permanent monocular visual loss occurred in half of the reported cases although ICDH-II criteria require reversible visual loss. Grosberg, et al, stated:
Based on this observation, the authors recommend migraine prophylactic treatment in an attempt to prevent permanent visual loss, even if attacks are infrequent. We also proposed a revision to the ICDH-II diagnostic criteria for retinal migraine.
Hill, et al, wrote an article entitled “Most Cases Labeled as “Retinal Migraine” Are Not Migraine,” in the Journal of Neuro-Ophthalmology in 2007. These authors pointed out that monocular vision loss has often been labeled “retinal migraine” yet many of the previously reported cases do not meet the diagnostic criteria of ICDH-II which defines “retinal migraine” as an attack of reversible monocular visual disturbance with migraine headache and a normal neuro-ophthalmic exam between attacks. In their paper these authors reported:
We performed a literature search of articles mentioning "retinal migraine," "anterior visual pathway migraine," "monocular migraine," "ocular migraine," "retinal vasospasm," "transient monocular visual loss," and "retinal spreading depression" using Medline and older textbooks. We applied the IHS criteria for retinal migraine to all cases so labeled. To be included as definite retinal migraine, patients were required to have had at least two episodes of transient monocular visual loss associated with, or followed by, a headache with migrainous features.
They found only 5 patients who met ICDH-II criteria for definite retinal migraine. No patient with permanent visual loss met the IHDH-II criteria for retinal migraine. Hill, et al, concluded that:
Definite retinal migraine, as defined by the IHS criteria, is an exceedingly rare cause of transient monocular visual loss. There are no convincing reports of permanent monocular visual loss associated with migraine. Most cases of transient monocular visual loss diagnosed as retinal migraine would more properly be diagnosed as "presumed retinal vasospasm."
Hill, et al, support specific comments on treatment although they felt that triptans or ergotamine should be avoided because of their vasoconstrictive properties. Patients should be counseled to avoid oral contraceptives. Preventive therapies such as calcium-channel blockers, tricyclic antidepressants, beta-blockers, and neuromodulators have only anecdotal data to support their use. Daily prophylactic aspirin would be reasonable advice.
Rucker CW. The Interpretation of Visual Fields. American Academy of Ophthalmology and Otolaryngology. 15 Second Street SW, Rochester, Minnesota 55901. Third Edition. 1957.
Spierings ELH. Monocular Symptoms in Retinal Migraine, comment. Journal Watch Neurology. 2007;9(5):38.
Grosberg BM, Solomon S, DI Friedman, RB Lipton (2006) Retinal migraine reappraised. Cephalalgia. 2006;26(11):1275–1286.
Hill DL, Daroff RB, Ducros A, Newman NJ, Biousse V. Most Cases Labeled as "Retinal Migraine" Are Not Migraine. Journal of Neuro-Ophthalmology. 2007;27(1):3-8.
Grosberg BM, Solomon S, Lipton RB. Retinal migraine. Curr Pain Headache Rep. 2005;9:268-271.
Grosberg BM, Solomon S. Retinal migraine: Two cases of prolonged but reversible monocular visual defects. Cephalalgia. 2006;26:754-757.
Flammer J, Pache M, Resink T. Vasospasm, its role in the pathogenesis of disease with particular reference to the eye. Prog Retinal Eye Res. 2001;20:319-349.
Van Harreveld A. Two mechanisms for spreading depression in the chicken retina. J Neurobiol. 1978;9:419-431.
Winterkorn JS, Kupersmith MJ, Wirtschafter JD, Forman S. Brief report: Treatment of vasospastic amaurosis fugax with calcium-channel blockers. N Engl J Med. 1993;329:396-399.
Diagnosis of Retinal Migraine
There are no diagnostic tests to confirm retinal migraine. Diagnosis is accomplished by reviewing the patient’s personal and family medical history, studying their symptoms, and conducting an examination. Retinal migraine is then diagnosed by ruling out other causes for the symptoms. With retinal migraine, it is essential that other causes of transient blindness, such as stroke of the eye (amaurosis fugax), be fully investigated and ruled out. Seeing an eye doctor (ophthalmologist) for a full eye exam is generally required for a good look at the back of the eyes.
Treatment for Retinal Migraines
There are no clear guidelines for the management of patients with retinal migraine. For infrequent attacks, medications used for other forms of migraine are often employed to relieve the other symptoms. These medications can include NSAIDs and antinausea medications. Triptans and ergotamines are avoided in these patients. Preventive therapies used for other migraines types should be explored, such as the calcium channel blockers, antiepileptic or tricyclic medications. Daily aspirin is considered for these people as well. People should stop smoking and discontinuation of oral contraceptives may be advisable.
Summary of Retinal Migraines
Retinal migraines are a subtype of migraine associated with monocular (one eye only) vision symptoms. Patients suspected of having retinal migraine should be carefully assessed by their doctor for an underlying cause.
To help distinguish retinal migraine from migraine with typical aura, it is important that people carefully assess whether their vision symptoms are perceived in one or both eyes by alternating looking through either eye. The distinction between retinal migraine and migraine with typical aura has ramifications for treatment choices as well as prognosis counselling.
Grosberg BM, Solomon S, Friedman DI, Lipton RB. Retinal migraine reappraised. Cephalalgia 2006; 26:1275.
Grosberg BM, Solomon S, Lipton RB. Retinal Migraine. Current pain and Headache Reports 2005; 9: 268-271.
Summary
Retinal Migraine is a very rare, somewhat controversial Migraine condition.
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Britt Talley Daniel MD
