Ten years of follow-up in a large family with familial hemiplegic migraine type 1: Clinical course and implications for treatment

Cephalalgia. 2018 May;38(6):1167-1176. doi: 10.1177/0333102417715229. Epub 2017 Aug 31.

Abstract

Background Familial hemiplegic migraine (FHM) is a rare, genetic form of migraine with aura. The severity of the aura imposes an effective prophylaxis that is currently based on standard anti-migraine drugs. To this concern, only short-term reports are currently available. Methods Eight patients from a multigenerational FHM type 1 family harbouring a T666M mutation in the CACNA1A gene were referred to our ataxia outpatient clinic. Medical history, general and neurological examination as well as therapeutic approaches were recorded regularly on a routine basis for an average period of 13 years (range 9-15 years). Brain imaging studies and EEG data were also collected. Results Our long-term follow-up revealed that ictal manifestations, which usually improve after the adolescence, may reoccur later in the adulthood. Permanent neurological signs as assessed by means of clinical evaluation as well as follow-up MRIs, EEGs and neuropsychological testing remained stable. Interval therapy with non-selective calcium antagonists reduced the burden of migraine attacks and was well tolerated in the long term.

Keywords: CACNA1A gene; Familial hemiplegic migraine; calcium antagonists; clinical phenotype; long-term treatment; migraine prophylaxis.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Calcium Channels / genetics
  • Child
  • Female
  • Follow-Up Studies
  • Humans
  • Male
  • Middle Aged
  • Migraine with Aura* / genetics
  • Migraine with Aura* / pathology
  • Migraine with Aura* / physiopathology
  • Mutation
  • Pedigree
  • Young Adult

Substances

  • CACNA1A protein, human
  • Calcium Channels