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Background: The Edinburgh Cognitive and Behavioural ALS Screen (ECAS) is a short assessment by which neuropsychological symptoms can be detected and quantified in people with ALS. To avoid potential practice effects with repeated administration, here we present alternative versions of the ECAS suitable for measuring change over time., Objective: To develop two alternate versions of the ECAS: ECAS-B and ECAS-C., Method: One hundred and forty-nine healthy adult participants were recruited. Thirty participants completed a pilot study in developing the alternate versions. Two groups of 40 participants were administered the ECAS-B or ECAS-C and compared to published data of the original ECAS (ECAS-A) to determine equivalence. An additional 39 participants were administered the ECAS consecutively, either repeating the original version (ECAS-A-A-A) serially or the different versions (ECAS-A-B-C) to determine potential practice effects. Recordings of assessments were scored by a second researcher to determine inter-rater reliability., Results: No significant differences were found between versions (A, B, C) of the composite performance measures of ALS Specific, ALS Non-Specific, and ECAS Total scores. Repeated serial administration of ECAS-A (A-A-A) produced some practice effects for composite scores, whereas no such effects were found when alternate versions were administered serially (A-B-C). Exceptionally high intra-class correlations were found for all three versions of the ECAS suggesting a high degree of rater agreement., Conclusion: The newly developed alternate forms of the ECAS are both highly equitable to the original ECAS-A and enable avoidance of practice effects, thus supporting their use in measuring cognition and behaviour over time.

Objectives: Executive and behavioural changes are well-recognised in classical amyotrophic lateral sclerosis (ALS), indicating a subclinical behavioural-variant frontotemporal dementia (bvFTD) in some patients. Social cognitive deficits in ALS have been recently described and an impairment was identified on a simple Theory of Mind (ToM) test, which assesses the judgement of the preference of another through direction of eye gaze. The present study further delineated this deficit, by distinguishing between Affective and Cognitive subcomponents, and determining the relationship to behavioural change, levels of empathy and self-awareness., Methods: The Cognitive-Affective Judgement of Preference Test was administered to 33 patients with ALS and 26 controls. Furthermore, a comprehensive neuropsychological battery and detailed behavioural assessment, with measures of empathy and awareness, were included., Results: Patients with ALS showed a significant impairment in Affective ToM only when compared with healthy controls, with a deficit in 36% of patients; 12% showed an isolated Affective ToM deficit while 24% showed more generic ToM dysfunction. A Cognitive ToM deficit was found in 27% of patients, with 3% showing an isolated Cognitive ToM deficit. The patients with ALS showed reduced empathy (Fantasy scale) and increased behavioural dysfunction with high levels of apathy. In addition, patients with either an Affective and/or Cognitive ToM deficit exhibited poor self-awareness of their performance and abnormalities on verbal fluency, while those with an Affective ToM deficit also displayed higher levels of apathy and a naming deficit., Conclusions: Dysfunctional ToM is a prominent feature of the cognitive profile of ALS. This specific difficulty in identifying and distinguishing the feelings and thoughts of another from a self-perspective may underpin the social behavioural abnormalities present in some patients with ALS, manifest as apathy and loss of awareness., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.)

We present a study examining cognitive functions in late non-balanced bilinguals with different levels of second language proficiency. We examined in two experiments a total of 193 mono- and bilingual university students. We assessed different aspects of attention (sustained, selective and attentional switching), verbal fluency (letter and category) as well as picture-word association as a measure of language proficiency. In Experiment 2 we also compared students in their first/initial (Y1) and fourth/final (Y4) year of either language or literature studies. There were no differences between both groups in category fluency. In selective attention, bilinguals outperformed monolinguals in Y1 and this difference remained significant in Y4 despite overall improvement in both groups. Contrasting results were found in attentional switching and letter fluency: while no differences were found in Y1 in both tasks, in Y4 there was an advantage for bilinguals in attentional switching and for monolinguals in letter fluency. We conclude that overall late-acquisition non-balanced bilinguals experience similar cognitive effects as their early-acquisition balanced counterparts. However, different cognitive effects may appear at different stages of adult second language acquisition., (Copyright © 2015 Elsevier B.V. All rights reserved.)

