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Item type:Publication, Inside minds, beneath diseases: social cognition in amyotrophic lateral sclerosis-frontotemporal spectrum disorder(2020) ;Patricia Lillo ;Paulo Caramelli ;Gada Musa ;Teresa ParraoRicardo Hughes<jats:sec><jats:title>Objective</jats:title><jats:p>To compare social cognition performance between patients with amyotrophic lateral sclerosis (ALS) and those patients with behavioural variant frontotemporal dementia (bvFTD).</jats:p></jats:sec><jats:sec><jats:title>Methods</jats:title><jats:p>We included 21 participants with ALS, 20 with bvFTD and 21 healthy controls who underwent a comprehensive cognitive battery, including the short version of the Social Cognition and Emotional Assessment (Mini-SEA), which comprises the <jats:italic>faux pas</jats:italic> test and Facial Emotion Recognition Test (FERT); Mini-Mental State Examination; Frontal Assessment Battery; lexical fluency (F-A-S), category fluency (animals/minute), digit span (direct and backwards) tests and the Hayling test. A post hoc analysis was conducted with the patients with ALS divided into two subgroups: patients without cognitive impairment (ALScn; n=13) and patients with cognitive impairment (ALSci; n=8).</jats:p></jats:sec><jats:sec><jats:title>Results</jats:title><jats:p>No significant difference was noted between participant groups in terms of the age, sex and education. ALS-total group and patients with bvFTD had similar disease durations. Patients with ALSci performed poorly when compared with controls with regard to the FERT (p<0.001), the <jats:italic>faux pas</jats:italic> (p<0.004) and the Mini-SEA (p<0.002) total scores. Moreover, patients with bvFTD performed poorly in comparison with controls in executive and social cognition tests. The performance of patients with ALSci was similar to that of patients with bvFTD, while the performance of patients with ALScn was similar to that of controls.</jats:p></jats:sec><jats:sec><jats:title>Discussion</jats:title><jats:p>Our findings support a cognitive continuum between ALS and bvFTD and shed light on the cognitive heterogeneity of ALS, expanding its possible neuropsychological profiles.</jats:p></jats:sec>8Scopus© Citations 9 - Some of the metrics are blocked by yourconsent settings
Item type:Publication, A proposal for new diagnostic criteria for ALS(2020) ;Jeremy M. Shefner ;Ammar Al-Chalabi ;Mark R. Baker ;Li-Ying CuiMamede de CarvalhoScopus© Citations 405 1 - Some of the metrics are blocked by yourconsent settings
Item type:Publication, ALS-linked protein disulfide isomerase variants cause motor dysfunction(2016) ;Ute Woehlbier ;Alicia Colombo ;Mirva J Saaranen ;Viviana PérezJorge OjedaScopus© Citations 99 1 - Some of the metrics are blocked by yourconsent settings
Item type:Publication, ALS deficiency caused by an exon 2 deletion and a novel missense variant in the gene encoding ALS(2019) ;Gonzalo Dominguez-Menéndez ;Helena Poggi Mayorga ;Mónica Arancibia; Alejandro Martinez-AguayoScopus© Citations 6 2 - Some of the metrics are blocked by yourconsent settings
Item type:Publication, Chaperone Mediated Autophagy Degrades TDP-43 Protein and Is Affected by TDP-43 Aggregation(2020) ;Fernando Ormeño; ;José Moreno ;Felipe RiquelmeJaviera RiosScopus© Citations 49 2