title: Management of sporadic and familial hemiplegic migraine.
abstract: Sporadic and familial hemiplegic migraines (SHM and FHM) are rare paroxysmal disorders characterized by motor aura and headache. The distinction is based on whether other family members are affected. The majority of FHM families have a mutation in one of the ion channels CACNA1A, ATP1A2 and SCN1A. SHM is sometimes caused by a de novo mutation in one of the genes. Clinical trials of SHM and FHM have not been conducted. Seizure in FHM is secondary to cerebral edema and fever among other factors. The regional cerebral blood flow is reduced during the reversible aura symptoms. Triptans and ergotamine are contraindicated in the management of SHM and FHM based on the pathophysiology. Nimodipine is contraindicated. Acute and prophylactic management are otherwise based on the management principle of the migraine without aura and migraine with aura.
authors.affiliation: Head and Neck Research Group, Department of Neurology, Research Centre, Akershus University Hospital, Dr. Kobros vei 39, 1474 Nordbyhagen, Oslo, Norway. m.b.russell@medisin.uio.no
authors.forename: Michael Bjørn
authors.initials: MB
authors.lastname: Russell
mesh_terms: D000818:Animals; D015220:Calcium Channels / Q000235:genetics; D002986:Clinical Trials as Topic; D013181:Cortical Spreading Depression / Q000502:physiology; D006801:Humans; D007473:Ion Channels / Q000235:genetics; D008881:Migraine Disorders / Q000175:diagnosis* / Q000235:genetics / Q000503:physiopathology / Q000628:therapy*; D009154:Mutation / Q000235:genetics; D000254:Sodium-Potassium-Exchanging ATPase / Q000235:genetics; D020864:Calcium Channels, N-Type
chemical_list: D015220:Calcium Channels; D007473:Ion Channels; D000254:Sodium-Potassium-Exchanging ATPase; C105656:CACNA1A protein, human; D020864:Calcium Channels, N-Type; C498531:ATP1A2 protein, human