title: Long-term outcome of cognitive functions, emotional behavior, and quality of life in a family with familial hemiplegic migraine.
abstract: 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.
authors.affiliation: Clinical Department of Neurology, Medical University Innsbruck, Austria. elfriede.karner@uki.at
authors.forename: Elfriede
authors.initials: E
authors.lastname: Karner
authors.forename: Wolfgang
authors.initials: W
authors.lastname: Nachbauer
authors.forename: Thomas
authors.initials: T
authors.lastname: Bodner
authors.forename: Thomas
authors.initials: T
authors.lastname: Benke
authors.forename: Sylvia
authors.initials: S
authors.lastname: Boesch
authors.forename: Margarete
authors.initials: M
authors.lastname: Delazer
mesh_terms: D000328:Adult; D003071:Cognition / Q000502:physiology*; D003072:Cognition Disorders / Q000235:genetics / Q000503:physiopathology; D004644:Emotions / Q000502:physiology*; D004827:Epilepsy / Q000235:genetics; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D020325:Migraine with Aura / Q000235:genetics / Q000503:physiopathology*; D009483:Neuropsychological Tests; D010375:Pedigree; D011788:Quality of Life; D011795:Surveys and Questionnaires; D013997:Time Factors