Types of migraine and related syndromes in children

INTRODUCTION — Migraine is a group of primary headaches characterized by recurrent episodes typically consisting of pulsatile headaches often preceded by symptoms such as photophobia, phonophobia, nausea, and/or vomiting. Migraine is the most common acute and recurrent headache disorder in children. Specific forms of migraine and other related conditions may be classified according to distinctions in clinical features.

This topic will review the types of migraine and associated syndromes in children. Other aspects of migraine in children are discussed separately. (See "Pathophysiology, clinical features, and diagnosis of migraine in children" and "Acute treatment of migraine in children".)

TERMINOLOGY AND CLASSIFICATION — The terminology used to identify different migraine subtypes has evolved along with progress in understanding the various conditions and updates in the classification system used by the International Headache Society (IHS). The 1988 classification of headaches by the IHS replaced the terms "classic" and "common" for migraine with "with" and "without aura" [1]. In subsequent revisions of the IHS headache classification, the term "complicated migraine" was replaced by "complications of migraine" [2]. In addition, some syndromes unique to children who typically have a family history of migraine were reclassified as "periodic syndromes of childhood" or "episodic syndromes that may be associated with migraine" [3]. These include cyclical vomiting, abdominal migraine, benign paroxysmal vertigo of childhood, and benign paroxysmal torticollis.

Some of the other disorders discussed here, including Alice in Wonderland syndrome and confusional migraine, are not addressed in the International Classification of Headache Disorders system [3]. (See 'Other pediatric migraine variants' below.)

Other syndromes are no longer classified with migraine, despite older terminology. As an example, "ophthalmoplegic migraine" has been replaced by "recurrent painful ophthalmoplegic neuropathy" because symptoms are due to cranial neuralgia, rather than migraine. (See 'Associated nonmigrainous conditions' below.)

MIGRAINE WITHOUT AURA — Migraine without aura is a recurrent headache disorder characterized by attacks lasting 2 to 72 hours in children (table 1). Typical features include a headache of moderate to severe intensity with a pulsating quality of pain that is associated with nausea, photophobia, or phonophobia and is aggravated with routine physical activity [3]. Photophobia and phonophobia may be inferred based on behavior in young children unable to describe or name these symptoms.

Bilateral head pain is common in children with migraine. Unilateral headache is not typical of migraine until after puberty. In children, the headache is more often described as bifrontal or across the front or middle of the forehead, bitemporal, or generalized rather than unilateral. Posterior headache is unusual and should prompt a work-up for the possibility of a posterior fossa lesion. (See "Pathophysiology, clinical features, and diagnosis of migraine in children", section on 'Migraine headache phase'.)

MIGRAINE WITH AURA — Migraine with aura is a recurrent headache disorder characterized by attacks of transient neurologic symptoms usually followed by headaches (table 1). Symptoms last for 2 to 72 hours, and the character of the typical headache is also the same as is as described for migraine without aura. (See 'Migraine without aura' above.)

Auras are progressive neurologic deficits or disturbances associated with the headache. They often last for several minutes but may be longer. They typically precede but also may follow the onset of a headache. Symptoms of a migraine aura may be visual, sensory, speech and/or language, motor, brainstem, or retinal [3]. (See "Pathophysiology, clinical features, and diagnosis of migraine in children", section on 'Migraine aura'.)

Visual disturbances are the most common type of neurologic symptoms associated with migraine. Bilateral visual symptoms are less typical in children and adolescents than adults [3]. Focal numbness and tingling are the second-most common type of aura and usually involve the lips, lower face, and fingers of one hand (cheiro-oral). Less frequently, migraine aura causes speech, language, motor, brainstem, or retinal deficits. Some patients have several types of aura symptoms that vary with attacks. When the aura involves motor weakness along with visual, sensory, or speech impairment, the disorder is classified as hemiplegic migraine. (See 'Hemiplegic migraine' below.)

Migraine with aura may be subclassified by the specific symptoms that characterize the aura.

