Possible effect of corticoids on hemiplegic attacks in severe hemiplegic migraine

Pediatr Neurol. 2013 Oct;49(4):286-8. doi: 10.1016/j.pediatrneurol.2013.04.011. Epub 2013 Jul 4.


Background: Sporadic and familial hemiplegic migraines are rare paroxysmal disorders characterized by transient hemiparesis and headache. The distinction is based on whether other family members are affected. In 50% of cases, these migraines are caused by CACNA1 A missense mutations.

Patients: We describe a boy with a particularly severe phenotype and a de novo R1349Q mutation of the CACNA1 A gene.

Results: The patient suffered from early-onset profound mental retardation, epileptic seizures, cerebellar ataxia, and progressive cerebellar atrophy. He experienced prolonged attacks of migraine with hemiparesis, seizures, altered consciousness, and fever resulting from minor head traumas. A prolonged hemiplegic attack improved following a 5-day treatment of 100 mg/d methylprednisolone.

Conclusion: R1349Q mutation of the CACN1 A gene may be associated with a severe phenotype. Corticoids might be beneficial in prolonged hemiplegic attacks.

Keywords: CACNA1A gene; corticoids; hemiplegic; mental retardation; migraine.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Adrenal Cortex Hormones / therapeutic use*
  • Calcium Channels / genetics
  • Humans
  • Male
  • Migraine with Aura / diagnosis*
  • Migraine with Aura / drug therapy*
  • Migraine with Aura / genetics
  • Severity of Illness Index*
  • Treatment Outcome


  • Adrenal Cortex Hormones
  • CACNA1A protein, human
  • Calcium Channels