Neurológia pre prax 6/2010

Familial hemiplegic migraine

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.

Keywords: familial hemiplegic migraine, sporadic hemiplegic migraine, migraine with aura, autosomal dominant inheritance, gene mutation, chromosome.