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Cognitive Functions, Emotional Behavior, and Quality of Life in Familial Hemiplegic Migraine

Karner, Elfriede MSc; Delazer, Margarete PhD; Benke, Thomas MD; Bösch, Sylvia MD

Cognitive and Behavioral Neurology: June 2010 - Volume 23 - Issue 2 - p 106-111
doi: 10.1097/WNN.0b013e3181c3a8a6
Original Studies

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.

Clinical Department of Neurology, Innsbruck Medical University, Austria

Reprints: Elfriede Karner, MSc, Universitätsklinik für Neurologie, Anichstrasse 35, A-6020 Innsbruck (e-mail:

Received for publication January 14, 2009

accepted September 20, 2009

© 2010 Lippincott Williams & Wilkins, Inc.