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REVIEW

ROBERT S. KUNKEL, MD
CME
CREDIT
Consultant, Headache Center,
Department of Neurology,
The Cleveland Clinic Foundation

Migraine aura without headache:


Benign, but a diagnosis of exclusion
■ A B S T R AC T
M being followed bycanaoccur
IGRAINE AURAS alone, without
headache. This usu-
Migraine aura without headache should be considered as ally benign condition occurs more often in
a diagnosis in anyone who has recurrent episodes of older people with a history of migraine, but it
transient symptoms, especially those that are visual or may also occur in others.
neurological or involve vertigo. Visual and neurological This article covers both common and
symptoms due to migraine are not unusual and most unusual symptoms attributed to migraine aura
commonly occur in older persons with a history of without headache and how to distinguish
migraine headaches. Migraine aura without headache them from other conditions. The pathophysi-
should be diagnosed only when transient ischemic attack ology, epidemiology, and treatment of migraine
without headache are also discussed.
and seizure disorders have been excluded.
■ DEFINITION
■ KEY POINTS
Migraine auras are reversible and recurrent episodes of The classification of headache disorders, pub-
neurological symptoms that resolve within 1 hour. They lished by the International Headache Society
in 1988 and updated in 2004, defines aura as
are associated with migraine but may not precede a “a recurrent disorder manifesting in attacks of
headache. reversible focal neurological symptoms that
usually develop gradually over 5 to 20 minutes
Auras almost always involve visual symptoms. Images are and last for less than 60 minutes.”1
usually bright, shimmering, and dynamic, and may form “Migraine aura without headache” is now
geometric shapes. Ischemic symptoms, on the other hand, the accepted term for “migraine equivalents”
tend to be dark and not moving. or “acephalgic migraine”: episodic symptoms
believed to be migrainous auras but not fol-
Auras usually need no treatment. If desired, a short- lowed by a headache.
acting agent, such as a beta-agonist inhalant, sublingual
nitroglycerin, meclofenamate, or naproxen sodium, can be ■ VISUAL SYMPTOMS NOT UNCOMMON
tried. Verapamil or antiepileptic drugs may be used as
Migraine aura without headache can occur at
prophylaxis. any age and in people who never had a
migraine headache.2–5 However, it is most
Triptans should not be used to treat an aura. Oral triptans common in older people, especially in those
do not act fast enough to affect an aura, and the rapid- who had auras accompanied by migraine
acting injectable sumatriptan, if given during the aura, headaches when younger.6 Patients may con-
may not abort the subsequent headache. tinue to have migraine headaches in addition
to auras without a subsequent headache.
This paper discusses therapies that are experimental or that are not approved by the US Food Transient visual disturbances are not
and Drug Administration for the use under discussion. uncommon in the older population. On the

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Downloaded from www.ccjm.org on October 16, 2013. For personal use only. All other uses require permission.
MIGRAINE AURA KUNKEL

