Childhood Migraine Variants

Updated: Nov 19, 2019
  • Author: Wendy G Mitchell, MD; Chief Editor: Stephen L Nelson, Jr, MD, PhD, FAACPDM, FAAN, FAAP, FANA  more...
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Presentations of migraine in children may be similar to adult presentations and may include headache, with or without aura, accompanied by nausea, vomiting, photophobia, and relief with sleep. However, several variations of migraine are unique to children and rarely if ever occur in adults. In young children, migraine may present with prominent nonheadache symptoms (migraine without headache, or acephalalgic migraine), or neurologic symptoms (aura) may be much more prominent than the headache.

Previously called "childhood periodic syndromes that are commonly precursors of migraine" in International Headache Classification of Headache Disorders (ICHD)-II, these disorders were renamed "episodic syndromes that may be associated with migraine" in ICHD-3. [1]  Although historically noted to occur in childhood, they may also occur in adults.

Recognized childhood syndromes assumed to be pathophysiologically related to migraine include the following:

  • Benign paroxysmal vertigo of childhood

  • Abdominal migraine

  • Cyclic vomiting of childhood

  • Acute confusional migraine (acute confusional state)

  • Paroxysmal torticollis

  • Infant colic (epidemiologic association with migraine)

Basilar migraine (particularly in adolescent girls) may present with prominent dizziness and near-syncope or syncope, with or without a subsequent headache. Hemiplegic migraine (usually an autosomal dominant disorder) may present in early childhood and occasionally may continue into adulthood. Ophthalmoplegic migraine also may occur in childhood.

Evidence suggests that infant colic may be an early-life expression of migraine. Epidemiologic associations are found between both infant colic and later migraine in the child, and maternal migraine. [2, 3]

Migraine variants may cause significant disability from loss of school time for the child, loss of work time for parents, and general disruption of family function.


Pathophysiology and Etiology

Although migraine and variants of migraine have long been assumed to have a vascular etiology, increasing evidence points to underlying primary neurologic causes. Some forms of migraine are genetic. Specific markers on chromosome 19 were found in some families with hemiplegic migraine. Mitochondrial abnormalities, either from autosomal or mitochondrial DNA, may play a contributing role. Migraine, in general, may have a genetic predisposition with environmental and systemic triggers. Hemiplegic migraine may be autosomal dominant. Common triggers reported by patients include stress, bright light, intense emotional influences, and too much or too little sleep. [4]

Mitochondrial abnormalities (maternally inherited via mitochondrial DNA, recessively inherited via chromosomal DNA, sporadic) may account for some cases of abdominal migraine or cyclic vomiting of childhood. [5] There is increasing evidence that several mitochondrial DNA polymorphisms are associated both with cyclic vomiting syndrome and migraine without aura. [6] In subjects with mitochondrial disorders, fasting or systemic stress such as fever or illness may precipitate episodes.

As information from genetic diagnosis and whole exome sequencing rapidly expands, a number of channelopathy-related genetic syndromes have been found to include variable combinations of epilepsy syndrome, familial hemiplegic migraine, paroxysmal vertigo of childhood, episodic ataxia, and paroxysmal dyskinesias. For example, mutations in the SCN1a gene, originally associated with several epilepsy syndromes, are occasionally reported in familial hemiplegic migraine. [7] Similarly, mutations in PRRT2 [8, 9] or the ATP1A2 gene [10] may present with variable phenotypes, including benign familial infantile seizures, paroxysmal choreoathetosis, episodic ataxia, and/or hemiplegic migraine.



Benign paroxysmal vertigo of childhood, sometimes considered a migraine variant, generally presents in toddlers. Acute confusional migraine generally presents in the elementary school years. Less commonly, children can present either in the preschool years or in early adolescence. First attacks during the postpubertal teenage years are rare, although episodes may continue beyond puberty. Hemiplegic migraine may present in early childhood. Basilar migraine, particularly with syncope, often presents in the early teenage years.

In contrast to female predominance in adults, the overall frequency of migraine headaches in childhood is slightly higher in boys than in girls. Frequency of migraine variants is not known to vary between the sexes.

Epidemiological studies have linked infant colic with later migraine in children, suggesting that infant colic, a disorder in which there is apparently paroxysmal irritability and pain, may be a migraine variant as well. [3] In addition, maternal migraine history is epidemiologically associated with a higher incidence of infant colic, consistent with the assumption that tendency to migraine is inherited. [2]


Patient Education

For children with migraine variants, as for all migraine patients, education is an important part of care. Teach patients and families appropriate means of avoiding and managing attacks. Instruct the patient and the parents to keep a detailed diary of episodes, food consumed, activities, and medications. The goal is to identify avoidable precipitants, assess attack patterns, and determine the response to treatment.

Making a specific diagnosis that episodes are migrainous in origin may be quite helpful. Often families are sufficiently relieved to know that the child does not have a more serious condition (eg, a brain tumor) and that further medical intervention may not be necessary.

For patient education resources, see the Headache Center, as well as Causes and Treatments of Migraine and Related Headaches, Alternative and Complementary Approaches to Migraine and Cluster Headaches, Migraine Headache in Children, and Understanding Migraine and Cluster Headache Medications.