Migraine is one of the disorders most commonly seen by neurologists and ophthalmologists. Only about 56% of migraineurs know that they have the condition, however, because they often misdiagnose their headaches as ocular strain, sinus headaches, or stress headaches.

EPIDEMIOLOGY

In a given year, migraine has a prevalence of 12% (17.1% in women and 5.6% in men).¹ Annually, some 35 million people suffer migraine in the United States. The cumulative incidence of this disorder by age 85 is 18.5% in men and 44.3% in women, with onset before the age of 25 in 50% of cases, before the age of 35 years in 75%, and over the age of 50 in only 2%.² The median age of onset is 25 years. Approximately 8% of boys and 11% of girls have migraine.³ Chronic migraine—with attacks occurring on 15 or more days per month for at least 3 months—affects about 3.2 million people per year in the United States, 80% of whom are women.⁴

MIGRAINE WITHOUT AURA

Clinical Features

Pain is unilateral in 60% of cases and bilateral in 40%. Approximately 15% of migraineurs report so-called side-locked headaches in which migraine always occurs on the same side. The pain is often more intense in the frontotemporal and ocular regions before it spreads to the parietal and occipital areas. Any part of the head or face may be affected, including the parietal region, the upper or lower jaw or teeth, the malar eminence, and the upper anterior neck. Throbbing pain is present in 85% of episodes of migraine, although up to 50% of patients describe steady pain during some attacks. As many as 75% of migraineurs report unilateral or bilateral tightness, stiffness, or throbbing pain in the posterior neck along with head pain. The neck pain can occur during the migraine prodrome, the attack itself, or the postdrome.⁵ (See Criteria for Migraine Without Aura.)

Migraines last 4 to 72 hours if left untreated or if unsuccessfully treated. One that persists for more than 72 hours is termed status migrainosus. Without treatment, 80% of patients experience moderate to severe pain, and 20% have mild pain. Usually increased by physical activity or movement, the pain is associated with nausea in about 80% of episodes, vomiting in about 30%, photophobia in about 90%, and phonophobia in about 80%.⁶

When patients deny a history of light and noise sensitivity, physicians should ask the following questions:

• During a headache, would you prefer to be in bright sunlight or in a dark room?
• During a headache, would you prefer to be in a room with loud music or in a quiet room?

Approximately 20% of patients become aware of associated light and noise sensitivity because of the aforementioned questions.⁷

In general, 25% of migraineurs suffer four or more severe attacks a month, 35% have one to four severe attacks per month, 38% experience one or fewer severe attacks per month, and 37% have five or more headache days per month.¹ In one study, during migraine attacks, most migraineurs (53.7%) reported severe impairment or the need for bed rest, whereas only 7.2% reported no attack-related impairment. Over a 3-month period, 35.1% of the migraineurs had at least 1 day of restricted activity related to headache.

Migraine triggers are present in 76% of migraineurs. One study reported the following triggers from affected patients: stress, 89%; female hormones, 65%; not eating, 57%; weather, 53%; physical exhaustion or travel, 53%; sleep disturbance, 50%; perfume or odor, 44%; bright lights, 38%; neck pain; 38%; alcohol, 38%; smoke, 36%; sleeping late, 32%; heat, 30%; food, 27%; and exercise, 22%.⁸

Cranial autonomic symptoms are caused by parasympathetic activation of the sphenopalatine ganglion during an attack that innervates the tear ducts and sinuses. At least one symptom is present in 56% of migraineurs, usually bilaterally and not present during each attack. The most common are forehead/facial sweating, conjunctival injection and/or lacrimation, and nasal congestion and/or rhinorrhea.⁹ Migraineurs and some physicians misdiagnose the headaches as “sinus,” because the pain occurs in the face or forehead, a change in weather is a common trigger, and the presence of cranial autonomic symptoms seems like sinus symptoms. Migraines are confused with stress or tension-type headaches, because they commonly occur in the neck and are often triggered by psychological stress. The table compares the features of migraine, tension-type, and cluster headaches.

