Neurol. praxi. 2010;11(6):400-405

Familial hemiplegic migraine

MUDr.Jiří Mastík

1. neurologická klinika FN u sv. Anny a LF MU, Brno

Familial hemiplegic migraine (FHM) is a rare autosomal dominant hereditary disease. It is the first type of migraine with aura in which

a genetic defect on chromosome 19 (FHM 1) or, more rarely, on chromosome 1 (FHM 2) and chromosome 2 (FHM 3) has been shown.

In addition to migraine headache, a typical presentation includes the presence of motor weakness and an identical impairment in at

least one relative. The motor impairment may even be hemiplegic in nature and last for days or even weeks. Triptans are not recommended

in the treatment for their potent vasoconstrictive effect; nonspecific antimigraine drugs are preferred. When the course is more

severe, prophylaxis similar to that in the treatment of migraine with aura is appropriate. The differential diagnosis must distinguish

other types of migraine, strokes, focal epileptic seizures and some rare hereditary diseases such as MELAS and CADASIL. Sporadic

hemiplegic migraine has the same clinical presentation except for a negative family history.

Also included are two case reports: a family with familial hemiplegic migraine and a case of a woman with sporadic hemiplegic migraine

with an emphasis placed on the diagnostic pitfalls of this disease.

Even though the prognosis of HM is mostly good with a low frequency and full reversibility of the attacks, some prolonged attacks may be

associated with considerable discomfort for the patients. Mutations of the gene affect the release of excitatory amino acids and serotonin

neurotransmitters. Knowledge from the field of molecular genetics (e. g. DNA analysis) may, in the future, contribute to introducing a more

effective and specific treatment than the one used so far.

gene mutation, chromosome.

Keywords: familial hemiplegic migraine, sporadic hemiplegic migraine, migraine with aura, autosomal dominant inheritance,

Published: December 31, 2010  Show citation