RT Journal Article
SR Electronic
T1 COGNITIVE IMPAIRMENT OCCURS BEFORE LOSS OF FUNCTION IN ALS
JF Journal of Neurology, Neurosurgery & Psychiatry
JO J Neurol Neurosurg Psychiatry
FD BMJ Publishing Group Ltd
SP e1
OP e1
DO 10.1136/jnnp-2016-315106.99
VO 87
IS 12
A1 Gabel, Matt
A1 Tsermentseli, Stella
A1 Goldstein, Laura H
A1 Al-Chalabi, Ammar
A1 Young, Alexandra
A1 Alexander, Daniel
A1 Leigh, P. Nigel
A1 Cercignani, Mara
YR 2016
UL http://jnnp.bmj.com/content/87/12/e1.239.abstract
AB Objectives Using a historical amyotrophic lateral sclerosis (ALS) dataset comprising detailed cognitive and clinical assessments, we used a novel event-based model to analyse the likely ordering of these biomarkers in the progression of ALS.Methods The dataset was derived from a cross-sectional sample of 28 ALS patients who were not classified as having ALS-FTD and 26 matched controls (see1 regarding cognitive and clinical assessments). The event-based model was adapted for ALS from previous work in Alzheimer's disease.2 ,3 Unlike traditional models of disease progression, event-based models do not rely on a priori staging of patients but extract the event ordering directly from the data, thus minimising subjective bias.Results The most likely order of progression showed that changes in executive function occur at an early stage of disease evolution. Language impairment tended to occur after executive dysfunction. Changes in functional ability assessed by rate of change of ALSFRS-R occurred after the changes in executive function.Conclusions These findings suggest that executive dysfunction occurs early in the evolution of ALS.