title: Cognitive, anxiety and mood disorders in the fragile X-associated tremor/ataxia syndrome.
abstract: OBJECTIVE
  We evaluated patients with fragile X-associated tremor/ataxia syndrome (FXTAS), a neurodegenerative disorder associated with a CGG repeat expansion in the premutation range in the fragile X mental retardation 1 (FMR1) gene.
  
  
  METHODS
  Neurological, psychiatric and neuropsychological evaluations were performed on 15 male patients with FXTAS.
  
  
  RESULTS
  Seven cases were diagnosed with dementia, and seven others were diagnosed with mood and/or anxiety disorders. Twelve subjects demonstrated deficits on neuropsychological testing.
  
  
  CONCLUSIONS
  Physicians assessing dementia patients are urged to consider this newly described syndrome, especially in patients with dementia associated with a movement disorder and in those with a family history of mental retardation. If FXTAS is a possible diagnosis, physicians may carry out FMR1 DNA testing; patients who test positive on DNA testing should undergo magnetic resonance imaging, be referred to neurology and receive genetic counseling.
authors.affiliation: Department of Psychiatry and Behavioral Sciences, School of Medicine, University of California-Davis, Sacramento, CA 95817, USA. james.bourgeois@ucdmc.ucdavis.edu
authors.forename: James A
authors.initials: JA
authors.lastname: Bourgeois
authors.forename: Jennifer B
authors.initials: JB
authors.lastname: Cogswell
authors.forename: David
authors.initials: D
authors.lastname: Hessl
authors.forename: Lin
authors.initials: L
authors.lastname: Zhang
authors.forename: Michele Y
authors.initials: MY
authors.lastname: Ono
authors.forename: Flora
authors.initials: F
authors.lastname: Tassone
authors.forename: Faraz
authors.initials: F
authors.lastname: Farzin
authors.forename: James A
authors.initials: JA
authors.lastname: Brunberg
authors.forename: Jim
authors.initials: J
authors.lastname: Grigsby
authors.forename: Randi J
authors.initials: RJ
authors.lastname: Hagerman
mesh_terms: D000368:Aged; D001007:Anxiety / Q000453:epidemiology*; D001259:Ataxia; D002140:California / Q000453:epidemiology; D003072:Cognition Disorders / Q000453:epidemiology*; D015897:Comorbidity; D005600:Fragile X Syndrome / Q000503:physiopathology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D019964:Mood Disorders / Q000453:epidemiology*; D014202:Tremor