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.

Keywords::

• azetazolamide
• cortical spreading depression
• epilepsy
• ergotamine
• familial hemiplegic migraine
• management
• nimodipine
• seizures
• sporadic hemiplegic migraine
• triptans
• verapamil

Financial & competing interests disclosure

The author has no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

No writing assistance was utilized in the production of this manuscript.

Notes

FHM: Familial hemiplegic migraine; MA: Migraine with aura; MO: Migraine without aura; SHM: Sporadic hemiplegic migraine.