Pediatric Episodic Syndromes Associated With Migraine: Knowing What to Look For (Part I)

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Elucidating the source of these syndromes is key to properly treating them.
Elucidating the source of these syndromes is key to properly treating them.
This article is the first in a 2-part series on pediatric episodic syndromes that may be associated with migraine. You can read Part II here, which includes an expert Q&A on diagnosis and treatment.

As many as 9.8% of pediatric patients with migraines also experience episodic syndromes involving symptoms such as gastrointestinal pain, vomiting, or vertigo.1 These debilitating episodes have an abrupt onset followed by complete resolution, and patients are healthy between episodes. They often occur before migraine attacks, although that is not always the case.

"Migraines and the episodic syndromes share autonomic dysfunction as part of the clinical manifestations," wrote the authors of a review on the topic that was published in 2016 in Seminars in Pediatric Neurology.2 "Individuals with migraines can experience autonomic dysfunction without the headache."

Diagnosis and treatment of these disorders, termed "episodic syndromes that may be associated with migraine" in the International Classification of Headache Disorders-3 Beta (ICHD-III beta), can be challenging. Before diagnosis, affected patients may undergo extensive testing and frequent hospitalization, and there is little evidence to guide treatment. 

These disorders are summarized below:

Infant colic. Defined in the ICHD-III beta as crying for at least 3 hours per day on at least 3 days per week over a period of at least 3 weeks, infant colic typically presents around 3 months of age, resolves by approximately 9 months of age, and affects an estimated 5% to 19% of infants.4,5 Although it is commonly believed that colic is caused by abdominal distress, there is scant evidence of this etiology.

Links between infant colic and migraine have been found across a variety of studies. Mothers with migraine were shown to have twice the odds of having an infant with colic in a cross-sectional study, and an increased risk for migraine in individuals with a history of infant colic was found in a recent meta-analysis (odds ratio [OR], 5.6; 95% CI, 3.3-9.5), as well as in a prospective cohort study (risk ratio [RR], 2.7; 95% CI, 1.5-4.7).6-8

Excessive crying is associated with shaken baby syndrome, underscoring the importance of identifying the cause of colic and potential treatments.9

Benign paroxysmal torticollis of infancy. Likely the rarest of the episodic syndromes, this disorder typically begins around age 6 months and resolves by 3 or 4 years of age. It is characterized by periodic bouts of torticollis that last minutes, hours, or days, and may also involve symptoms such as irritability, vomiting, pallor, ataxia, and drowsiness. Affected infants often have a family history of migraine, and some patients have been found to have a mutation of the CACNA1A gene that has been linked to familial hemiplegic migraine.10,11

Benign paroxysmal vertigo. Benign paroxysmal vertigo affects an estimated 2.6% of children aged 5 to 15 years. Onset typically occurs between 1 to 4 years of age, with spontaneous resolution by age 10 to 12 years, although it can persist into young adulthood. The recurrent attacks of vertigo that characterize the disorder typically have a duration of less than 5 minutes but can persist for hours, and they may also involve nausea or vomiting, ataxia, pallor, nystagmus, apparent fearfulness, and headache. Various study results show that 70% of these patients have a family history of migraine, and between one-third and 69% of patients had migraines later in life.12,13

Cyclic vomiting syndrome (CVS). The mean age of onset is 5.2 years in children. The diagnostic criteria for CVS include vomiting that occurs at least 4 times per hour for at least 1 hour. Episodes occur an average of once per month and can last up to 5 days, and they may involve headache. No evidence of gastrointestinal pathology is found in these cases. 

Presentation varies between patients, with some cases requiring hospitalization for rehydration. Patients can range in age from infancy to adulthood, and they often have a personal or family history of migraine.14

Abdominal migraine. This disorder affects an estimated 4.1% of children aged 5 to 15 years, with a mean age of onset of 7 years (SD 3.2). It is characterized by attacks of "moderate to severe, periumbilical or poorly localized abdominal pain" lasting from 2 to 72 hours.2 These attacks are not accompanied by headache or gastrointestinal pathology, and must involve 2 or more of the following symptoms: nausea, vomiting (though less intense than that observed in CVS), anorexia, or pallor. It is likely that abdominal migraine is the most common of these episodic syndromes observed in pediatric headache clinics, accounting for 48.9% of these in a 2014 study.15

References

  1. Teixeira KC, Montenegro MA, Guerreiro MM. Migraine equivalents in childhood. J Child Neurol. 2014; 29(10):1366-1369.
  2. Lebron D, Vasconcellos E. The episodic syndromes that maybe associated with migraines. Semin Pediatr Neurol. 2016 23(1):6-10.
  3. Gelfand AA. Episodic syndromes that may be associated with migraine: a.k.a. "the childhood periodic syndromes." Headache. 2015; 55(10):1358-1364.
  4. Castro-Rodriguez JA, Stern DA, Halonen M, et al. Relation between infantile colic and asthma/atopy: a prospective study in an unselected population. Pediatrics. 2001;108:878-882.
  5. Lucassen PL, Assendelft WJ, van Eijk JT, Gubbels JW, Douwes AC, van Geldrop WJ. Systematic review of the occurrence of infantile colic in the community. Arch Dis Child. 2001;84:398-403.
  6. Gelfand AA, Thomas KC, Goadsby PJ. Before the headache: infant colic as an early life expression of migraine. Neurology. 2012;79:1392-1396. 
  7. Gelfand AA, Goadsby PJ, Allen IE. The relationship between migraine and infant colic: a systematic review and meta-analysis. Cephalalgia. 2015;35(1):63-72.
  8. Sillanpaa M, Saarinen M. Infantile colic associated with childhood migraine: A prospective cohort study. Cephalalgia. 2015;35(14):1246-1251.
  9. Barr RG, Trent RB, Cross J. Age-related incidence curve of hospitalized Shaken Baby Syndrome cases: convergent evidence for crying as a trigger to shaking. Child Abuse Negl. 2006;30(1):7-16.
  10. Giffin NJ, Benton S, Goadsby PJ. Benign paroxysmal torticollis of infancy: four new cases and linkage to CACNA1A mutation. Dev Med Child Neurol. 2002;44(7):490-493.
  11. Vila-Pueyo M, Gene GG, Flotats-Bastardes M, et al. A loss-of -function CACNA1A mutation causing benign paroxysmal torticollis of infancy. Eur J Paediatr Neurol. 2014;18(3):430-433.
  12. Batuecas-Caletrio A, Martin-Sanchez V, Cordero-Civantos C, et al. Is benign paroxysmal vertigo of childhood a migraine precursor? Eur J Paediatr Neurol. 2013;17(4):397-400.
  13. Krams B, Echenne B, Leydet J, Rivier F, Roubertie A. Benign paroxysmal vertigo of childhood: long-term outcome. Cephalgia. 2011;31(4):439-443.
  14. Lee LY, Abbott L, Mahlangu B, Moodie SJ, Anderson S. The management of cyclic vomiting syndrome: a systematic review. Eur J Gastroenterol Hepatol. 2012;24(9):1001-1006.
  15. Tarantino S, Capuano A, Torriero R, et al. Migraine equivalents as part of migraine syndrome in childhood. Pediatr Neurol. 2014;51(5):645-649.
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