Familial Hemiplegic Migraine (FHM) is a rare type of migraine associated with genetic defects in ion-transporting proteins.
Clinical features:
(1) familial occurrence with autosomal dominant inheritance
(2) migraine with aura
(3) transient motor weakness and/or other aura symptoms
While neurological findings are often reversible over weeks or months, permanent neurologic deficits can occur.
Type
|
Gene Mutated (Chromosome)
|
Protein Affected
|
FHM1
|
CACNA1A (19p13)
|
pore-forming alpha1 subunit of the neuronal calcium channel Cav2.1
|
FHM2
|
ATP1A2 (1q23.2)
|
alpha-2 subumit of the glial Na/K ATPase
|
FHM3
|
SCN1A (2q24.3)
|
pore-forming alpha subunit of the voltage-gated sodium channel Nav1.1
|