Cortical spreading depression and familial hemiplegic migraine 2015

Familial Hemiplegic Migraine and Spreading Depression

OBJECTIVE Familial hemiplegic migraine (FHM) is an autosomal dominantly inherited subtype of migraine with aura, characterized by transient neurological signs and symptoms. Typical hemiplegic migraine attacks start in the first or second decade of life. Some patients with FHM suffer from daily recurrent attacks since childhood. Results from extensive studies of cellular and animal models have i...

familial hemiplegic migraine and spreading depression

how to cite this article: kazemi h, speckmann ej, gorji a. familial hemiplegic migraine and spreading depression. iran j child neurol. 2014 summer;8(3): 6-11.   abstract objective familial hemiplegic migraine (fhm) is an autosomal dominantly inherited subtype of migraine with aura, characterized by transient neurological signs and symptoms. typical hemiplegic migraine attacks start in the first...

Increased Susceptibility to Cortical Spreading Depression in the Mouse Model of Familial Hemiplegic Migraine Type 2

Familial hemiplegic migraine type 2 (FHM2) is an autosomal dominant form of migraine with aura that is caused by mutations of the α2-subunit of the Na,K-ATPase, an isoform almost exclusively expressed in astrocytes in the adult brain. We generated the first FHM2 knock-in mouse model carrying the human W887R mutation in the Atp1a2 orthologous gene. Homozygous Atp1a2(R887/R887) mutants died just ...

Enhanced subcortical spreading depression in familial hemiplegic migraine type 1 mutant mice.

Familial hemiplegic migraine type 1, a monogenic migraine variant with aura, is linked to gain-of-function mutations in the CACNA1A gene encoding Ca(V)2.1 channels. The S218L mutation causes severe channel dysfunction, and paroxysmal migraine attacks can be accompanied by seizures, coma, and hemiplegia; patients expressing the R192Q mutation exhibit hemiplegia only. Familial hemiplegic migraine...

Familial hemiplegic migraine.