Donald W.Lewis, MD
Migraine is a chronic, progressive, and debilitating disorder that has an impact on the
lives of millions of individuals. The origins of the disability can be traced into childhood
and adolescence for most adult migraine sufferers.1Accurate diagnosis and aggres-
sive treatment interventions during childhood and adolescence are essential to
prevent the decades of suffering and diminished quality of life that are directly attribut-
able to migraine. Adequately addressing migraine during adolescence has as much
importance on the patient’s overall well-being as providing immunizations and weight
Diagnosing migraine in children can be a particular challenge. The clinical manifes-
tations of migraine vary widely through childhood because the disorder may be
expressed differently or incompletely. Mimickers of migraine alsoemerge during child-
hood to complicate the diagnostic landscape. Such entities as mitochondrial or
metabolic disorders, epilepsy syndromes, vascular disorders, and congenital malfor-
mations may present with episodic symptoms, including headache. In addition, the
medical history can be limited by the child’s inability to articulate the symptoms,
coupled with parental interpretation, distortion, and editorial. Furthermore, children
are often brought for medical evaluation at the onset of transient neurologic, auto-
nomic, gastrointestinal, or visual symptoms, before the characteristic recurrent
pattern is established, and, curiously, headache may not be the primary symptom.
The key aspect to recognizing the spectrum of migraine in children is to appreciate
that migraine is an episodic disorder separated by symptom-free intervals.
The purpose of this article is to review the clinical manifestations and management
options for migraine in children and adolescents, with an emphasis on those entities
peculiar to young children. All pharmacologic comment is ‘‘off-label.’’
Headaches are common during childhood. Bille’s landmark epidemiologic survey
conducted in the 1950s in Sweden of 6000 school children found that the prevalence
of generic ‘‘headache’’ ranged from 37% to 51% in 7-year-old children and gradually
The author has received research grant support from Ortho McNeil Neurologics, Abbott
Laboratories, Glaxo Smith Kline, Merck, Astra Zeneca, and American Home Products.
Department of Pediatrics, Children’s Hospital of The King’s Daughters, Eastern Virginia Medical
School, 601 Children’s Lane, Norfolk, VA 23507, USA
E-mail address: email@example.com
? Migraine ? Headache ? Children ? Adolescents
Neurol Clin 27 (2009) 481–501
0733-8619/08/$ – see front matter ª 2009 Elsevier Inc. All rights reserved.
rose to 57% to 82% by adolescence.2Frequent or recurring patterns of headache, of
which migraine represents a significant subset, occurred in 2.5% of 7-year-olds and in
up to 15% of 15-year-olds.
Subsequent epidemiologic studies have found that the prevalence of migraine
headache steadily increases through childhood, peaking in adolescence. Depending
on the diagnostic criteria used, the prevalence increases from 3% in the preschool
years, to 4% to 11% by the elementary school years, and then up to 8% to 23% during
the high school years. Before puberty, boys have more headaches than girls, but after
puberty, migraine headaches occur more frequently in girls.3–5
The incidence of migraine peaks earlier in boys than in girls.6The mean age of onset
of migraine is 7 years for boys and 11 years for girls; the gender ratio also shifts during
the adolescent years (Table 1). The incidence of migraine with aura peaks earlier than
the incidence of migraine without aura.5–13
Classification of Pediatric Migraine
The International Classification of Headache Disorders for migraine is shown in Box 1
and is available on-line (14).
There are three primary groups:
1. Migraine without aura (formerly common migraine)
2. Migraine with aura (formerly classic migraine)
3. Childhood ‘‘periodic syndromes’’ that are commonly precursors of migraine
Notably absent in the 2004 classification system are several clinical entities peculiar
to childhood, such as ‘‘Alice in Wonderland’’ syndrome, benign paroxysmal torticollis,
confusional migraine, and ophthalmoplegic migraine (OM), which are discussed for
This is the most frequent form of migraine in children and adolescents (60%–85% of
cases). The diagnostic criteria are shown in Box 2 and include three modifications to
increase sensitivity of diagnosis for children: brief duration (1–72 hours), bilateral or
bifrontal location (age <15 years), and the inference of photophobia and phonophobia
by behavioral response rather than verbal report.
