title: Motor function and behaviour across the ALS-FTD spectrum.
abstract: BACKGROUND
  Behavioural/functional disturbances, characteristic of frontotemporal dementia (FTD), are also a feature of amyotrophic lateral sclerosis (ALS) and patients with combined ALS and FTD (FTD-ALS).
  
  
  OBJECTIVE
  To investigate the progression of behavioural disturbances in ALS and FTD using the FTD functional rating scale (FTDFRS).
  
  
  METHODS
  Patients with ALS, FTD-ALS and FTD were recruited from specialist clinics. Baseline assessments included the FTDFRS and the ALS functional rating scale-revised (ALSFRS-R). Baseline assessments were included, as were longitudinal assessments in a proportion of patients.
  
  
  RESULTS
  In total, 21 ALS, 12 FTD-ALS and 14 behavioural variant FTD (bvFTD) patients were included in the study. Moderate or severe behavioural disturbance was common in patients with ALS at baseline (47.6%), although less frequent than in bvFTD patients; patients with FTD-ALS displayed intermediate impairment. The ALSFRS-R showed the opposite pattern and did not correlate with the FTDFRS. During the follow-up period, significant (P < 0.05) behavioural deterioration was demonstrated in patients with bvFTD and FTD-ALS, with a trend for decline in patients with ALS (P = 0.06).
  
  
  CONCLUSIONS
  Motor disturbance is the primary marker of disease severity in ALS, but behavioural and functional impairment are common, and may decline independently of motor function. As such, the FTDFRS may provide valuable information in the assessment and monitoring of ALS.
authors.affiliation: Neuroscience Research Australia, Sydney, NSW, Australia.|University of New South Wales, Sydney, NSW, Australia.
authors.forename: D
authors.initials: D
authors.lastname: De Silva
authors.affiliation: Brain and Mind Research Institute, The University of Sydney, Sydney, NSW, Australia.
authors.forename: S
authors.initials: S
authors.lastname: Hsieh
authors.affiliation: Brain and Mind Research Institute, The University of Sydney, Sydney, NSW, Australia.
authors.forename: J
authors.initials: J
authors.lastname: Caga
authors.affiliation: Neuroscience Research Australia, Sydney, NSW, Australia.|University of New South Wales, Sydney, NSW, Australia.|ARC Centre of Excellence in Cognition and its Disorders, University of New South Wales, Sydney, NSW, Australia.
authors.forename: F V C
authors.initials: FV
authors.lastname: Leslie
authors.affiliation: Neuroscience Research Australia, Sydney, NSW, Australia.|Brain and Mind Research Institute, The University of Sydney, Sydney, NSW, Australia.
authors.forename: M C
authors.initials: MC
authors.lastname: Kiernan
authors.affiliation: Neuroscience Research Australia, Sydney, NSW, Australia.|University of New South Wales, Sydney, NSW, Australia.|ARC Centre of Excellence in Cognition and its Disorders, University of New South Wales, Sydney, NSW, Australia.
authors.forename: J R
authors.initials: JR
authors.lastname: Hodges
authors.affiliation: Department of Psychiatry, Cambridge University, Cambridge, UK.
authors.forename: E
authors.initials: E
authors.lastname: Mioshi
authors.affiliation: Neuroscience Research Australia, Sydney, NSW, Australia.|University of New South Wales, Sydney, NSW, Australia.|ARC Centre of Excellence in Cognition and its Disorders, University of New South Wales, Sydney, NSW, Australia.
authors.forename: J R
authors.initials: JR
authors.lastname: Burrell
mesh_terms: D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000150:complications / Q000175:diagnosis*; D005260:Female; D057180:Frontotemporal Dementia / Q000150:complications / Q000175:diagnosis*; D006801:Humans; D008297:Male; D008875:Middle Aged; D009043:Motor Activity
keywords: amyotrophic lateral sclerosis; dementia; neurodegenerative disorders