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Timothy C. Hain, MD 
Page last modified:
June 18, 2009
This disorder is an autosomal domininant condition characterized by migraine, hemiparesis, and in some families, progressive cerebellar atrophy. FHM1 is associated with a mutation in the a-1a subunit gene for calcium channels. Four different missence mutations have been identified. Mutations in this subunit have also been identified in one of the episodic ataxias, and in one of the spinocerebellar atrophies (SCA-6). FHM2 is associated with a mutaiton in the ATPase gene. It has recently been suggested that both of these mutations render the brain more susceptible to glutamate release (Moskowitz et al, 2004).
FHM1 and FHM2 are characterized by enhanced susceptibility and sustained attacks of visual, somatosensory and aphasic auras as well as by prolonged motor weakness or paralysis. Photophobia, phonophobia, nausea and vomiting are also common symptoms. Motor weakness is uncommon in classic migraine (migraine with aura).
Moskowitz and others. Deciphering migraine mechanisms: clues from familial hemiplegic migraine genotypes. Ann Neurology, 2004, 55, #2, 276-279.
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