The key feature of migraine with aura in children is episodes of intense disabling
headache separated by symptom-free intervals. The criteria require at least five
distinct attacks lasting 1 to 72 hours and permit attacks to be briefer than in adults
(range: 4–72 hours). The location of the pain may be unilateral or, in children younger
than 15 years of age, bilateral (bifrontal or bitemporal). The quality of pain is typically
pulsing or throbbing, a symptom that may require specific questioning in young chil-
dren. By definition, the pain is moderate to intense and aggravated by routine physical
activity, such as walking or climbing stairs. The accompanying associated autonomic
Prevalence of migraine headache through childhood
Gender ratioBoys > girlsBoys 5 girlsGirls > boys
Migraine without aura
Migraine with aura
Typical aura with migraine headache
Typical aura with nonmigraine headache
Typical aura without headache
Familial hemiplegic migraine
Sporadic hemiplegic migraine
Childhood periodic syndromes that are commonly precursors of migraine
Benign paroxysmal vertigo of childhood
Complications of migraine
Persistent aura without infarction
Diagnostic criteria forpediatricmigrainewithout aura
A. At least five attacks fulfilling criteria B through D
B. Headache attacks lasting 1 to 72 hours
C. Headache has at least two of the following characteristics:
1. Unilateral location, which may be bilateral or frontotemporal (not occipital)
2. Pulsing quality
3. Moderate or severe pain intensity
4. Aggravation by or causing avoidance of routine physical activity (eg, walking, climbing
D. During the headache, at least one of the following:
1. Nausea or vomiting
2. Photophobia and phonophobia, which may be inferred from a child’s behavior
E. Not attributed to another disorder
features (nausea, vomiting, photophobia, and phonophobia) may be as disabling as
the pain. The latter two features may be inferred by the patient’s behavior if the child
withdraws to a quiet dark place during the attack. The International Headache Society
(IHS) criteria wisely also state that the headache must ‘‘not attributed to another
disorder,’’ implying that the prudent physician should carefully consider other possible
causes for the recurrent headaches.
MIGRAINE WITH AURA
The disorders within the migraine with aura spectrum reflect the concept that the focal
symptoms, such as visual disruptions, hemiparesis, and aphasia, are manifestations of
the regional neuronal depolarization and oligemia caused by cortical spreading depres-
sion (CSD). Clinical entities of childhood with focal neurologic symptoms, previously
termed migraine variants, such as hemiplegic and basilar type, now are included within
this category of migraine with aura.
Approximately 15% to 30% of children and adolescents who have migraine report
visual disturbances, distortions, or obscurations before, or as, the headache begins.
The visual symptoms begin gradually and last for several minutes (typical aura). The
most frequent forms are binocular visual impairment with scotoma (77%), distortion
or hallucinations (16%), and monocular visual impairment or scotoma (7%).15Formed
illusions (eg, spots, balloons, colors, rainbows) or other bizarre visual distortions (eg,
Alice in Wonderland syndrome) may be described, albeit infrequently.
Sudden images and complicated visual perceptions should prompt consideration of
benign occipital epilepsy, specifically Panayiotopoulos syndrome.16Transient visual
obscurations may also be described with idiopathic intracranial hypertension; thus,
not all visual symptoms with headache are attributable to migraine with aura.
Basilar-type migraine (BM) represents 3% to 19% of childhood migraine and has
a mean age onset of 7 years. Attacks are characterized by episodes of dizziness,
vertigo, visual disturbances, ataxia, or diplopia as the aura, followed by the headache
phase. The pain of BM may be occipital in location, unlike the usual frontal or bitem-
poral pain of typical migraine. The diagnostic criteria require two or more symptoms
and emphasize bulbar and bilateral sensorimotor features (Box 3). Familiar forms of
BM linked to the same genes as familial hemiplegic migraine (FHM), types 1 and 2,
have recently been reported.17
FAMILIAL HEMIPLEGIC MIGRAINE
No form of migraine has yielded more information about the underlying molecular
genetics of migraine than FHM. FHM, type 1, is an uncommon autosomal dominant
form of migraine with aura caused by a missense mutation in the calcium channel
gene (CACNA1A) linked to chromosome 19p13. Clinically, FHM is a migraine head-
ache heralded by an aura that has ‘‘stroke-like’’ qualities, producing some degree
of hemiparesis (Box 4). The transient episodes of focal neurologic deficits precede
the headache phase by 30 to 60 minutes but, occasionally, extend well beyond the
headache itself (hours to days). The location of headache is often (but not invariably)
contralateral to the focal deficits. Many children and adolescents report transient
somatosensory symptoms heralding an attack with focal paresthesias around the
mouth and hand (eg, chiro-oral) without weakness; this does not fulfill the criteria for
hemiplegic migraine. Genetic testing is commercially available for FHM type 1.
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