期刊名称：Revue de Neuropsychologie Neurosciences Cognitives et Cliniques
印刷版ISSN：2101-6739
电子版ISSN：2102-6025
出版年度：2010
卷号：2
期号：4
页码：283-291
DOI：10.1684/nrp.2010.0099
出版社：John Libbey Eurotext
摘要：Authors Audrey Mondou , Béatrice Desgranges , Christelle Giry , Nadège Loisel , Francis Eustache , Fausto Viader , Laurence Carluer Inserm – EPHE – Université de Caen/Basse-Normandie, Unité U923, GIP Cyceron, CHU Côte de Nacre, Caen Key words: amyotrophic lateral sclerosis, cognition, behaviour, emotion, fronto-temporal dementia DOI : 10.1684/nrp.2010.0099 Page(s) : 283-91 Published in: 2010 Amyotrophic lateral sclerosis (ALS) is a degenerative disease that affects motor neurons of the spinal cord, brain stem and cerebral cortex. It has been typically regarded as a purely motor neuron disease, but a number of studies have shown that brain lesions extend far beyond motor areas. Recently, neuropsychological studies have been central in establishing the presence of “extra-motor” involvement in ALS. The main deficits concern “frontal” function with the presence of disorders of executive function, behavioural disorders, and sometimes a frontotemporal dementia (FTD). Moreover, many studies report unexpectedly low scores of depression in ALS patients which may suggest an emotional indifference and recent neuropsychological researches suggest an emotional perception failure in ALS.
