title: Female gender doubles executive dysfunction risk in ALS: a case-control study in 165 patients.
abstract: BACKGROUND
  Cognitive impairment, mainly characterised by executive dysfunction, occurs in about half of cases in amyotrophic lateral sclerosis (ALS). There is evidence that gender influences some clinical features of the disease, but its influence on the cognitive spectrum is unknown. Our objective was to investigate the impact of gender on cognitive profiles of patients with ALS.
  
  
  METHODS
  A retrospective study based on an exhaustive neuropsychological battery was performed in a group of 165 (70 females, 95 males) sporadic, non-demented patients with ALS compared with 134 healthy control participants. This assessment primarily focused on executive, memory and language functions.
  
  
  RESULTS
  47 (29%) patients revealed impairment in executive function and 30 (18%) patients revealed cognitive non-executive impairment. Independent from mood tone and clinical variables, a significantly greater executive impairment was determined in female patients than in male patients and control participants. The relative risk for ALS females having impairment in executive function compared with male patients was 2.6 (95% CI 1.6 to 4.4; p=0.0003). ALS females scored lower in Phonemic Fluency, Trial Making, and Wisconsin Card Sorting test.
  
  
  CONCLUSIONS
  Results highlight a significant vulnerability of ALS female patients to develop cognitive dysfunctions peculiar to the disease, independently of bulbar onset. The explicative hypotheses of the data are focused on two interpretative lines not mutually exclusive: the role of gonadal hormones and gender-related brain asymmetry pre-existing to the disease. These findings, never reported before in the literature, can have important implications for models of ALS pathogenesis and for future clinical trial designs.
authors: Department of Neurosciences, University of Padova, Padova, Italy Department of Philosophy, Sociology, Pedagogy and Applied Psychology (FISPPA), University of Padova, Padova, Italy.
authors: Arianna
authors: A
authors: Palmieri
authors: Department of General Psychology, University of Padova, Padova, Italy.
authors: Giovanni
authors: G
authors: Mento
authors: Department of Philosophy, Sociology, Pedagogy and Applied Psychology (FISPPA), University of Padova, Padova, Italy.
authors: Vincenzo
authors: V
authors: Calvo
authors: Department of Neurosciences, University of Padova, Padova, Italy.
authors: Giorgia
authors: G
authors: Querin
authors: Department of Neurosciences, University of Padova, Padova, Italy.
authors: Carla
authors: C
authors: D'Ascenzo
authors: IRCCS San Camillo, Venice, Italy.
authors: Chiara
authors: C
authors: Volpato
authors: Department of Philosophy, Sociology, Pedagogy and Applied Psychology (FISPPA), University of Padova, Padova, Italy.
authors: Johann Roland
authors: JR
authors: Kleinbub
authors: Department of General Psychology, University of Padova, Padova, Italy.
authors: Patrizia Silvia
authors: PS
authors: Bisiacchi
authors: IRCCS San Camillo, Venice, Italy.
authors: Gianni
authors: G
authors: Sorarù
mesh_terms: D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000150:complications / Q000523:psychology*; D016022:Case-Control Studies; D003072:Cognition Disorders / Q000150:complications / Q000523:psychology*; D056344:Executive Function; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009483:Neuropsychological Tests; D012189:Retrospective Studies; D012727:Sex Characteristics
keywords: ALS; Neuropsychology