Typical aura — A migraine with typical aura consists of symptoms restricted to visual, sensory, speech, or language domains, or a combination of these. Less common motor, brainstem, or retinal symptoms are not a feature of migraine with typical aura (table 1) [3]. Additional features characterizing a typical aura include gradual development, duration no longer than one hour, and complete reversibility.

Migraine aura without headache — Some children may report typical or other aura symptoms followed by mild or even no headache. When isolated aura symptoms involve visual, sensory, speech, or language domains, or a combination of these, the syndrome is typical aura without headache [3]. Migraine aura without headache is uncommon, reported in one pediatric neurology practice in less than 3 percent of children with migraine [4]. Most patients were female and had a family history of migraine.

Isolated aura symptoms may be difficult to identify as migrainous, especially before a pattern of recurrence has been established or when symptoms are not typical. Evaluation for alternative causes of transient neurologic symptoms is warranted for patients suspected of having migraine aura without headache. (See "Pathophysiology, clinical features, and diagnosis of migraine in children", section on 'Differential diagnosis'.)

Migraine with brainstem aura — Migraine with brainstem aura, formerly known as basilar-type migraine, is a rare form of migraine characterized by aura symptoms of brainstem dysfunction, but motor weakness is not present [3]. The auras consist of two or more brainstem symptoms such as vertigo, dysarthria, tinnitus, diplopia, bilateral paresthesias, decreased level of consciousness, or hypacusis (table 2). Onset is usually between ages 7 and 20 and is more common in females. Attacks may evolve to more typical forms of migraine with age.

Symptoms of migraine with brainstem aura overlap with other migraine syndromes with clinical features that involve the brainstem. This commonly includes vestibular migraine and hemiplegic migraine. Patients with an aura that consists of isolated vertigo without other brainstem symptoms may be characterized as having vestibular migraine or other forms of vertigo. Those with a migraine aura consisting of limb weakness along with brainstem symptoms are classified as having hemiplegic migraine. Approximately 60 percent of patients with familial hemiplegic migraine have basilar-type symptoms [5]. (See 'Vestibular migraine of childhood' below and 'Hemiplegic migraine' below.)

Migraine with brainstem aura is discussed in greater detail separately. (See "Migraine with brainstem aura".)

Hemiplegic migraine — The primary feature that separates hemiplegic migraine from other types of migraine with aura is the presence of motor weakness as a key manifestation of aura in the attacks. In addition to weakness, the aura in hemiplegic migraine also includes visual, sensory, and/or language disturbance [3]. Symptoms associated with migraine with brainstem aura such as vertigo or diminished consciousness may also occur in some patients. Some patients report prolonged auras lasting several hours [6]. Most patients report headache with or after onset of the aura. Attacks can recur for many years [7], but overall prognosis is favorable with waning frequency of attacks over time. Hemiplegic migraine may occur as a familial or sporadic form.

Hemiplegic migraine is discussed in greater detail separately. (See "Hemiplegic migraine".)

Retinal migraine — Retinal migraine is a rare disorder characterized by a sudden loss of vision or the perception of bright light (photopsia) or scintillations in one eye only [8]. The aura spreads gradually and lasts 5 to 60 minutes; the monocular visual aura is usually accompanied by or followed within one hour by headache [3]. The headache is typically ipsilateral and periorbital. Visual symptoms may occur without headache. Vision usually recovers, although permanent visual loss may occur. (See "Pathophysiology, clinical manifestations, and diagnosis of migraine in adults", section on 'Retinal migraine'.)

Although classified as retinal migraine [3], some experts prefer the term "ocular migraine," since both retinal and ciliary circulations may be involved [9].

CHRONIC MIGRAINE — Some patients with an episodic migraine pattern whose headaches become progressively more frequent transition to a chronic migraine. Chronic migraine is defined by headache that occurs on ≥15 days per month for more than three months, with features of migraine headache on at least 8 days per month [3]. Patients may have migraines with or without aura (table 3). Many patients with chronic migraine have daily or near-daily headaches of low to moderate severity, associated with less prominent migrainous features. Superimposed on this baseline are exacerbations of pain with more prominent migrainous features such as photophobia, phonophobia, osmophobia, nausea, vomiting, and cutaneous allodynia.

