Which clinical history findings are characteristic of hemiplegic migraine in children?

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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Hemiplegic migraine presents with hemiplegia or hemiparesis, with or without a speech or language disturbance, which clears in minutes to hours. Headache may be less dramatic than the hemiplegia. Other migrainous symptoms (eg, nausea, vomiting, and photophobia) are present to varying degrees. Hemiplegia may precede or accompany the headache.

Hemiplegic migraine is often familial, is dominantly inherited, and is linked to chromosome 19 in some studies. [11, 12] An alternative locus has been described on chromosome 1. [13, 14] 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). [15, 16] 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.

Additional genetic associations have been found including with mutations in SCN1a, ATP1A2, and PRRT2 genes. All of these mutations include variable phenotypes with epilepsy, paroxysmal dyskinesias, episodic ataxia, and hemiplegic migraine seen in affected families. Families with the same mutation may manifest differing combinations of symptoms, and the same symptoms may be due to different mutations. [7, 9, 10]

The diagnosis cannot be made during the first episode, though it may be suspected in the presence of a positive family history. Exclude more serious causes of headache with hemiplegia or hemiparesis, including intracranial hemorrhage, mass, infection, and 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.

The 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 (MELAS). On occasion, hemiplegic migraine may be associated with reversible unilateral cerebral edema on imaging. [17]

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