Recent evidence suggests a positive impact of bilingualism on cognition, including later onset of dementia. However, monolinguals and bilinguals might have different baseline cognitive ability. We present the first study examining the effect of bilingualism on later-life cognition controlling for childhood intelligence. We studied 853 participants, first tested in 1947 (age = 11 years), and retested in 2008-2010. Bilinguals performed significantly better than predicted from their baseline cognitive abilities, with strongest effects on general intelligence and reading. Our results suggest a positive effect of bilingualism on later-life cognition, including in those who acquired their second language in adulthood., (© 2014 The Authors Annals of Neurology published by Wiley Periodicals, Inc. on behalf of American Neurological Association. This is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.)

With the population aging and a dramatic increase in the number of senior citizens, public health systems will be increasingly burdened with the need to deal with the care and treatment of individuals with dementia. We review evidence demonstrating how a particular experience, bilingualism, has been shown to protect cognitive function in older age and delay onset of symptoms of dementia. This paper describes behavioral and brain studies that have compared monolingual and bilingual older adults on measures of cognitive function or brain structure and reviews evidence demonstrating a protective effect of bilingualism against symptoms of dementia. We conclude by presenting some data showing the potential savings in both human costs in terms of demented patients and economic considerations in terms of public money if symptoms of dementia could be postponed.

The relationship between motor neurone disease (MND) and frontotemporal dementia (FTD) has been a topic of scientific exploration for over hundred years. A connection between both diseases was first postulated in 1932 and has been strengthened by a steady stream of case reports since then. By the late 20th century, the link between both diseases was firmly established, with the resulting condition often referred to as MND/FTD. Several strands of evidence support the notion of an MND/FTD overlap. First, a small but well-documented group of patients present with a full-blown FTD, associated with MND. Second, subtle but characteristic changes in frontal-executive functions and social cognition have been described in non-demented MND patients, often in association with frontal atrophy/hypoactivity on neuroimaging. Third, amyotrophic features have been documented in patients primarily diagnosed with FTD. Moreover, the same genetic defect can lead to FTD and MND phenotypes in different members of the same family. However, as the current research is moving toward a more fine-grained evaluation, an increasingly complex picture begins to emerge. Some features, such as psychotic symptoms or severe language deficits (particularly in comprehension and verb processing), seem to occur more often in MND/dementia than in the classical FTD. On the basis of the review of 100 years of literature as well as 10 years of clinical experience of longitudinal follow-up of MND/dementia patients, this review argues in favor of MND/dementia (or, more precisely, MND/dementia/aphasia) as a separate clinical entity, not sufficiently explained by a combination of MND and FTD.

Studies examining the potential effects of bilingualism on interference suppression show inconsistent results. Our study approaches this topic by distinguishing two potential subcomponents within interference suppression (i.e., Stimulus-Stimulus and Stimulus-Response conflict). We investigated the two subcomponents through their operationalisation in different tasks and examined the role of language proficiency in modulating it. A sample of 111 young adult participants performed four non-linguistic cognitive tasks measuring both visual and auditory domains of cognitive control. Bilinguals outperformed monolinguals in tasks involving Stimulus-Stimulus conflict, but showed comparable performance in tasks involving Stimulus-Response conflict. Specific effects of language proficiency on cognitive control were observed: group differences in auditory inhibition and visual orienting were only observed between high-proficient bilinguals and monolinguals. Taken together, types of conflicts involved in interference tasks and language proficiency could differentially affect performance in monolinguals and bilinguals. [ABSTRACT FROM AUTHOR]

Edinburgh Cognitive and Behavioural ALS Screen (ECAS) is a brief, standardized assessment of cognitiveimpairment inamyotrophic lateral sclerosis. Objective: We aimed to createa normative dataset for the ECAS Czech version (ECAS-CZ) in order to make the assessment applicable for clinical settings. Method: Included were 102 healthy participants (mean age: 54.92 ± 14.55; education: 14.52 ± 2.44; 54:48 females/males) that fulfilled rigorous exclusion criteria and controlled for depressive symptoms. Results: The internal consistency of ECAS-CZ was acceptable (Cronbach's α =.69). We found medium correlations (rho ≈.5) of age and education with ECAS-CZ Total score but not with gender. Cut-offs with −2 SD's threshold are presented for the differentiation of cognitive impairment. We report percentile values for ECAS-CZ Total including all subscales. Conclusion: We provide normative values for ECAS-CZ that are well suited for the detection of cognitive impairment in clinical settings especially for patients with ALS. Supplemental data for this article is available online at https://doi.org/10.1080/13854046.2021.1978553. [ABSTRACT FROM AUTHOR]

