Chapter 3 Familial Hemiplegic Migraine

Migraine is a severely debilitating episodic disorder affecting up to 12% of the general population. Migraine arises from both genetic and environmental factors, complicating our understanding of what makes the migraine brain susceptible to attacks. In recent years, powerful genetic screening tools have revealed several single genes linked to migraine. One example of a monogenic subtype of migraine is familial hemiplegic migraine (FHM), a rare form of migraine with aura. The fact that FHM and common multifactorial migraine have many overlapping clinical features indicates that they likely share underlying pathophysiological pathways. In addition, the identification of monogenic subtypes has made it possible to generate suitable animal models for migraine. The purpose of this review is to present an overview of the clinical features of migraine and discuss the continuing highway of migraine gene discovery. The genes associated with FHM will be discussed, including what we have learned from studying the functional consequences of FHM mutations in cellular and animal models. (C) 2008, Elsevier Inc.