eMedicine Specialties > Neurology > Pediatric Neurology

Childhood Migraine Variants

Author: Wendy G Mitchell, MD, Professor of Neurology, University of Southern California School of Medicine; Consulting Staff, Division of Child Neurology, Children's Hospital Los Angeles, Los Angeles County-University of Southern California
Contributor Information and Disclosures

Updated: Feb 5, 2009

Introduction

Background

Migraine in children may be similar to adult presentations and 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. Migraine may present with prominent nonheadache symptoms in young children (migraine without headache), or neurologic symptoms (aura) may be much more prominent than the headache.

Various recognized childhood syndromes assumed to be pathophysiologically related to migraine include benign paroxysmal vertigo of childhood, abdominal migraine, cyclic vomiting of childhood, and acute confusional migraine (acute confusional state). Basilar migraine (particularly in adolescent girls) may present with prominent dizziness and near-syncope and/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.

Pathophysiology

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 contribute particularly to the cyclic vomiting syndrome.

Sex

  • 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.

Age

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.

Clinical

History

Variant migraine episodes may be independent of actual head pain. Other symptoms may predominate and be significantly more troublesome.

  • Hemiplegic migraine
    • Hemiplegic migraine presents with hemiplegia or hemiparesis, with or without a speech and/or language disturbance, which clears in minutes to hours.
    • Headache may be less dramatic than the hemiplegia. Other migrainous symptoms such as nausea, vomiting, and photophobia are present variably. Hemiplegia may precede or accompany the headache.
    • This variant is often familial, dominantly inherited, and linked to chromosome 19 in some studies. An alternative locus has been described on chromosome 1. A defect in the gene for the calcium channel is documented in some families (affecting the voltage-dependent P/Q-type calcium channel alpha-1A [CACNA1A] subunit). An increased risk of stroke exists in families with this disorder. Cerebral autosomal dominant arteriopathy and subcortical infarcts and leukoencephalopathy (CADASIL), an autosomal dominant disorder in which multiple subcortical strokes may lead to dementia in early adulthood, sometimes presents in adolescence as atypical hemiplegic migraine. The CADASIL mutation also localizes to chromosome 19.
    • Diagnosis cannot be made during the first episode, although it may be suspected in the presence of a positive family history. Exclude more serious causes of headache with hemiplegia and/or hemiparesis including intracranial hemorrhage, mass, infection, or stroke. With repeated stereotyped episodes and complete clearing between episodes, the diagnosis can be made with more confidence, particularly in the presence of a positive family history.
    • Differential diagnosis of repeated episodes includes alternating hemiplegia of childhood, unobserved partial seizures with postictal paralysis, and mitochondrial cytopathies, particularly the mitochondrial encephalomyopathy, lactic acidosis, and stroke syndrome.
  • Confusional migraine
    • Although this variant is more common in younger children, it sometimes presents in postpubertal adolescents or adults. Occasionally, a child whose episodes began in the prepubertal years continues to have episodes into adolescence. The child has a period of confusion and disorientation, with or without agitation, followed by vomiting, which is relieved by sleep. Headache may not be prominent or may be elicited only retrospectively.
    • Making the diagnosis during the first episode is difficult, and it can be made only after the episode has resolved fully.
    • Acute differential diagnosis of a single episode includes all types of encephalopathy and/or encephalitis, toxic ingestion, intoxication, and/or an unobserved seizure with postictal agitation.
  • Abdominal migraine
    • The child may complain of episodic pain, nausea, and vomiting. The headache may be minimal or absent. An aura may precede the pain but is not frequent. Symptoms are relieved by sleep and antiemetic and/or antimigraine therapies.
    • Cyclic vomiting of childhood, which can be associated with a mitochondrial cytopathy, may be a severe variant of abdominal migraine.
    • Diagnosis is difficult to make during the first episode.
  • Basilar migraine
    • The patient may have an aura followed by dizziness, vertigo, syncope, and dysarthria. Headache may be minimal or absent.
    • It is observed most frequently in adolescent girls.
    • Differential diagnosis includes cardiogenic or vasovagal syncope, inner ear disease, and posterior fossa tumors.
  • Migraine aura without headache
    • Migraineurs of any age may experience an aura with or without the typical headache. In some, the headache may be minimal while neurologic symptoms predominate.
    • Visual symptoms without subsequent headache are fairly frequent. These include scintillating scotomata, formed visual hallucinations (usually stereotyped, in a single visual field), micropsia, and tunnel vision.
    • Differential diagnosis includes occipital epilepsy, with or without an identifiable lesion. If episodes never are accompanied by headache, the diagnosis is speculative.
    • Auditory hallucinations as migraine auras are infrequent but can occur.
    • Sensory dysesthesias (usually hemisensory numbness and/or tingling) similar to more typical migraine aura may occur without subsequent head pain.
  • Benign paroxysmal vertigo of childhood
    • Based on a high proportion of children with a family history of migraine, it is sometimes considered a migraine variant. This syndrome is not uncommon and frequently is followed by the development of more typical migraine headaches later in childhood.
    • This syndrome presents with brief episodes of vertigo.
    • Toddlers may be unable to verbalize the symptoms but typically cling to the parent and look frightened.
    • Older children often verbalize that they are "moving."
    • Headache does not follow the attack.
  • Ophthalmoplegic migraine
    • This migraine variant may begin in childhood.
    • Acute disorders of eye movement, unilateral abnormal pupillary response, or Horner syndrome may precede or accompany the headache.

