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Long-term Outcome of Cognitive Functions, Emotional Behavior, and Quality of Life in a Family With Familial Hemiplegic Migraine

Karner, Elfriede MS; Nachbauer, Wolfgang MD; Bodner, Thomas PhD; Benke, Thomas MD; Boesch, Sylvia MD; Delazer, Margarete PhD

Cognitive and Behavioral Neurology: June 2012 - Volume 25 - Issue 2 - p 85–92
doi: 10.1097/WNN.0b013e318259cb36
Original Studies

Objective: To investigate long-term cognitive outcome, mood, and quality of life in a family with genetically proven familial hemiplegic migraine (FHM).

Background: FHM is a rare autosomal-dominant subtype of migraine with aura, characterized by some degree of hemiparesis during the aura. In a previous study, we showed preserved and impaired cognitive functions in patients with FHM. Until now, the progression of cognitive dysfunctions has not been known. However, the ability to predict progression is important for counseling patients about education, career, and family life.

Methods: Seven years after extensive baseline neuropsychological testing, we retested 6 members of a family with FHM, including questionnaires about mood and quality of life.

Results: The follow-up assessment revealed no global cognitive decline. All the patients' linguistic abilities and verbal memory remained intact; however, their figural memory, attention, and some aspects of executive function were impaired. Half the patients had a slight deterioration in their visuoconstructional functions. Half had higher scores on the trait and state anxiety measures.

Conclusions: Cognitive deficits in a family with FHM persisted, but without marked progression. Worsening of visuoconstructional abilities may be related to executive dysfunction, confirming a disturbance of cerebrocerebellar circuits.

Clinical Department of Neurology, Medical University Innsbruck, Innsbruck, Austria

The authors declare no conflicts of interest.

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

Received November 14, 2011

Accepted March 6, 2012

© 2012 Lippincott Williams & Wilkins, Inc.