Chronic migraine is discussed in greater detail separately. (See "Chronic migraine".)

OTHER PEDIATRIC MIGRAINE VARIANTS — Some variant forms of migraine occur primarily in children or in both children and adults. These variants have been described in case reports and series but characterizations and their relationships to other forms of migraine are incompletely understood.

Alice in Wonderland syndrome — Alice in Wonderland syndrome is characterized by episodes of visual hallucinations, bizarre perceptual distortions (eg, of body image), or impairments of time sense that may accompany or precede a headache [10,11]. The child is not frightened and can describe the experience without amnesia. In one series of 48 patients, the most common complaints were visual misperceptions including [12]:

●Micropsia (objects appear too small)

●Teleopsia (objects appear farther away)

●Macropsia (objects appear too large)

●Metamorphopsia (objects have distorted shapes)

The perception disorder typically lasts for several days but sometimes can persist for several months [13]. However, children typically recover without sequelae. The syndrome can affect patients of any age, although it is more common in childhood and adolescence [11,14]. Some investigators reserve the term "Alice in Wonderland syndrome" to signify a perceptual disorder involving altered body image and prefer the term "Alice in Wonderland–like syndrome" to signify an extrapersonal perceptual disorder involving vision, time, or levitation [11]. However, this distinction is not settled in the literature.

The cause of Alice in Wonderland syndrome is uncertain. Migrainous ischemia and cortical irritability is a proposed etiology [14], although others have questioned this [12,15]. The Alice in Wonderland syndrome has also been described in patients with viral encephalitis, epilepsy, Epstein-Barr virus, other infections, and head trauma, among others [11,12]. In many cases, no obvious cause or association is identified.

Confusional migraine — Confusional migraine is described in patients with a headache with migrainous features associated with symptoms of agitation, disorientation, and aphasia that last longer than the headache.

The clinical features of confusional migraine were described in a series of 13 patients who were hospitalized for migraine [16]. Children ranged in age from 6 to 15 years (mean 10.8 years), and males predominated (11 males, 2 females). The duration of confusion was 2 to 24 hours. The history was positive for mild head trauma (4 patients), agitation (8 patients), past headache (7 patients), and migraine on the maternal side of the family (10 patients).

Some overlap exists between confusional migraine and transient global amnesia, Alice in Wonderland syndrome, and trauma-induced migraine. Confusional migraine may evolve in some cases to a more typical form of migraine [17].The differential diagnosis also includes toxic or infectious encephalopathies and seizures.

Estrogen-associated migraine — Menstrual migraine (also called catamenial migraine) occurs in close temporal relationship to the onset of menstruation; this time period usually encompasses two days before through three days after the onset of menses [3]. Females with migraine that also occurs at other times during the month are classified as having menstrually related migraine (table 4). Menstrual migraine is usually not associated with aura even in those who have migraine with aura at other times. Menstrual migraine is common, occurring in up to 70 percent of adolescent females with a history of headache [18,19].

Menstrual migraine and menstrually related migraine are discussed in detail separately. (See "Estrogen-associated migraine headache, including menstrual migraine".)

COMPLICATIONS OF MIGRAINE — Migrainous attacks are typically self-limited. However, complications may occur with severe attacks. Complications of migraine include the following [3]:

●Status migrainosus – A debilitating migraine attack lasting more than 72 hours

●Migrainous infarction – Ischemic infarction on neuroimaging in a territory that correlates with one or more migraine aura symptoms

●Persistent aura without infarction – Aura symptoms persisting for one week or more without neuroimaging evidence of infarction

●Migraine aura–triggered seizure – Seizure triggered by an attack of migraine with aura

Migrainous infarction is discussed separately. (See "Migraine-associated stroke: risk factors, diagnosis, and prevention", section on 'Diagnostic criteria'.)