Benign Episodic Unilateral Mydriasis

Benign episodic unilateral mydriasis is transient and isolated with normal vision and pupillary reactivity to light. In young adults or children, the disorder may occasionally accompany migraine headaches (although cases have been described without accompanying headache).¹⁰ Lasting 15 minutes to 24 hours and often associated with blurred vision, episodes average two to three per month. Ocular and motility abnormalities are absent. The pupil dilates due either to parasympathetic insufficiency of the iris sphincter or to sympathetic hyperactivity of the iris dilator muscle. Angle-closure glaucoma should be excluded.

MIGRAINE WITH AURA

Epidemiology

In a given year in the United States, the prevalence of migraine with aura is 5.3% in women (30.8% of female migraineurs) and 1.9% in men (32% of male migraineurs).⁶ As many as 81% of those who have migraine with aura also have attacks of migraine without aura.¹¹

The reported age of onset is between a mean of 11.9 years (range, 4-17)¹² and a mean of 21 years (range, 5-77).¹³ In one study, 54.9% of patients suffered less than one attack per month, and 9.7% reported more than three attacks per month.¹⁴ In another study, the mean number of episodes/year/patient was 29 (range, >1-156).¹⁵

Clinical Features

In a study of 362 patients suffering from migraine with aura—at least in some attacks—99% had a visual aura, 54% had a sensory aura, and 32% had an aphasic aura.¹⁶ Most had a combination of aura symptoms as follows: 28% had a visual and sensory aura; 25% had a visual, sensory, and aphasic aura; 6% had a visual and aphasic aura; and 39% had a visual aura exclusively. When more than one aura symptom occurred, they did so in succession in 96% versus simultaneously in 4% of patients. Ninety-one percent experienced a gradual onset of visual aura symptoms. The mean age of the patients in this study was 46 years (range, 12-90). (See Criteria for Migraine With Aura.)

The “classic” visual aura is the fortification (looks like a fortified town as viewed from above) spectra or teichopsia (“seeing fortifications”), which is a jagged figure with fortification lines arranged at right angles to one another beginning from a paracentral area, which usually spreads outward, leaving visual loss behind. There are often scintillations, which may be white or gray or have colors similar to a kaleidoscope in a semicircle or C shape surrounding the scotoma or area of visual loss. Scintillating scotomata are typically in one hemifield, with visual field defects beginning around fixation and spreading outward. The symptoms reported by patients, however, may be quite variable.

Approximately 50% of patients report that the visual auras begin in the periphery, whereas the remaining 50% see them starting in or adjacent to the center of the visual field. In a series of 122 clinical patients,¹⁷ the laterality of the visual auras was reported as follows: always on the same side of vision (right or left), 22.1%; one sided but not always on the same side, 23.8%; always on both sides of vision, 23.8%; and sometimes on one side, sometimes on both sides, 29.5%.¹⁸ The reported color of the visual auras was always black-and-white (30.3%), always black and silver (20.5%), always colorful (18%), both black-and-white and colorful (22.2%), and colorless (9%). The visual phenomena were described with the following characteristics and percentages of patients: blurred vision, 54.1%; small, bright dots, 47.5%; zigzag lines, 41.8%; flashes of bright light, 38.5%; “blind spots,” 33.6%; flickering light, 30.3%; “like looking through heat waves or water,” 24.6%; blindness in half of a visual field, 23.8%; white spots, 22.1%; colored dots/spots of light, 19.7%; curved or circular lines, 18.9%; small, black dots, 17.2%; bean-like forms such as a crescent or C shape, 16.4%; black spots, 14.8%; and tunnel vision, 9.8%. Less common visual auras include corona phenomena, palinopsia,¹⁸ metamorphopsia, macropsia, micropsia, telescopic vision, teleopsia, mosaic vision, and multiple images.

According to the International Classification of Headache Disorders, third edition (ICHD-III), the typical duration of migraine aura is 5 to 60 minutes. The ICHD-III labels aura lasting longer than an hour but less than a week as probable migraine with aura. Visual aura has reportedly lasted more than 1 hour in 6% to 10% of patients.¹⁹ Other aura symptoms enduring more than 1 hour are somatosensory aura in 14% to 27% of patients and aphasic aura in 17% to 60%.