TA B L E 1 Fisher,2 in a series of 120 patients over age


40 years with transient episodes resembling
Transient episodic migraine auras, found that most had visual
symptoms of migraine aura symptoms and 20% had nonvisual neurologi-
without headache cal symptoms.
Visual
Photopsia ■ PATHOPHYSIOLOGY OF MIGRAINE AURAS
Scotoma
Hemianopsia Migraine is a very complex inherited disorder,
Diplopia and its pathophysiology is not well under-
Blindness stood.
Metamorphopsia A migraine attack apparently starts in the
Neurological brainstem and involves activation of the
Paresthesias trigeminal nerve and areas of the cortex.
Vertigo Branches of the trigeminal nerve innervate
Amnesia the anterior cerebral vessels as well as the
Confusion structures of the anterior head and face.
Alteration of mood Various peptides and other vasoactive sub-
Hemiparesis stances are released at the neurovascular junc-
Hearing loss tion, causing mild vascular constriction fol-
Other lowed by dilation and perivascular inflamma-
Cyclic vomiting tion.7 The decreased blood flow does not seem
Recurrent abdominal pain to cause cerebral ischemia.
Coronary artery spasm In addition, a wave of depolarization may
Raynaud disease spread across the cortex, especially in the
occipital lobe. This so-called “spreading
depression” accounts for the slowly evolving
Visual aura other hand, nonvisual symptoms that may be nature of the visual symptoms typical of a
symptoms are migrainous are so uncommon that very few migraine aura. Nonvisual and non-neurologi-
studies exist on their prevalence and epidemi- cal symptoms that are believed to be migrain-
common in ology. ous (eg, variant angina, vertigo, and abdomi-
older people; The Framingham study reported that nal migraine) are likely due to dysfunction of
migrainous-type visual symptoms occurred in the autonomic nervous system.
nonvisual aura 1% to 2% of the elderly participants.4 Of those The vascular system in general is much
symptoms are reporting such symptoms, 77% said they more reactive in patients with migraines.
less so occurred for the first time after age 50 years, They have vasomotor instability and are more
42% had no history of migraine, and 58% said prone to conditions such as Raynaud disease,
episodes were never associated with a livedo reticularis, vasomotor rhinitis, cardiac
headache. arrhythmias, syncope, urticaria, and flushing.
Mattsson and Lundberg5 compared 100
women with migraines in a headache clinic in ■ SYMPTOMS INVOLVED IN AURAS
Sweden with 245 women in the general popu-
lation and found that the lifetime prevalence According to the International Headache
of visual disturbances without a headache was Society, migraine auras gradually develop over a
37% in those with migraines and 13% in the few minutes and last less than 60 minutes. A
general population. Undoubtedly, some of number of symptoms have been reported as
those in the general population had migraines being migraine aura without headache (TABLE 1).
as well.
Ziegler and Hassanein6 found that 44% of Visual symptoms—bright and dynamic
patients diagnosed as having migraine with Any symptom may be involved in an aura, but
aura reported having had an aura occur with- visual symptoms occur in 99% of migraine
out a headache at some time. auras8 and tend to accompany any other neu-

530 CLEVELAND CLINIC JOURNAL OF MEDICINE VOLUME 72 • NUMBER 6 JUNE 2005


rological symptoms that may also occur. generically for any visual events of a migrain-
The most common visual symptom in an ous nature occurring without a headache.
aura with or without a headache is photopsia
(unformed flashes of light). Teichopsia, also Neurological symptoms
known as fortification spectrum, is believed to Neurological symptoms are the second most
be the most diagnostic of migraine. It involves common aura symptoms that occur without a
a bright, zigzag border that looks like an aerial headache and, as with other migraine auras,
view of an old fortress. tend to last 10 to 30 minutes.
Other common symptoms include sco- Neurosensory symptoms, ie, numbness,
toma (partial loss of sight or a “blind spot” tingling, or paresthesias, are most common.
that is often crescent-shaped), visual distor- The paresthesias typically slowly spread up or
tion, “heat waves,” blurring, and hemianopsia. down the limbs: most commonly, the sensa-
Much less common are diplopia (double tion starts in the fingers, then spreads to the
vision) and metamorphopsia (altered or dis- hand and slowly up the arm. This “march” or
torted objects). spreading sensation may go on for several
A typical visual aura is not static but minutes and may progress to the face and
grows and moves across the visual field. This mouth area, cross over, and descend the other
dynamic quality may help differentiate arm. Episodic numbness of the tongue without
migraine symptoms from symptoms of a tran- other symptoms is thought to most likely be
sient ischemic attack (TIA). due to migraine.2
Migraine visual defects are often bright Mild weakness of the limbs, with the
and shimmering—even a blind spot usually hands and arms most often involved, may also
has a bright, shimmering border. Defects may occur. In migraine, the area where the symp-
also be multicolored or form a geometric pat- toms first appear (usually the hand) clears
tern. Dark defects or dimming of the vision first, in contrast to ischemic symptoms, in
suggests ischemic disease. which the area initially involved usually clears
Visual migrainous auras usually last 15 to last.
30 minutes. In contrast, TIAs with visual Total global amnesia is most often seen in A typical visual
symptoms are usually shorter (3–10 minutes). patients with migraine, usually without a aura grows and
Partial seizures are also usually short, lasting headache. Typically, it occurs only once or
less than 5 minutes. An arteriovenous malfor- twice in a lifetime, but some people have moves across
mation involving the occipital lobe may cause repeated spells. The amnesia usually lasts 1 to the visual field
transient visual symptoms, which are also 2 hours but may be briefer. People appear to
short in duration.9 If visual symptoms are function normally during the period of amne-
recurrent and last longer than 60 minutes, an sia and are able to carry on normal conversa-
underlying disorder such as retinal disease, tions and activities, such as driving or shop-
recurrent emboli, a coagulopathy, or vasculitis ping, but cannot afterwards recall anything
should be considered. that happened. The preamnesia memory is
The visual symptoms of migraine are intact when the episode is over.
homonymous—occurring in the correspond- Speech disturbances, ie, expressive apha-
ing vertical halves of the visual fields of both sia or dysarthria (disturbed articulation),
eyes—due to occipital lobe cortical dysfunc- sometimes occur during migraine attacks,
tion. However, it is often difficult for patients occasionally without an accompanying
to determine this because they tend to notice headache.
a problem on only one side of their vision. A Vertigo not uncommonly accompanies
strictly monocular defect suggests retinal dis- migraine, sometimes without a headache.10
ease or ischemia due to carotid artery disease. Vestibular studies are normal, and the attacks
Ocular migraine, also known as retinal are much shorter than usually seen in Meniere
migraine, is very rare and causes strictly uni- disease. Migraine aura without headache
lateral visual symptoms. It usually occurs in should be considered in anyone with recur-
young people with a history of migraine.9 rent attacks of vertigo with normal auditory
Some people use the term “ocular migraine” and vestibular testing.