EPISODIC SYNDROMES THAT MAY BE ASSOCIATED WITH PEDIATRIC MIGRAINE — Some transient, recurrent pediatric syndromes not established as migraine variants are typically considered as potential precursors of migraine. These include those classified in the International Classification of Headache Disorders (ICHD) as "episodic syndromes that may be associated with migraine" [3,20]. The previously used term for these conditions was "childhood periodic syndromes." Some evidence suggests that they are not only precursors but also manifestations of migraine [20,21].

Recurrent vestibular symptoms — Recurrent episodes of vertigo in children have been associated with migraine [3,20]. These include vestibular migraine and recurrent benign paroxysmal vertigo.

When vestibular symptoms are accompanied by other brainstem symptoms as part of an aura, the syndrome is classified as migraine with brainstem aura. (See 'Migraine with brainstem aura' above.)

Vestibular migraine of childhood — Vestibular migraine is characterized by spontaneous moderate to severe vestibular symptoms (vertigo, dizziness) lasting 5 minutes to 72 hours, with at least half of the episodes having migrainosus features of headache, nausea, vomiting, and/or pallor (table 5) [3]. When the syndrome starts before 18 years of age, it is called vestibular migraine of childhood [20]. It was also known as migraine-associated vertigo/dizziness, migrainous vertigo, and migraine-related vestibulopathy. In young children, dizziness or vertigo may be inferred by observed unsteadiness.

The term "probable vestibular migraine of childhood" is used for patients who meet only some of the criteria for vestibular migraine [20].

Benign paroxysmal vertigo of childhood — Benign paroxysmal vertigo (or recurrent vertigo) of childhood is characterized by brief, recurrent episodes of vertigo. Children may be frightened during episodes and have nausea or may vomit. Gait is unsteady, and nystagmus may be observed. Episodes occur suddenly and typically resolve within minutes but may last up to 72 hours. There is no headache with benign paroxysmal vertigo of childhood, unlike vestibular migraine. It is considered a precursor of migraine [20]. (See "Nonepileptic paroxysmal disorders in children", section on 'Benign paroxysmal vertigo'.)

Recurrent gastrointestinal symptoms — Infants and children with recurrent episodes of gastrointestinal dysfunction may have syndromes that are associated with migraine. These include infantile colic, cyclic vomiting syndrome, and abdominal migraine (table 6).

Infantile colic — Infantile colic is used to describe healthy babies with excessive, frequent crying, irritability, and fussiness since birth. Episodes last ≥3 hours/day, occurring at least three days/week for three or more weeks. The prevalence of infantile colic is approximately 20 percent and is more likely to occur if either parent has migraine. Several etiologies have been associated with infantile colic, including gastrointestinal disturbance and developmental immaturity, as well as migraine. (See "Infantile colic: Clinical features and diagnosis", section on 'Proposed etiologies'.)

Cyclic vomiting syndrome — Cyclic vomiting syndrome (CVS) is a disorder characterized by repeated and usually stereotypical episodes of nausea and vomiting that last for hours to days, separated by symptom-free periods of variable length. CVS shares clinical features with abdominal migraine and other forms of migraine, suggesting a common pathophysiologic process (table 6). CVS may respond to migraine treatments [22]. However, CVS has also been linked in some patients to food allergy and mitochondrial, metabolic, and endocrine disorders.

CVS is discussed in greater detail separately. (See "Cyclic vomiting syndrome".)

Abdominal migraine — Abdominal migraine is characterized by recurrent episodes of abdominal pain in an otherwise healthy child who is normal between attacks [23-25]. The pain is typically midline or poorly localized, moderate to severe in intensity, and associated with at least two additional features such as anorexia, nausea, vomiting, or pallor. Headache is not a prominent feature during attacks and may be overlooked if the child is not asked about it [26]. Photophobia or phonophobia are uncommon. Precipitating factors are often searched for but not commonly identified; concerns often include dietary and psychological stress.

