When more is needed: The utility of the frontotemporal dementia scale in ALS

Abstract

ALS and frontotemporal dementia (FTD) utilize different functional rating scales: the ALSFRS-R assesses physical disability whereas the Frontotemporal Dementia Rating Scale assesses behavioural and functional impairment to produce an index of dementia staging. To better consider the applicability of the FRS in an ALS population, 130 patient-carer dyads were investigated. Scores on the ALSFRS-R and FRS were not significantly correlated (r_s = 0.12, p > 0.10). Furthermore, patients with mild physical disability may rate as severe on the FRS; conversely, individuals who are severely physically disabled may exhibit very few behavioural symptoms. Measures typically used in ALS studies do not fully encapsulate the range of clinical symptoms, particularly from a behavioural perspective.

Key words::

• Disease progression
• ALSFTD
• ALSFRS-R
• FRS

Acknowledgements

We are very grateful for the support of patients and carers as well as MND Australia.

Declaration of interest: S. Hsieh is supported by the ARC Centre of Excellence in Cognition and its Disorders. P. Lillo was a recipient of a CONICYT Scholarship provided by the Government of Chile. J. Hodges is in receipt of an Australian Research Council Federation Fellowship Grant. M. Kiernan is in receipt of National Health and Medical Research Project Grants. E. Mioshi is a recipient of an NHMRC Early Career Fellowship and a Motor Neurone Disease Research Institute of Australia Grant.

The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.