S M Gillingham1,2, Y Yunusova3,4, A Ganda4,5, E Rogaeva5,6, S E Black1,4,5,7,8, D T Stuss1,2,4,5,7, L Zinman4,7,8. 1. a Rotman Research Institute at Baycrest , Toronto , Canada. 2. b Department of Psychology , University of Toronto , Toronto , Canada. 3. c Department of Speech-Language Pathology , University of Toronto , Toronto , Canada. 4. d Hurvitz Brain Sciences Research Programme , and Sunnybrook Research Institute , Toronto , Canada. 5. e Department of Medicine, (Neurology) , University of Toronto , Toronto , Canada. 6. f Tanz Centre for Research in Neurodegenerative Diseases , University of Toronto , Toronto , Canada. 7. g Department of Medicine (Neurology) , Sunnybrook Health Sciences Centre and. 8. h L.C. Campbell Cognitive Neurology Research Unit , Sunnybrook Research Institute, University of Toronto , Toronto , Canada.
Abstract
OBJECTIVE: It is generally acknowledged that at least 50% of individuals with amyotrophic lateral sclerosis (ALS) will exhibit cognitive deficits outside of the characteristic motor neuron involvement. However, a specific cognitive profile has been difficult to ascertain due to disease-related testing barriers and limitations in the sensitivity and specificity of available assessment methods. This study assessed the level of functioning of extramotor frontal cognitive processes in ALS, and the amount of change in the functioning in these processes over time as disease progresses. METHODS: Empirical tests validated for a model of frontal lobe functioning were modified into an assessment battery appropriate for individuals with ALS in a clinical setting (the ALS-CFB, Computerised Frontal Battery). Twenty ALS participants and 36 age- and education-matched neurologically healthy controls were tested, and a sub-sample of each group (11 ALS and 20 controls) re-tested after approximately nine months. RESULTS AND CONCLUSIONS: Compared to standard neuropsychological screening tests that did not show a difference between ALS participants and healthy controls, the ALS-CFB illustrated a profile of extramotor frontal dysfunction involving energisation (preparing the neural system to respond) and executive functions, a profile that may be indicative of the nature of neurodegeneration in ALS.
OBJECTIVE: It is generally acknowledged that at least 50% of individuals with amyotrophic lateral sclerosis (ALS) will exhibit cognitive deficits outside of the characteristic motor neuron involvement. However, a specific cognitive profile has been difficult to ascertain due to disease-related testing barriers and limitations in the sensitivity and specificity of available assessment methods. This study assessed the level of functioning of extramotor frontal cognitive processes in ALS, and the amount of change in the functioning in these processes over time as disease progresses. METHODS: Empirical tests validated for a model of frontal lobe functioning were modified into an assessment battery appropriate for individuals with ALS in a clinical setting (the ALS-CFB, Computerised Frontal Battery). Twenty ALSparticipants and 36 age- and education-matched neurologically healthy controls were tested, and a sub-sample of each group (11 ALS and 20 controls) re-tested after approximately nine months. RESULTS AND CONCLUSIONS: Compared to standard neuropsychological screening tests that did not show a difference between ALSparticipants and healthy controls, the ALS-CFB illustrated a profile of extramotor frontal dysfunction involving energisation (preparing the neural system to respond) and executive functions, a profile that may be indicative of the nature of neurodegeneration in ALS.