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MIGRAINE AURA KUNKEL

TA B L E 2 Cyclic vomiting involves severe, self-lim-


ited vomiting spells that may last for several
Visual symptoms of migraine aura hours. The child is typically irritable and may
vs transient ischemic attack (TIA) be photophobic. Episodes end after the child
FEATURE MIGRAINE AURA TIA
sleeps.
Abdominal migraine involves episodic
Duration 15–30 minutes 3–10 minutes midline abdominal pain lasting 1 to 8 hours. It
is associated with nausea, vomiting, often pal-
Quality Dynamic, bright, multicolored Static, dark lor,14 and sometimes a mild nonmigrainous
Forms geometric patterns Dimming of vision headache.
Abdominal migraine can occur in adults
but is extremely rare. One woman patient of
In children, benign paroxysmal vertigo is mine had a history of migraine and recurrent
believed to be migrainous; most children diag- epigastric pains. Several workups revealed no
nosed with it eventually develop migraines.11 abnormality. The attacks ceased when she was
The attacks involve sudden vertigo that lasts treated with a beta-blocker, a drug commonly
for a few minutes to a few hours: the child may used to prevent recurrent migraines.
suddenly stop playing and fall to the ground.
Recovery is complete, with no residual audito- ■ DIFFERENTIAL DIAGNOSIS
ry or vestibular abnormalities evident.
Confusion can accompany migraine, If other causes are eliminated, any recurrent,
especially in children, and may occur without transient, and episodic symptoms that are fully
headache. Patients with migraines are more reversible and last less than 1 hour should be
likely than the general population to develop considered migrainous. The diagnosis of
confusion after a mild head trauma, such as a migraine aura without headache should be
sports injury. Adolescents with spells of confu- made only when other possible causes have
sion independent of a migraine headache may been excluded.
Seizures come be suspected of drug abuse. A history of TIAs must be ruled out, since migraine
on much more migraine headaches may be helpful in this sit- aura without headache commonly affects
uation. older persons, in whom vascular disease is
rapidly than more prevalent. Visual symptoms of TIAs can
migraine auras ■ OTHER POSSIBLY MIGRAINOUS often be distinguished from those of migraine
CONDITIONS auras (TABLE 2). Ischemic eye symptoms are usu-
ally shorter, do not move and spread across the
A few recurring conditions that are very rare visual field, and generally result in dimming of
are also thought to be migrainous. vision.
Cardiac migraine, described in 1974, is Retinal disease can be manifested as
believed to be due to coronary artery spasm in flashes of light but tends to linger for long
patients with migraines, at least in some periods of time. Ischemia and retinal diseases
cases.12 The prevalence of migraine in a series cause symptoms that are strictly monocular,
of patients with variant-type angina (coronary not homonymous as in migraine.
artery spasm) was found to be 26%, vs 6% in Partial seizures may cause repetitive
patients with typical coronary artery disease stereotypic symptoms similar to auras but do
and 10% in noncoronary controls.13 not occur initially in an older person unless a
Two rare conditions, cyclic vomiting and brain lesion exists (eg, from trauma, vascular
abdominal migraine, arise mainly in children, disease, neoplasm). A history of months or
especially those with a family history of years of repetitive transient symptoms without
migraine. Most children diagnosed with either evidence of permanent deficits would suggest
of these conditions develop migraine later in that such episodes are migrainous.
life. Before diagnosing these conditions, any Seizures hit very quickly, and if there is
possible underlying disease must be excluded numbness or tingling, it very rapidly spreads
by examination and testing. over a limb in a few seconds. Migrainous

