Clinical and Genetic Features of Familial Hemiplegic Migraine

ABSTRACT

Migraine is a common cause of episodic headache disorder, and multiple factors are associated with the pathogenic mechanism underlying migraine. Recent studies suggest that genetic determinants influence migraine, most likely as part of a multifactorial mechanism. Due to the similarity in clinical features and pathophysiological mechanisms between familial hemiplegic migraine (FHM) and migraine with aura, FHM can be used as a model for migraine and aura. Mutations in the CACNA1A gene on chromosome 19p13 (FHM1), in the ATP1A2 gene at 1q23 (FHM2), and in the SCNA1A gene at 2q24 (FHM3) are responsible for most of the representative types of FHM. The recent beta version of the third edition of the International Classification of Headache Disorders contains revised diagnostic criteria for FHM, which include the above-three genetic factors. In this review we discuss the clinical and genetic features of FHM.