title: Possible effect of corticoids on hemiplegic attacks in severe hemiplegic migraine.
abstract: 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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  R1349Q mutation of the CACN1 A gene may be associated with a severe phenotype. Corticoids might be beneficial in prolonged hemiplegic attacks.
authors.affiliation: Department of Child Neurology, Children's Hospital, University of Tübingen, Germany. Electronic address: iciar.sanchez@med.uni-tuebingen.de.
authors.forename: Iciar
authors.initials: I
authors.lastname: Sánchez-Albisua
authors.forename: Martin
authors.initials: M
authors.lastname: Schöning
authors.forename: Karin
authors.initials: K
authors.lastname: Jurkat-Rott
authors.forename: Holger
authors.initials: H
authors.lastname: Lerche
mesh_terms: D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D015220:Calcium Channels / Q000235:genetics; D006801:Humans; D008297:Male; D020325:Migraine with Aura / Q000175:diagnosis* / Q000188:drug therapy* / Q000235:genetics; D012720:Severity of Illness Index; D016896:Treatment Outcome; D020864:Calcium Channels, N-Type
chemical_list: D000305:Adrenal Cortex Hormones; D015220:Calcium Channels; C105656:CACNA1A protein, human; D020864:Calcium Channels, N-Type
keywords: CACNA1A gene; corticoids; hemiplegic; mental retardation; migraine