A sensory aura consists of numbness, tingling, or a pins-and-needles sensation. The aura, which is usually unilateral, commonly affects the hand and then the face, or it may affect either one alone.²⁰ Paresthesia of one side of the tongue is typical. Less often, the leg and trunk may be involved. A true motor aura is rare, but sensory ataxia or a heavy feeling is often misinterpreted as weakness. Patients frequently report a speech disturbance when the spreading paresthesias reach the face or tongue. Slurred speech may be present. With involvement of the dominant hemisphere, paraphasic errors and other types of impaired language production and comprehension may occur. Rarely, other aura symptoms may be described, including déjà vu and olfactory and gustatory hallucinations.

Migraine aura is considered by many to be a distinct phase of the migraine attack preceding the headache. In a prospective study of 861 attacks of migraine with aura in 201 patients, however, during the aura phase, 73% of attacks were associated with headaches; 54% of the headaches fulfilled migraine criteria during the first 15 minutes of the aura’s onset.²¹ Aura follows headache in approximately 3% to 8% of cases.¹⁶ The headache may be contralateral to the side of the visual aura, ipsilateral in up to 62% of patients for some attacks,¹⁷ or bilateral.

Migraine Aura Versus Cerebral Ischemia and Seizures

Unlike symptoms due to cerebral ischemia, visual or sensory auras from migraine typically spread slowly across the visual field or body part followed by a gradual return to normal function in the areas first affected after 20 to 60 minutes.²² The onset of cerebral ischemic events is usually sudden with an equal distribution in the relevant vascular territory, although the affected area can expand stepwise if blood flow drops in additional vessels.²³ The progression in partial seizures is generally much more rapid. The return of function in areas first affected while symptoms begin in newly affected areas occurs in migraine aura but not in ischemia or seizures. As noted earlier, when more than one type of aura occurs, they almost always do so one after the other; in contrast, multiple neurological symptoms typically occur at the same time in cerebral ischemia. Finally, migraine aura often begins with positive phenomena such as shimmering lights, zigzags in vision, or tingling. It is then frequently followed minutes later by negative symptoms such as scotoma, numbness, or a loss of sensation, which can also occur during seizures but usually with a faster progression of symptoms. This cycle from positive to negative symptoms is not typical of cerebral ischemia.

Late-Life Migraine Accompaniments and Migraine Aura Without Headache

Fisher reported new-onset “late life migrainous accompaniments” in 85 patients aged 40 to 73 years.²⁴ The episodes resembled transient ischemic attacks²⁵ similar to those of the 120 patients he had previously described. Most had visual symptoms occurring alone (44/205) or combined with other aura symptoms. Headache was associated with the episodes in 40% of cases. In the Framingham study, visual migrainous symptoms were reported by 1.23% of subjects (1.33% of women and 1.08% of men) who had an onset after age 50 in 77% with the following characteristics: stereotyped in 65%, never accompanied by headaches in 58%, ranging in number of episodes from one to 500 with 10 or more in 69% of subjects, and lasting 15 to 60 minutes in 50%.²⁶ Of 1,000 patients presenting for a comprehensive eye examination in Alabama,²⁷ 6.5% reported visual symptoms consistent with migraine aura without headache, 8.6% women and 2.9% men with risk factors such as a history of migraine headaches and a history of childhood motion sickness.

A retrospective study of 100 aura patients compared those whose age at onset was 45 years or older versus younger than 45 years.²⁸ The investigators found no difference in gender distribution, family or personal history of migraine without aura, type of aura symptoms, or imaging findings. Aura symptoms were mostly visual. The duration of the auras was similar in both groups, breaking out as follows in the late-onset group: less than 20 minutes, 47.8%; 20 to 60 minutes, 39.1%; and longer than 60 minutes, 13%. Headache was associated with auras less often in patients who were older at onset. The patients whose age at onset was 65 or older were similar to those with an onset age of 45 or older.

“Alice in Wonderland” Syndrome

“Alice in Wonderland” syndrome, a term coined by Todd in 1955,²⁹ is a rare migraine aura. Usually, affected patients experience a distortion in body image that they know is unreal (ie, enlargement, diminution, or distortion of part of or the whole body). The syndrome can occur at any age but is more common in children. The cause may be migrainous ischemia of the nondominant posterior parietal lobule. Other causes include medication (topiramate,³⁰ cough syrup with dihydrocodeine phosphate and DL-Methylephredrine hydrochloride), Epstein-Barr virus and other infections,³¹ depression, seizures, and a right medial temporal lobe stroke.

