Familial hemiplegic migraine with cerebellar ataxia and paroxysmal psychosis

Familial hemiplegic migraine is a rare autosomal dominant disorder associat ed with stereotypic neurologic au ra phenomena including hemiparesis, So fa r two chromosomal loci have been identified. Families linked to the chromos ome 19 locus display missense mutations within the CACNL1A4 gene. Here we r eport on a family with familial hemiplegic migraine and cerebellar ataxia w ith recurrent episodes of acute paranoid psychosis with anxiety and visual hallucinations associated with migraine attacks. Based on the clinical and haplotype evidence indicating linkage to chromosome 19 in this family, we h ypothesize that a dysfunction of the mutated calcium channel may be involve d not only in the development of hemiplegic migraine but also in the acute psychotic episodes observed in these patients.