The Edinburgh Cognitive and Behavioural ALS Screen in a Chinese Amyotrophic Lateral Sclerosis Population

Ye, Shan; Ying, Jianming; Li, Chengyu; He, Ji; Liu, Xiaolu; Fan, Dongsheng

doi:10.1371/journal.pone.0155496

Cited by 63 publications

(60 citation statements)

References 36 publications

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“…based on ACE-III and FAB, respectively This is similar to the rates of cognitive impairment found in previous studies [13,16] ( Table 2). The prevalence of behavioral impairment in our cohort is also just over 30% and approximately 40% based on ALS-FTD-Q and MiND-B.…”

Section: Discussionsupporting

confidence: 90%

“…Age and lower levels of education have been observed in several previous ALS studies as risk factors for cognitive impairment [13,21,50], although others found no significant effect of these factors [35,51,52]. Using age corrected or education level corrected scoring guide would be desirable in future studies of cognition in ALS.…”

Section: Accepted Manuscriptmentioning

confidence: 97%

“…Therefore a variety of simple tools have been used to screen for cognitive impairment in ALS. These include Addenbrooke's cognitive examination-III (ACE-III), an updated version of the ACE-R [5][6][7][8], the Frontal assessment battery (FAB) [9][10][11], the Edinburgh cognitive and behavioral ALS screen (ECAS), which includes tests of social cognition, [12][13][14][15][16] and the ALS cognitive behavioral screen (ALS CBS) [17]. Table 1 shows the elements tested in the different tools.…”

Section: Accepted Manuscript Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Screening for cognitive and behavioural impairment in patients with amyotrophic lateral sclerosis: frequency of abnormality and effect on survival

Alruwaili

Henderson

et al. 2017

J Neurol Neurosurg Psychiatry

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Section: Discussionsupporting

confidence: 90%

Section: Accepted Manuscriptmentioning

confidence: 97%

Section: Accepted Manuscript Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Screening for cognitive and behavioural impairment in patients with amyotrophic lateral sclerosis: frequency of abnormality and effect on survival

Alruwaili

Henderson

et al. 2017

J Neurol Neurosurg Psychiatry

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“…Not only has the ECAS shown high sensitivity and specificity to cognitive impairment against a full neuropsychological battery (17)(18)(19)(20)(21), it has high clinical utility in describing the nature of these impairments. Monitoring progression of cognitive and behavioural symptoms may have important implications for patient management, treatment, prognosis, end-oflife decision making, and caregiver burden (22)(23)(24)(25)(26)(27)(28).…”

Section: Discussionmentioning

confidence: 99%

“…The ECAS is a short screening tool designed with high clinical utility and is administrable by non-neuropsychological health care professionals. It has been validated against a comprehensive neuropsychological battery in Scottish (17), German/Swiss-German (15,18), Italian (19), Chinese (20), and Irish populations (21).…”

Section: Introductionmentioning

confidence: 99%

ECAS A-B-C: alternate forms of the Edinburgh Cognitive and Behavioural ALS Screen

Crockford

Kleynhans

Wilton

et al. 2017

Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

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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.

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Novel mutation in optineurin causing aggressive ALS+/−frontotemporal dementia

Feng

Che

Feng

et al. 2019

Ann Clin Transl Neurol

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ObjectiveMutations in optineurin (OPTN) have been identified in familial and sporadic amyotrophic lateral sclerosis (ALS). We screened a cohort of Chinese patients for mutations in optineurin. We also performed an extensive literatures review of all mutations in optineurin identified previously to detect genotype–phenotype associations.MethodsAll 16 exons of the OPTN gene in a cohort of 15 familial ALS indexes and 275 sporadic ALS patients of Chinese origin were sequenced by targeted next generation sequencing.ResultsTwo known heterozygous missense mutations in the OPTN, c.1481T> G (p.L494W), and c.1546G> C (p.E516Q), as well as one novel heterozygous missense mutation c.1690G> C (p.D564H) were each detected in one sporadic ALS patient. The patient carrying the p.E516Q mutation developed clinical features of ALS‐frontotemporal dementia (FTD) and the patient carrying the p.D564H mutation showed a phenotype of ALS. They both had an aggressive course, with a survival of 18 and 14 months respectively. Literature review showed that the clinical phenotypes in OPTN mutated ALS were not homogeneous, although some individuals showed a relatively slow progression and a long duration, some mutations carriers developed an aggressive progression and a short survival.Interpretation OPTN mutations contribute to ALS in Chinese population and account for 0.8% of sporadic ALS patients and 1.5% of familial ALS in the pooled Chinese ALS cohorts. Mutations in optineurin can cause aggressive ALS+/−FTD.

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The Edinburgh Cognitive and Behavioural ALS Screen in a Chinese Amyotrophic Lateral Sclerosis Population

Cited by 63 publications

References 36 publications

Screening for cognitive and behavioural impairment in patients with amyotrophic lateral sclerosis: frequency of abnormality and effect on survival

Screening for cognitive and behavioural impairment in patients with amyotrophic lateral sclerosis: frequency of abnormality and effect on survival

ECAS A-B-C: alternate forms of the Edinburgh Cognitive and Behavioural ALS Screen

Novel mutation in optineurin causing aggressive ALS+/−frontotemporal dementia

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