Persistent Visual Aura

Rarely (35 cases reported), migraineurs may have persistent visual aura.^32,33 This aura usually consists of simple, unformed hallucinations in the entire visual field of both eyes, including innumerable dots, television static, clouds, heat waves, flashing or flickering lights, lines of ants, a rain-like or snow-like pattern, squiggles, bubbles, and grainy vision. Occasionally, palinopsia (the persistence of visual images), micropsia, or formed hallucinations occur. The ICHD-III describes persistent aura without infarction as aura symptoms persisting for 1 week or more without evidence of infarction on neuroimaging.

Migraine With Brainstem Aura

Brainstem aura was formerly termed basilar-type migraine, a label no longer used because involvement of the basilar artery is unlikely (see Criteria for Migraine With Brainstem Aura). Migraine with brainstem aura is a rare disorder that usually affects patients aged 7 to 20 years and rarely presents in individuals older than 50 years.³⁴ One study reported the following aura symptoms: vertigo, 61%; dysarthria, 53%; tinnitus, 45%; diplopia, 45%; bilateral visual symptoms, 40%; bilateral paresthesias, 24%; decreased level of consciousness, 24%; and hypoacusis, 21%. Visual symptoms—usually blurred vision, shimmering colored lights accompanied by blank spots in the visual field, scintillating scotoma, and graying of vision—may start in one visual field and then spread to become bilateral. The median duration of aura was 60 minutes (range, 2 minutes-72 hours), with two or more aura symptoms always occurring.

Retinal Migraine

Retinal migraine is rare.³⁵ The mean age at onset is 25 years. Patients present with fully reversible, monocular, positive and/or negative visual phenomena lasting less than 2 hours (see Criteria for Retinal Migraine). Typically, patients report flashing rays of light and zigzag lightning and, less often, brightly colored streaks, halos, or diagonal lines. Negative phenomena may be blurring, “gray-outs,” and “blackouts” causing partial or complete blindness. Elementary forms of scotoma are perceived as blank areas, black dots, or spots in the field of vision. Visual field defects can be altitudinal, quadrantic, central, or arcuate. The headache is usually ipsilateral to the visual loss. Almost 50% of those affected have a history of migraine with visual aura. Some patients who report monocular visual disturbance have hemianopsia of which they are not aware, because they do not perform a cover/uncover test. Diagnosis is one of exclusion of other causes of transient monocular blindness. Retinal migraine can lead to permanent monocular visual loss.

Randolph W. Evans, MD, is a clinical professor of neurology at Baylor College of Medicine in Houston. Dr. Evans may be reached at revansmd@gmail.com.

 

