Ten years of follow-up in a large family with familial hemiplegic migraine type 1: Clinical course and implications for treatment. (May 2018)

• Record Type: Journal Article
• Authors: Indelicato, Elisabetta; Nachbauer, Wolfgang; Eigentler, Andreas; Donnemiller, Evelin; Wagner, Michaela; Unterberger, Iris; Boesch, Sylvia
• Is Part Of: Cephalalgia. Volume 38:Number 6(2018)
• Page Start: 1167
• Page End: 1176
• Publication Date: 2018-05
• Subjects: Familial hemiplegic migraine -- CACNA1A gene -- long-term treatment -- migraine prophylaxis -- calcium antagonists -- clinical phenotype; Headache -- Periodicals; 616.8491
• DOI: 10.1177/0333102417715229
• Languages: English
• ISSNs: 0333-1024

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.