title: [A descriptive study of the neuropsychological and psychopathological profile in patients with type 1 myotonic dystrophy].
abstract: BACKGROUND
  Type 1 myotonic dystrophy (MD-1) or Steinert disease is a multisystemic progressive disorder. Studies have shown cognitive deficits, depressive symptoms and a high incidence of anxiety personality traits that compromise both the functionality and the quality of life of these patients.
  
  
  OBJECTIVE
  To describe the cognitive and psychopathological profile of a sample of patients with the adult variant of MD-1.
  
  
  METHODS
  A sample of 27 patients diagnosed with MD-1 was selected from those being followed up in the neurology service of the Complejo Hospitalario de Navarra. Eligibility criteria were age under 50 years and the absence of any other pathology or physical condition that prevented them from performing the psychological evaluation. A neuropsychological evaluation battery specifically designed for this kind of pathology was used, together with psychopathological and functionality measures.
  
  
  RESULTS
  The neuropsychological evaluation revealed mainly deficits in visual-constructional and visuospatial skills, alternating attention and in other-reported dysexecutive signs and symptoms. The group of patients did not present any clinically significant symptoms of depression or anxiety, but did score high on obsession-compulsion, interpersonal sensitivity, paranoid ideation and psychoticism. The results point towards a deterioration in functionality.
  
  
  CONCLUSIONS
  In the integral approach to MD-1, the characterisation and developmental monitoring of the cognitive, psychopathological and personality profile, as well as the level of functionality, all contribute to an improvement in the quality of life of these patients.
authors.affiliation: Complejo Hospitalario de Navarra. Servicio Navarro de Salud-Fundacion Argibide, Pamplona, Espana.
authors.forename: Raquel
authors.initials: R
authors.lastname: Seijas-Gomez
authors.affiliation: Complejo Hospitalario de Navarra. Servicio Navarro de Salud-Fundacion Argibide, Pamplona, Espana.
authors.forename: Izaskun
authors.initials: I
authors.lastname: Basterra-Jimenez
authors.affiliation: Complejo Hospitalario de Navarra.Servicio Navarro de Salud-Fundacion Argibide, Pamplona, Espana.
authors.forename: Pilar
authors.initials: P
authors.lastname: Luna-Lario
authors.affiliation: Complejo Hospitalario de Navarra. Servicio Navarro de Salud-Fundacion Argibide, Pamplona, Espana.
authors.forename: Javier
authors.initials: J
authors.lastname: Tirapu-Ustarroz
authors.affiliation: Complejo Hospitalario de Navarra. Servicio Navarro de Salud., Pamplona, Espana.
authors.forename: Teresa
authors.initials: T
authors.lastname: Cabada-Giadas
authors.affiliation: Complejo Hospitalario de Navarra. Servicio Navarro de Salud., Pamplona, Espana.
authors.forename: Marina
authors.initials: M
authors.lastname: Iridoy-Zulet
authors.affiliation: Complejo Hospitalario de Navarra. Servicio Navarro de Salud., Pamplona, Espana.
authors.forename: Ivonne
authors.initials: I
authors.lastname: Jerico-Pascual
authors.affiliation: Complejo Hospitalario de Navarra. Servicio Navarro de Salud., Pamplona, Espana.
authors.forename: Álvaro
authors.initials: Á
authors.lastname: Gargallo-Vaamonde
authors.affiliation: Universidad Publica de Navarra, Pamplona, Espana.
authors.forename: José Javier
authors.initials: JJ
authors.lastname: Lopez-Goni
mesh_terms: D000328:Adult; D001008:Anxiety Disorders / Q000209:etiology; D003072:Cognition Disorders / Q000209:etiology; D003193:Compulsive Personality Disorder; D003866:Depressive Disorder / Q000209:etiology; D018450:Disease Progression; D056344:Executive Function; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009223:Myotonic Dystrophy / Q000150:complications / Q000503:physiopathology / Q000523:psychology*; D009483:Neuropsychological Tests; D010260:Paranoid Personality Disorder; D010551:Personality; D010556:Personality Tests; D011618:Psychotic Disorders / Q000209:etiology; D011788:Quality of Life; D012959:Socioeconomic Factors