Abdominal migraine is common, affecting up to 4 percent of children [23,24,26], including up to 15 percent of children with chronic periodic abdominal pain [27]. The age at presentation is usually between 2 and 10 years. Most children stop having attacks by early adolescence, but rarely attacks may persist into adulthood. However, most children with abdominal migraine evolve to develop migraine headaches. In one study of 54 children with abdominal migraine who were followed for 7 to 10 years, current or previous migraine headache was identified in 70 percent [28].

Abdominal migraine is a clinical diagnosis; there are no confirmatory diagnostic tests. The history and physical examination show no evidence of gastrointestinal or kidney disease [3]. The many causes of acute abdominal pain need to be considered in the differential diagnosis. Testing is determined by the history and physical examination, but the search for a mechanical process such as obstruction, kidney disorder, infection, or metabolic condition may be in order. (See "Causes of acute abdominal pain in children and adolescents" and "Chronic abdominal pain in children and adolescents: Approach to the evaluation".)

Alternating hemiplegia of childhood — Alternating hemiplegia of childhood is a condition characterized by recurrent hemiplegic attacks of either side occurring in infants, typically before the age of 18 months [29]. Initial clinical features may resemble migraine with motor aura. However, alternating hemiplegic of childhood is a neurodevelopmental disorder typically associated with additional clinical features such as progressive encephalopathy, developmental delay, seizures, oculomotor palsy, and autonomic dysfunction. Some children may also have paroxysmal tonic spells, posturing, and typical movements. The condition is linked to a variant form of the ATP1A3 gene.

Benign paroxysmal torticollis — Benign paroxysmal torticollis of infancy is characterized by periods of an abnormal, sustained posture of the head and neck in which the head tilts to either side, with or without slight rotation. The syndrome begins during infancy with paroxysmal events each lasting minutes to days. Associated symptoms can include pallor, irritability, malaise, vomiting, and ataxia. The child is alert and responsive during an attack. Episodes recur less frequently as the child gets older, resolving by the age of five years in most cases. It is considered to be a migraine equivalent disorder. Some patients go on to develop migraine headaches or benign paroxysmal vertigo of childhood. (See "Acquired torticollis in children", section on 'Benign paroxysmal torticollis'.)

ASSOCIATED NONMIGRAINOUS CONDITIONS — Some conditions that have been associated with migraine due to similar presenting features such as recurrent auras or unilateral headache are now considered distinct entities with nonmigrainous pathophysiologies.

●Visual snow syndrome – Visual snow syndrome is a visual phenomenon reported as flickering dots throughout the visual field of both eyes, reminiscent of the "static" seen on television screens. Although initially described as part of the spectrum of migraine and migraine aura [30], visual snow is now recognized as a separate entity. Other associated symptoms and signs include tinnitus, anxiety, depression, tremor, and gait imbalance. The syndrome has been reported in adults and children [31]. The prevalence in children is unknown, although many adults report symptoms were present in childhood and do not recall ever having seen differently [32]. (See "Approach to the patient with visual hallucinations".)

●Recurrent painful ophthalmoplegic neuropathy (ophthalmoplegic migraine) – Recurrent painful ophthalmoplegic neuropathy, previously called "ophthalmoplegic migraine," is characterized by repeated attacks of paresis of one or more ocular cranial nerves with ipsilateral headache [3]. The third cranial nerve is most often involved, resulting in ptosis, pupillary dilation, exotropia, and diplopia. This condition is considered nonmigrainous. Thickening and enhancement of the cisternal segment of the affected cranial nerve may be detected by magnetic resonance imaging, consistent with a recurrent demyelinating neuropathy rather than migraine [33-35]. (See "Overview of craniofacial pain", section on 'Recurrent painful ophthalmoplegic neuropathy'.)

Other conditions in the differential diagnosis of migraine in children and other sources of craniofacial pain are discussed in detail separately. (See "Pathophysiology, clinical features, and diagnosis of migraine in children", section on 'Differential diagnosis' and "Overview of craniofacial pain".)

SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Migraine and other primary headache disorders".)