The Edinburgh Cognitive and Behavioural ALS Screen (ECAS) was developed to assess cognitive and behavioural changes in an anterior frontotemporal syndrome (executive functions, language, fluency and behaviour), common in Amyotrophic Lateral Sclerosis (ALS) and also assesses posterior cerebral dysfunction (memory and visuospatial abilities). Objectives: To validate the ECAS in behavioural variant Frontotemporal Dementia (bvFTD) without ALS, as compared with Alzheimer's disease (AD), against comprehensive neuropsychological assessment. Compare its sensitivity to that of the Addenbrooke's Cognitive Examination (ACE‐III) and investigate behavioural changes in both types of dementia. Methods: Retrospective study of 16 people with bvFTD (without ALS), 32 with AD, and 48 healthy controls completed the ECAS, ACE‐III and extensive neuropsychological assessment. Results: The ECAS showed higher sensitivity (94%) and marginally lower specificity (96%) than the ACE‐III for both the bvFTD and AD groups. The anterior composite subscore was sensitive for bvFTD (94%), and slightly less so for AD (84%), while the posterior composite subscore was sensitive for AD (97%), and less so for bvFTD (75%). All people with bvFTD that were impaired on the ECAS total and anterior composite scores were also impaired on the anterior function's tests of the neuropsychological assessment. A cut‐off of four or more behavioural domains affected differentiated well between the bvFTD and AD groups, while a qualitative analysis of the behavioural interview found different themes between groups. Conclusions: The ECAS is a valid and sensitive assessment for bvFTD without ALS and for AD. The carer behavioural interview makes it particularly suitable to detect behavioural abnormalities related to frontal lobe disorders Key Points: The ECAS showed higher sensitivity (94%) and specificity (96%) for both the bvFTD and AD groups than the ACE‐III.The ECAS performed well against standard comprehensive neuropsychological assessment with perfect concordance between ECAS Total and Anterior functions composite scores, and performance on the anterior functions' tests of the neuropsychological assessment for the bvFTD group. The ECAS total and posterior functions composite scores also showed good validity against the posterior functions' tests of the neuropsychology assessment for the AD group.The most recurrent abnormal behaviour for the bvFTD group was loss of sympathy/empathy (100%), while the most recurrent theme for AD was loss of interest in normal activities (56%). Important thematic differences between diagnoses were (26%) lack of awareness and (66%) lack of manners in people with bvFTD, while AD patients (33%) had a loss of self‐confidence. [ABSTRACT FROM AUTHOR]

While much of the literature on bilingualism and cognition focuses on group comparisons (monolinguals vs bilinguals or language learners vs controls), here we examine the potential differential effects of intensive language learning on subjects with distinct language experiences and demographic profiles. Using an individual differences approach, we assessed attentional performance from 105 university-educated Gaelic learners aged 21–85. Participants were tested before and after beginner, elementary, and intermediate courses using tasks measuring i.) sustained attention, ii.) inhibition, and iii.) attention switching. We examined the relationship between attentional performance and Gaelic level, previous language experience, gender, and age. Gaelic level predicted attention switching performance: those in higher levels initially outperformed lower levels, however lower levels improved the most. Age also predicted performance: as age increased attention switching decreased. Nevertheless, age did not interact with session for any attentional measure, thus the impact of language learning on cognition was detectable across the lifespan. [ABSTRACT FROM AUTHOR]

Objective: Approximately 35% of patients with amyotrophic lateral sclerosis (ALS) exhibit mild cognitive deficits in executive functions, language and fluency, without dementia. The precise pathology of these extramotor symptoms has remained unknown. This study aimed to determine the pathological correlate of cognitive impairment in patients with non-demented ALS.Methods: In-depth neuropathological analysis of 27 patients with non-demented ALS who had undergone cognitive testing (Edinburgh Cognitive and Behaviour ALS Screen (ECAS)) during life. Analysis involved assessing 43 kDa Tar-DNA binding protein (TDP-43) accumulation in brain regions specifically involved in executive functions, language functions and verbal fluency to ascertain whether functional deficits would relate to a specific regional distribution of pathology.Results: All patients with cognitive impairment had TDP-43 pathology in extramotor brain regions (positive predictive value of 100%). The ECAS also predicted TDP-43 pathology with 100% specificity in brain regions associated with executive, language and fluency domains. We also detected a subgroup with no cognitive dysfunction, despite having substantial TDP-43 pathology, so called mismatch cases.Conclusions: Cognitive impairment as detected by the ECAS is a valid predictor of TDP-43 pathology in non-demented ALS. The profile of mild cognitive deficits specifically predicts regional cerebral involvement. These findings highlight the utility of the ECAS in accurately assessing the pathological burden of disease. [ABSTRACT FROM AUTHOR]