Physical

  • Typically, as with all vascular headache syndromes, the neurologic examination between episodes is completely normal.
  • If persistent neurologic signs (eg, hemiparesis, visual changes, sensory loss) are evident between episodes, strongly consider and investigate alternative diagnoses.

Causes

  • Migraine, in general, may have a genetic predisposition with environmental and systemic triggers.
  • Hemiplegic migraine may be autosomal dominant.
  • 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.
  • In subjects with mitochondrial disorders, fasting or systemic stress such as fever or illness may precipitate episodes.

More on Childhood Migraine Variants

Overview: Childhood Migraine Variants
Differential Diagnoses & Workup: Childhood Migraine Variants
Treatment & Medication: Childhood Migraine Variants
Follow-up: Childhood Migraine Variants
References
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Further Reading

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Keywords

abdominal migraine, acute confusional migraine, basilar migraine, benign paroxysmal vertigo of childhood, cyclic vomiting of childhood, hemiplegic migraine, migraine, migraine aura without headache, ophthalmoplegic migraine, vascular headache, childhood migraine variants, migraine in children

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Contributor Information and Disclosures

Author

Wendy G Mitchell, MD, Professor of Neurology, University of Southern California School of Medicine; Consulting Staff, Division of Child Neurology, Children's Hospital Los Angeles, Los Angeles County-University of Southern California
Wendy G Mitchell, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, American Epilepsy Society, Child Neurology Society, and International Child Neurology Association
Disclosure: Questcor Honoraria Consulting

Medical Editor

James J Riviello Jr, MD, George Peterkin Endowed Chair in Pediatrics, Professor of Pediatrics, Section of Neurology and Developmental Neuroscience, Professor of Neurology, Peter Kellaway Section of Neurophysiology, Baylor College of Medicine; Chief of Neurophysiology, Director of the Epilepsy and Neurophysiology Program, Texas Children's Hospital
James J Riviello Jr, MD is a member of the following medical societies: American Academy of Pediatrics
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Kenneth J Mack, MD, PhD, Senior Associate Consultant, Department of Child and Adolescent Neurology, Mayo Clinic
Kenneth J Mack, MD, PhD is a member of the following medical societies: American Academy of Neurology, Child Neurology Society, Phi Beta Kappa, and Society for Neuroscience
Disclosure: Nothing to disclose.

CME Editor

Selim R Benbadis, MD, Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, University of South Florida School of Medicine, Tampa General Hospital
Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association
Disclosure: Nothing to disclose.

Chief Editor

Amy Kao, MD, Assistant Professor, Department of Pediatrics, Division of Pediatric Neurology, Department of Neurology, Oregon Health and Science University; Consulting Staff, Shriners Hospital for Children
Amy Kao, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, American Epilepsy Society, and Child Neurology Society
Disclosure: Nothing to disclose.

 
 
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