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paresthesias generally take a few minutes to For patients who want treatment, a few
spread up a limb. The neurological symptoms medications can be tried. However, it is diffi-
in a seizure disorder are much shorter than cult to shorten attacks because they are so
those of migraine. brief and require very fast-acting agents.
Recurrent emboli to the brain can cause Isoproterenol, an inhaled beta agonist,
transient visual or neurological symptoms, but may shorten the aura.15
it would be unusual for the same pattern of Vasodilators. With the recent evidence
symptoms to recur many times. Clotting dis- that aura is caused by spreading neuronal
orders, polycythemia, thrombocytosis, and depression rather than significant cerebral
vasculitis may cause transient visual or neuro- ischemia, it is possible that vasodilating drugs
logical disturbances and need to be excluded have other effects in addition to their effects
by the appropriate blood tests. on vessels. Although sometimes useful for
treating migraine headache, vasodilators often
■ EVALUATION exacerbate the pain of migraine if taken at
times other than very early in the aura.16
If a patient has had recurring symptoms for Sublingual nitroglycerine has been useful
some time that are typical of migraine aura in a few of my patients who travel a lot and
but has no deficits found on the physical or feared an attack of visual loss while driving.
neurological examination, a complete workup Sublingual nifedipine has also been help-
with laboratory and imaging tests is probably ful but is no longer used because of the risk of
not necessary. profound hypotension.
However, a complete evaluation should Rapid-acting nonsteroidal anti-inflam-
be done if a patient is seen after having only matory drugs such as meclofenamate or
one or two attacks. naproxen are occasionally effective in short-
Magnetic resonance imaging should be ening the duration of symptoms.
done to rule out a cerebral infarction or a mass Triptans should not be used to treat an
lesion. aura. Oral triptans do not act fast enough to
Magnetic resonance angiography and affect an aura, and if the rapid-acting injectable Generally,
vascular ultrasonography should also be done sumatriptan is given during the aura, it may not auras without
to evaluate the intracranial and extracranial abort a subsequent headache.17 Because they
vessels for stenotic lesions, due either to arte- may cause vascular constriction, triptans need headache
riosclerosis or a vasculitis. to be used with great caution in older patients require no
Electroencephalography is needed if the who may have vascular disease, hypertension,
attacks are not typical of migraine aura and or other cardiovascular risk factors. treatment
might be due to epilepsy.
Laboratory testing for clotting disorders Preventive therapy
and inflammatory vascular disease may also be Preventive therapy should be offered if attacks
necessary. are frequent enough or severe enough to cause
disability.
■ TREATMENT Calcium channel blockers, particularly
verapamil, are often very effective.
Generally, auras without headache do not Antiepileptic drugs such as valproic acid,
occur frequently and require no treatment. gabapentin, and topiramate are also used and
Once the diagnosis is made, patients can be are effective in reducing the frequency and
reassured that the condition is benign. severity of auras without headache.18

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