The Edinburgh Cognitive and Behavioral ALS Screen (ECAS) was developed to assess cognitive and behavioral changes common in Amyotrophic Lateral Sclerosis and other diseases affecting motor functions. It focuses on domains typically affected by the frontotemporal syndrome (executive and language functions, fluency and behavior), but assesses also memory and visuospatial functions. Objectives: (A) To investigate the relationship between the ECAS and the Addenbrooke's Cognitive Examination (ACE-III). (B) To investigate the effects of age, education, and IQ on the ECAS and create appropriate cutoff scores to determine abnormality. Methods: (A) 57 healthy participants (aged 35-80) were assessed with the ECAS, the Wechsler Abbreviated Scale of Intelligence (WASI-II), and the ACE-III. (B) 80 healthy participants (aged 51-80) were divided into four groups according to age and education; and were tested with the ECAS and the WASI-II. Results: The ECAS and the ACE-III have a good convergent validity with a significant correlation. Regression analysis revealed that IQ, followed by age, were the strongest predictors of the total ECAS score. IQ predicted 24% of the ECAS and 46% of the ACE-III variance. Education was not a significant predictor over and above IQ for both the ECAS and the ACE-III. Abnormality cutoff scores adjusted for age and education are presented. Conclusions: The ECAS shows good convergent validity with the ACE-III, but is less influenced by intelligence and presents less ceiling effects. The inclusion of an executive function assessment and behavioral interview in the ECAS makes it particularly useful for the assessment of frontal lobe disorders. [ABSTRACT FROM AUTHOR]

Background: Verb fluency (VF) is the less commonly used fluency test, despite several studies suggesting its potential as a neuropsychological assessment tool.Objective: To investigate the presence of VF deficits in mild cognitive impairment (MCI) and mild Alzheimer's disease (AD) dementia; to assess the usefulness of VF in the detection of cognitively healthy (CH) people who will convert to MCI, and from MCI to dementia; and to establish the VF cut-offs useful in the cognitive assessment of Spanish population.Methods: 568 CH, 885 MCI, and 367 mild AD dementia individuals were administered the VF test and a complete neuropsychological battery. Longitudinal analyses were performed in 231 CH and 667 MCI subjects to search for VF predictors of diagnosis conversion.Results: A worsening on VF performance from CH, MCI to AD dementia groups was found. Lower performances on VF were significantly related to conversion from CH to MCI/MCI to dementia. When the effect of time to conversion was analyzed, a significant effect of VF was found on the faster conversion from CH to MCI, but not from MCI to dementia. Moreover, VF cut-off scores and sensitivity/specificity values were calculated for 6 conditions (3 age ranges by 2 educational levels).Conclusion: The VF test may be a useful tool for the differential diagnosis of cognitive failure in the elderly. Since VF deficits seem to take place in early stages of the disease, it is a suitable neuropsychological tool for the detection not only of CH people who will convert to MCI, but also from MCI to dementia. [ABSTRACT FROM AUTHOR]

Bilingualism has been found to delay onset of dementia and this has been attributed to an advantage in executive control in bilinguals. However, the relationship between bilingualism and cognition is complex, with costs as well as benefits to language functions. To further explore the cognitive consequences of bilingualism, the study used Frontotemporal dementia (FTD) syndromes, to examine whether bilingualism modifies the age at onset of behavioral and language variants of Frontotemporal dementia (FTD) differently. Case records of 193 patients presenting with FTD (121 of them bilingual) were examined and the age at onset of the first symptoms were compared between monolinguals and bilinguals. A significant effect of bilingualism delaying the age at onset of dementia was found in behavioral variant FTD (5.7 years) but not in progressive nonfluent aphasia (0.7 years), semantic dementia (0.5 years), corticobasal syndrome (0.4 years), progressive supranuclear palsy (4.3 years) and FTD-motor neuron disease (3 years). On dividing all patients predominantly behavioral and predominantly aphasic groups, age at onset in the bilingual behavioral group (62.6) was over 6 years higher than in the monolingual patients (56.5, p=0.006), while there was no difference in the aphasic FTD group (60.9 vs. 60.6 years, p=0.851). The bilingual effect on age of bvFTD onset was shown independently of other potential confounding factors such as education, gender, occupation, and urban vs rural dwelling of subjects. To conclude, bilingualism delays the age at onset in the behavioral but not in the aphasic variants of FTD. The results are in line with similar findings based on research in stroke and with the current views of the interaction between bilingualism and cognition, pointing to advantages in executive functions and disadvantages in lexical tasks. [ABSTRACT FROM AUTHOR]