INFORMATION FOR PATIENTS — UpToDate offers two types of patient education materials, "The Basics" and "Beyond the Basics." The Basics patient education pieces are written in plain language, at the 5^th to 6^th grade reading level, and they answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials. Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are written at the 10^th to 12^th grade reading level and are best for patients who want in-depth information and are comfortable with some medical jargon.

Here are the patient education articles that are relevant to this topic. We encourage you to print or e-mail these topics to your patients. (You can also locate patient education articles on a variety of subjects by searching on "patient info" and the keyword(s) of interest.)

●Basics topics (see "Patient education: Migraines in children (The Basics)")

●Beyond the Basics topics (see "Patient education: Headache in children (Beyond the Basics)")

SUMMARY

●Migraine without aura – Migraine without aura is a recurrent headache disorder characterized by attacks lasting 2 to 72 hours in children (table 1). Typical features include a unilateral headache of moderate to severe intensity with a pulsating quality of pain that is associated with nausea, photophobia, or phonophobia and is aggravated with routine physical activity. (See 'Migraine without aura' above.)

●Migraine with aura – Migraine with aura is a recurrent headache disorder characterized by attacks of auras usually followed by headaches lasting 2 to 72 hours (table 1). Auras are progressive neurologic deficits or disturbances with subsequent complete recovery. Symptoms of a migraine aura may be visual, sensory, speech and/or language, motor, brainstem, or retinal. They often last for several minutes but may be longer. They may precede or follow the onset of a headache. Some report aura symptoms without headache. (See 'Migraine with aura' above.)

Typical aura symptoms involve visual, sensory, speech, or language domains, or a combination. Other subtypes of migraine with aura are classified by the specific symptoms of the aura:

•Migraine with brainstem aura – Two or more brainstem symptoms such as vertigo, dysarthria, tinnitus, diplopia, bilateral visual symptoms, bilateral paresthesias, decreased level of consciousness, or hypacusis (table 2). Weakness is not present. (See 'Migraine with brainstem aura' above.)

•Hemiplegic migraine – Weakness and at least one other symptom, such as visual symptoms, numbness, vertigo, or diminished consciousness. (See 'Hemiplegic migraine' above.)

•Retinal migraine – Loss of vision or the perception of bright light (photopsia) or scintillations in one eye only. (See 'Retinal migraine' above.)

●Chronic migraine – Chronic migraine is defined by headache that occurs on ≥15 days per month for more than three months, with features of migraine headache on at least eight days per month (table 3). (See "Chronic migraine".)

●Migraine variants in children

•Alice in Wonderland syndrome is characterized by episodes of visual hallucinations, bizarre perceptual distortions (eg, of body image), or impairments of time sense that may accompany or precede a headache. (See 'Alice in Wonderland syndrome' above.)

•Confusional migraine is described in patients with a headache with migrainous features associated with symptoms of agitation, disorientation, and aphasia that last longer than the headache. (See 'Confusional migraine' above.)

•Menstrual migraine occurs in close temporal relationship to the onset of menstruation; this time period usually encompasses two days before through three days after the onset of menses. Females with migraine that also occurs at other times during the month are classified as having menstrually related migraine (table 4). (See 'Estrogen-associated migraine' above.)

●Complications of migraine – Complications may occur with severe migraine attacks, including status migrainosus, migrainous infarction, persistent aura without infarction, or migraine aura–triggered seizure. (See 'Complications of migraine' above.)

●Other migraine-associated episodic syndromes – Some transient, recurrent pediatric syndromes are considered as precursors or other early manifestations of migraine (see 'Episodic syndromes that may be associated with pediatric migraine' above):

•Vestibular migraine of childhood

•Benign paroxysmal vertigo of childhood

•Infantile colic

•Cyclic vomiting syndrome

•Abdominal migraine

•Alternating hemiplegia of childhood

•Benign paroxysmal torticollis

ACKNOWLEDGMENT — The UpToDate editorial staff acknowledges Robert P Cruse, DO, who contributed to earlier versions of this topic review.