TY - JOUR
T1 - Decline of cognitive and behavioral functions in amyotrophic lateral sclerosis
T2 - a longitudinal study
AU - Bersano, Enrica
AU - Sarnelli, Maria Francesca
AU - Solara, Valentina
AU - Iazzolino, Barbara
AU - Peotta, Laura
AU - De Marchi, Fabiola
AU - Facchin, Alessio
AU - Moglia, Cristina
AU - Canosa, Antonio
AU - Calvo, Andrea
AU - Chiò, Adriano
AU - Mazzini, Letizia
N1 - Funding Information:
Adriano Chiò serves on scientific advisory boards for Mitsubishi Tanabe, Roche, Biogen, and Cytokinetics and has received a research grant from Italfarmaco. Andrea Calvo has received research grant from Cytokinetics.
Publisher Copyright:
© 2020 World Federation of Neurology on behalf of the Research Group on Motor Neuron Diseases.
Copyright:
Copyright 2020 Elsevier B.V., All rights reserved.
PY - 2020/7/2
Y1 - 2020/7/2
N2 - Background: A cognitive impairment, ranging from frontotemporal dementia (FTD) to milder forms of dysexecutive or behavioral dysfunction, is detected in 30–50% of patients affected by amyotrophic lateral sclerosis (ALS) at diagnosis. Such condition considerably influences the prognosis, and possibly impacts on the decision-making process with regards to end-of-life choices. The aim of our study is to examine the changes of cognitive and behavioral impairment in a large population of ALS from the time of diagnosis to a 6-month follow-up (IQR 5.5–9.0 months), and to examine to what extent the progression of cognitive impairment affects survival time and rate of disease progression. Methods: We recruited 146 ALS patients classified according to revised criteria of ALS and FTD spectrum disorder. In a multidisciplinary setting, during two subsequent visits we examined clinical features with ALSFRS-r score, FVC% and BMI, and cognitive status with an extensive neuropsychological evaluation. Results: At second examination, one-third of patients showed a worsening of cognitive impairment, namely 88% of ALSbi, 27% of ALSci, 40% of ALScbi, and, interestingly, also 24% of cognitive normal ALS developed a significant cognitive dysfunction. We find that those who changed their cognitive status presented a lower ALSFRS-r score at t1 and a shorter survival time compared to those who did not change, regardless of the type of cognitive impairment. Conclusion: We show how cognitive disorders in ALS patients can not only be present at diagnosis, but also manifest during disease and influence the progression of motor deficit and the prognosis.
AB - Background: A cognitive impairment, ranging from frontotemporal dementia (FTD) to milder forms of dysexecutive or behavioral dysfunction, is detected in 30–50% of patients affected by amyotrophic lateral sclerosis (ALS) at diagnosis. Such condition considerably influences the prognosis, and possibly impacts on the decision-making process with regards to end-of-life choices. The aim of our study is to examine the changes of cognitive and behavioral impairment in a large population of ALS from the time of diagnosis to a 6-month follow-up (IQR 5.5–9.0 months), and to examine to what extent the progression of cognitive impairment affects survival time and rate of disease progression. Methods: We recruited 146 ALS patients classified according to revised criteria of ALS and FTD spectrum disorder. In a multidisciplinary setting, during two subsequent visits we examined clinical features with ALSFRS-r score, FVC% and BMI, and cognitive status with an extensive neuropsychological evaluation. Results: At second examination, one-third of patients showed a worsening of cognitive impairment, namely 88% of ALSbi, 27% of ALSci, 40% of ALScbi, and, interestingly, also 24% of cognitive normal ALS developed a significant cognitive dysfunction. We find that those who changed their cognitive status presented a lower ALSFRS-r score at t1 and a shorter survival time compared to those who did not change, regardless of the type of cognitive impairment. Conclusion: We show how cognitive disorders in ALS patients can not only be present at diagnosis, but also manifest during disease and influence the progression of motor deficit and the prognosis.
KW - Amyotrophic Lateral Sclerosis
KW - cognitive impairment
KW - fronto-temporal dementia
KW - longitudinal study
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U2 - 10.1080/21678421.2020.1771732
DO - 10.1080/21678421.2020.1771732
M3 - Article
C2 - 32484726
AN - SCOPUS:85086779142
VL - 21
SP - 373
EP - 379
JO - Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
JF - Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
SN - 2167-8421
IS - 5-6
ER -