Cognitive, behavioural, and functional assessment is crucial in longitudinal studies of neurodegenerative dementias (NDD). Central issues, such as the definition of the study population (asymptomatic, at risk, or individuals with dementia), the detection of change/decline, and the assessment of relevant outcomes depend on quantitative measures of cognitive, behavioural, and functional status. Currently, we are far from having available reliable protocols and tools for the assessment of dementias in Europe. The main problems are the heterogeneity of the tools used across different European countries, the lack of standardisation of administration and scoring methods across centres, and the limited information available about the psychometric properties of many tests currently in widespread use. This situation makes it hard to compare results across studies carried out in different centres, thus hampering research progress, in particular towards the contribution to a “big data” common data set. We present here the results of a project funded by the Joint Program for Neurodegenerative Diseases (JPND) and by the Italian Ministry of Health. The project aimed at providing a consensus framework for the harmonisation of assessment tools to be applied to research in neurodegenerative disorders affecting cognition across Europe. A panel of European experts reviewed the current methods of neuropsychological assessment, identified pending issues, and made recommendations for the harmonisation of neuropsychological assessment of neurodegenerative dementias in Europe. A consensus was achieved on the general recommendations to be followed in developing procedures and tools for neuropsychological assessment, with the aim of harmonising tools and procedures to achieve more reliable data on the cognitive-behavioural examination. The results of this study should be considered as a first step to enhancing a common view and practise on NDD assessment across European countries. [ABSTRACT FROM AUTHOR]

Literature on bilingualism and cognition is characterised by a large amount of conflicting evidence. In some studies, bilinguals perform better then monolinguals on executive tasks involving inhibition, monitoring and switching but are slower on tasks of lexical processing. Other studies don't find any significant effects and challenge the very existence of cognitive differences between monolinguals and bilinguals. In this paper I question the assumption that different studies performed in different parts of the world should yield the same results. I argue that the environment (in the widest sense of the word) in which an experiment is conducted can exert profound influence on its outcome. Against the background of the current debate about the replication crisis in science, I propose that conflicting evidence is not a threat to the trustworthiness of scientific research but a sign of the health of a discipline and a welcome opportunity to identify new relevant variables. [ABSTRACT FROM AUTHOR]

We investigated the impact of a short intensive language course on attentional functions. We examined 33 participants of a one-week Scottish Gaelic course and compared them to 34 controls: 16 active controls who participated in courses of comparable duration and intensity but not involving foreign language learning and 18 passive controls who followed their usual routines. Participants completed auditory tests of attentional inhibition and switching. There was no difference between the groups in any measures at the beginning of the course. At the end of the course, a significant improvement in attention switching was observed in the language group (p < .001) but not the control group (p = .127), independent of the age of participants (18–78 years). Half of the language participants (n = 17) were retested nine months after their course. All those who practiced Gaelic 5 hours or more per week improved from their baseline performance. In contrast, those who practiced 4 hours or fewer showed an inconsistent pattern: some improved while others stayed the same or deteriorated. Our results suggest that even a short period of intensive language learning can modulate attentional functions and that all age groups can benefit from this effect. Moreover, these short-term effects can be maintained through continuous practice. [ABSTRACT FROM AUTHOR]