1. Lipton RB, Bigal ME, Diamond M, et al. Migraine prevalence, disease burden, and the need for preventive therapy. Neurology. 2007;68:343-349.
2. Stewart WF, Wood C, Reed ML, et al; AMPP Advisory Group. Cumulative lifetime migraine incidence in women and men. Cephalalgia. 2008;28(11):1170-1178.
3. Wöber-Bingöl C. Epidemiology of migraine and headache in children and adolescents. Curr Pain Headache Rep. 2013;17:341.
4. Buse DC, Manack AN, Fanning KM, et al. Chronic migraine prevalence, disability, and sociodemographic factors: results from the American Migraine Prevalence and Prevention Study. Headache. 2012;52:1456-1470.
5. Calhoun AH, Ford S, Millen C, et al. The prevalence of neck pain in migraine. Headache. 2010;50(8):1273-1277.
6. Lipton RB, Scher AI, Kolodner K, et al. Migraine in the United States: epidemiology and patterns of health care use. Neurology. 2002;58:885-894.
7. Evans RW, Seifert T, Kailasam J, et al. The use of questions to determine the presence of photophobia and phonophobia during migraine. Headache. 2008;48:395-397.
8. Kelman L. The triggers or precipitants of the acute migraine attack. Cephalalgia. 2007;27:394-402.
9. Lai TH, Fuh JL, Wang SJ. Cranial autonomic symptoms in migraine: characteristics and comparison with cluster headache. J Neurol Neurosurg Psychiatry. 2009;80:1116-1119.
10. Evans RW, Jacobson DM. Transient anisocoria in a migraineur. Headache. 2003;43:416-418.
11. Queiroz LP, Rapoport AM, Weeks RE, et al. Characteristics of migraine visual aura. Headache. 1997;37:137-141.
12. Lanzi G, Balottin U, Borgatti R. A prospective study of juvenile migraine with aura. Headache. 1994;34:275-278.
13. Eriksen MK, Thomsen LL, Andersen I, et al. Clinical characteristics of 362 patients with familial migraine with aura. Cephalalgia 2004;24:564-575.
14. Manzoni GC, Farina S, Lanfranchi M, et al. Classic migraine—clinical findings in 164 patients. Eur Neurol. 1985;24:163-169.
15. Crotogino J, Feindel A, Wilkinson F. Perceived scintillation rate of migraine aura. Headache. 2001;41:40-48.
16. Eriksen MK, Thomsen LL, Olesen J. Sensitivity and specificity of the new international diagnostic criteria for migraine with aura. J Neurol Neurosurg Psychiatry. 2005;76:212–217.
17. Queiroz LP, Friedman DI, Rapoport AM, Purdy RA. Characteristics of migraine visual aura in Southern Brazil and Northern USA. Cephalalgia. 2011;31:1652-1658.
18. Belcastro V, Cupini LM, Corbelli I, et al. Palinopsia in patients with migraine: a case-control study. Cephalalgia. 2011;31:999-1004.
19. Viana M, Sprenger T, Andelova M, Goadsby PJ. The typical duration of migraine aura: a systematic review. Cephalalgia. 2013;33:483-490.
20. Russell MB, Olesen J. A nosographic analysis of the migraine aura in a general population. Brain. 1996;119:335-361.
21. Hansen JM, Lipton RB, Dodick DW, et al. Migraine headache is present in the aura phase: a prospective study. Neurology. 2012;79:2044-2049.
22. Foroozan R, Cutrer FM. Transient neurologic dysfunction in migraine. Neurol Clin. 2009;27:361-378.
23. Cutrer FM, Huerter K. Migraine aura. Neurologist. 2007;13:118-125.
24. Fisher CM. Late-life migraine accompaniments as a cause of unexplained transient ischemic attacks. Can J Neurol Sci. 1980;7:9-17.
25. Fisher CM. Late-life migraine accompaniments—further experience. Stroke. 1986;17:1033-1042.
26. Wijman CA, Wolf PA, Kase CS, et al. Migrainous visual accompaniments are not rare in late life: the Framingham Study. Stroke. 1998:1539-1543.
27. Fleming JB, Amos AJ, Desmond RA. Migraine aura without headache: prevalence and risk factors in a primary eye care population. Optometry. 2000;71:381-389.
28. Martins IP, Goucha T, Mares I, Antunes AF. Late onset and early onset aura: the same disorder. J Headache Pain. 2012;13:243-245.
29. Todd J. The syndrome of Alice in Wonderland. Can Med Assoc J. 1955;73:701-704.
30. Evans RW. Reversible palinopsia and the Alice in Wonderland syndrome associated with topiramate use in migraineurs. Headache. 2006;46(5):815-818.
31. Lanska JR, Lanska DJ. Alice in Wonderland syndrome: somesthetic vs visual perceptual disturbance. Neurology. 2013;1262-1264.
32. Evans RW, Aurora SK. Migraine with persistent visual aura. Headache. 2012;52:494-501.
33. Simpson JC, Goadsby PJ, Prabhakar P. Positive persistent visual symptoms (visual snow) presenting as a migraine variant in a 12-year-old girl. Pediatr Neurol. 2013;49:361-363.
34. Kirchmann M, Thomsen LL, Olesen J. Basilar-type migraine: clinical, epidemiologic, and genetic features. Neurology. 2006;66:880-886.
35. Evans RW, Grosberg BM. Retinal migraine: migraine associated with monocular visual symptoms. Headache. 2008;48:142-145.