What is the pathophysiology of childhood migraine variants (equivalents)?

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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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.

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