Our objective was to assess the validity of the Edinburgh Cognitive and Behaviour ALS Screen (ECAS), a multi-domain screen designed to detect cognitive deficits in patients with motor disorders. Forty ALS patients (without pre-diagnosed dementia) and 40, age-, gender- and education-matched healthy controls were recruited. All participants underwent extensive neuropsychological assessment and the ECAS. Performance at neuropsychological assessment across five domains (fluency, executive function, language, memory and visuospatial function) was compared to the ECAS ALS-Specific (fluency, executive functions and social cognition, language), ALS Non-specific (memory, visuospatial functions), and Total scores. Data from the healthy controls produced population-based abnormality cut-offs: composite score performance ≤ 2 SD in any domain classified impairment at neuropsychological assessment. Thirty-three percent of patients were impaired, most commonly in a single domain (executive or language dysfunction). Receiver Operator Curve (ROC) analyses using ECAS Total scores and ALS-Specific scores revealed 85% sensitivity and 85% specificity in the detection of cognitive impairment characteristic of ALS (fluency, executive function, language). A five-point borderline range produced optimal values (ALS-Specific Score 77-82, and ECAS-Total Score 105-110). In conclusion, validation against gold standard extensive neuropsychology demonstrated that the ECAS is a screening tool with high sensitivity and specificity to impairment characteristic of ALS. [ABSTRACT FROM AUTHOR]

The Edinburgh Cognitive and Behavioural ALS Screen (ECAS) has recently been developed as a fast and easy cognitive screening tool specifically designed for patients with motor impairments in routine clinical use. The German/Swiss-German version of the ECAS was validated in a German-Swiss consortium. One hundred and thirty-six non-demented ALS patients and 160 healthy controls were included in the study. In addition, the Frontal Assessment Battery (FAB), Montreal Cognitive Assessment (MoCA) and Consortium to Establish a Registry for Alzheimer's Disease plus Scale (CERAD plus) were administered to subgroups of patients. Results showed that administration of ECAS was fast (mean 24 min). Similar to the population in the UK version, ALS patients performed significantly worse in the ALS-specific functions ( p < 0.001), specifically in the domain of language ( p < 0.001), verbal fluency ( p = 0.005) and executive functions ( p = 0.02), but not for the non-ALS-specific functions. Carers reported behavioural abnormalities in about 30% and psychotic symptoms in 6% of the patients. Compared to ECAS, FAB, MoCA and CERAD were more generic and performance was not adjusted to motor speed. We conclude that the German/Swiss-German version of the ECAS is a fast and easy to administer cognitive screening instrument sensitive for ALS-specific dysfunctions and behaviour change. [ABSTRACT FROM AUTHOR]

Recent studies, using predominantly visual tasks, indicate that early bilinguals tend to outperform monolinguals on attention tests. It remains less clear whether such advantages extend to those bilinguals who have acquired their second language later in life.We examined this question in 38 monolingual and 60 bilingual university students. The bilingual group was further subdivided into early childhood (ECB), late childhood (LCB), and early adulthood bilinguals (EAB). The assessment consisted of five subtests from the clinically validated Test of Everyday Attention (TEA). Overall, bilinguals outperformed monolinguals on auditory attention tests, but not on visual search tasks. The latter observation suggests that the differences between bilinguals and monolinguals are specific and not due to a generally higher cognitive performance in bilinguals. Within the bilingual group, ECB showed a larger advantage on attention switching, LCB/EAB on selective attention. We conclude that the effects of bilingualism extend into the auditory domain and are not confined to childhood bilinguals, although their scope might be slightly different in early and late bilinguals. [ABSTRACT FROM AUTHOR]

This study presents the Edinburgh Cognitive and Behavioural ALS Screen (ECAS), developed for ALS patients with physical disability for use by health care professionals. The screen is designed to detect the specific profile of cognition and behaviour changes in ALS and to differentiate it from other disorders. Forty-eight ALS patients (none with evident dementia), 40 healthy controls and 20 carers were recruited. The ECAS, a 15-20-min screen, includes an ALS-Specific score (executive functions and social cognition; fluency; language); an ALS Non-specific score (memory; visuospatial functions); and a carer behaviour screen of five domains characteristic of frontotemporal dementia (FTD). Data from healthy controls produced abnormality cut-offs of 77/100 ALS-Specific score; 24/36 ALS Non-specific score; 105/136 ECAS Total. Twenty-nine percent of patients showed abnormal ALS-Specific scores, and 6% also showed abnormal ALS Non-specific scores. The most prevalent deficit occurred in language functions (35%) followed by executive functions and fluency (23% each). Forty percent of carers reported behaviour change in at least one domain, while 15% met criteria for possible FTD. In conclusion, the ECAS is an effective within-clinic assessment for ALS that determines the presence, severity and type of cognitive and/or behavioural changes, an essential first step to managing these symptoms. [ABSTRACT FROM AUTHOR]