OBJECTIVES: To describe the cognitive functions, mood, and quality of life in a family with genetically proved familial hemiplegic migraine (FHM), carrying a missense mutation on chromosome 19 (T666M), corresponding to the most frequent FHM subtype. BACKGROUND: FHM is an autosomal dominant subtype of migraine with an aura, characterized by hemiparesis during the aura. Whereas the genetic background of FHM has been studied intensely, less attention has been paid to cognitive functions and mood between attacks. METHOD: Six patients performed neuropsychologic assessment between attacks. Depression, anxiety, and quality of life were evaluated by questionnaires. Cerebral magnetic resonance imaging was performed. RESULTS: Neuropsychologic assessment revealed a distinct pattern of preserved and impaired functions. Whereas linguistic abilities and verbal memory were intact, all patients showed deficits in figural memory, executive functions, in some aspects of attention, and in dexterity. Intelligence of 1 patient was below average. All but 1 patient reported normal quality of life; there were no symptoms of depression or state anxiety. All patients showed cerebellar atrophy and cerebellar ataxia. CONCLUSION: Cognitive abnormalities and cerebellar atrophy were found in all patients. FHM-related cognitive deficits may be associated to a disturbance of cerebrocerebellar circuits.
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