scholarly journals Screening for cognition in amyotrophic lateral sclerosis: test characteristics of a new screen

2021 ◽  
Author(s):  
Emma Beeldman ◽  
Rosanne Govaarts ◽  
Marianne de Visser ◽  
Michael A. van Es ◽  
Yolande A. L. Pijnenburg ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Cognitive Impairment ◽  
Sensitivity And Specificity ◽  
Roc Curve ◽  
Area Under The Curve ◽  
High Sensitivity ◽  
Screening Methods ◽  
Cognitive Screening ◽  
Test Characteristics ◽  
Lateral Sclerosis

AbstractCognitive and behavioural impairment in amyotrophic lateral sclerosis (ALS) negatively influences the quality of life and survival, and, therefore, screening for these impairments is recommended. We developed a cognitive screening tool, the amyotrophic lateral sclerosis–frontotemporal dementia–cognitive screen (ALS–FTD–Cog) and aimed to validate it in patients with ALS. During the current study, the Edinburgh Cognitive and Behavioural ALS Screen (ECAS) was published and we, therefore, decided to compare these two cognitive screening methods. The ALS–FTD–Cog was administered to 72 patients with ALS, 21 patients with behavioural variant FTD (bvFTD) and 34 healthy controls. Twenty-nine patients with ALS underwent the ECAS. ROC curve analyses were performed and sensitivity and specificity of the ALS–FTD–Cog and ECAS were calculated, with a neuropsychological examination (NPE) as the gold standard. Cognitive impairment was present in 28% of patients with ALS. ROC curve analyses of the ALS–FTD–Cog and ECAS showed an area under the curve (AUC) of 0.72 (95% CI 0.58–0.86) and 0.95 (95% CI 0.86–1.03), respectively. Compared to a full NPE, sensitivity and specificity of the ALS–FTD–Cog were 65.0% and 63.5% and of the ECAS 83.3% and 91.3%, respectively. The sensitivity and specificity of the ALS–FTD–Cog in patients with bvFTD were 94.4% and 100%, respectively. Test characteristics of the ALS–FTD–Cog were moderate, suggesting restricted practical value, as compared to a comprehensive NPE. The ECAS had an excellent AUC and high sensitivity and specificity, indicating that it is a valid screening instrument for cognitive impairment in ALS.

Effect of racial background on motor cortical function as measured by threshold tracking transcranial magnetic stimulation

2021 ◽  
Vol 126 (3) ◽  
pp. 840-844
Author(s):  
Yo-Ichi Suzuki ◽  
Yan Ma ◽  
Kazumoto Shibuya ◽  
Sonoko Misawa ◽  
Tomoki Suichi ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Transcranial Magnetic Stimulation ◽  
Sensitivity And Specificity ◽  
Magnetic Stimulation ◽  
High Sensitivity ◽  
Cortical Function ◽  
Asian Patients ◽  
Threshold Tracking ◽  
Racial Background ◽  
Lateral Sclerosis

Threshold tracking transcranial magnetic stimulation (TT-TMS) was applied for Caucasians, Han Chinese, and Japanese. No significant differences were found in TMS excitability indexes among races. Recent studies have disclosed that TT-TMS indexes differentiate amyotrophic lateral sclerosis (ALS) from ALS mimic disorders, with high sensitivity and specificity, in Caucasians. This study suggested that TT-TMS can be applied for the ALS diagnosis in Asian patients, as well as Caucasians.

scholarly journals TDP-43 and Phosphorylated TDP-43 Levels in Paired Plasma and CSF Samples in Amyotrophic Lateral Sclerosis

2021 ◽  
Vol 12 ◽  
Author(s):  
Yuting Ren ◽  
Siyuan Li ◽  
Siyu Chen ◽  
Xiaosun Sun ◽  
Fei Yang ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Sandwich Elisa ◽  
Area Under The Curve ◽  
High Sensitivity ◽  
Sporadic Amyotrophic Lateral Sclerosis ◽  
Healthy Controls ◽  
Clinical Indicators ◽  
Progression Of Disease ◽  
Als Patients ◽  
Lateral Sclerosis

Objective: The aim of this study was to measure both plasma and cerebrospinal fluid (CSF) TAR DNA-binding protein 43 (TDP-43) and phosphorylated TDP-43 (pTDP-43) levels in sporadic amyotrophic lateral sclerosis (sALS) patients, and to compare them with that of healthy controls. The correlation between plasma or CSF TDP-43/pTDP-43 and clinical indicators of ALS patients was assessed.Methods: Paired plasma and CSF TDP-43/pTDP-43 levels in 69 ALS patients and 59 healthy controls were measured by sandwich ELISA. Time to generalization (TTG), an indicator suggested that the time of symptoms spreading from spinal or bulbar localization to both, was evaluated in all patients screened for mutations in genes associated with ALS.Results: Both of the plasma TDP-43 and pTDP-43 levels were significantly higher in ALS patients than HCs (P < 0.001). The pTDP-43/TDP-43 ratios in plasma were significantly higher in HCs than ALS patients (P < 0.001). The area under the curve (AUC) value was 0.924 for plasma TDP-43 level, with a 91.3% sensitivity and 91.5% specificity. Moreover, the correlation between plasma and CSF TDP-43 was observed in each ALS patient (r = 0.195, P = 0.027). A correlation between CSF pTDP-43 levels and the ALSFRS-R (r = −0.245; P = 0.042) was established. A correlation was observed between plasma TDP-43 levels and TTG in ALS patients, which indicated that high levels of plasma TDP-43 correlated with prolonged TTG (r = 0.415; P = 0.004).Conclusion: The plasma TDP-43 and pTDP-43 levels might play an important role in diagnosis in the future study of ALS. The plasma TDP-43 might differentiate ALS and HC groups based on high sensitivity and specificity, and as an indicator of progression of disease.

scholarly journals The Edinburgh cognitive and behavioral amyotrophic lateral sclerosis screen (ECAS): sensitivity in differentiating between ALS and Alzheimer’s disease in a Greek population

2021 ◽  
Author(s):  
Panagiotis Kourtesis ◽  
Foteini Christidi ◽  
Eleni Margioti ◽  
Christina Demenega ◽  
Mihail Rentzos ◽  
...  
Keyword(s):  
Sensitivity And Specificity ◽  
Convergent Validity ◽  
Normative Data ◽  
Roc Analysis ◽  
Area Under The Curve ◽  
High Sensitivity ◽  
Operating Characteristics ◽  
Als Patients ◽  
Lateral Sclerosis ◽  
Better Than

Objectives: (1) Adapt the ECAS into Greek, validate it in ALS patients and compare with the ALS-CBS. (2) Determine the sensitivity and specificity of ECAS in the differentiation between AD and non-demented ALS patients as compared with the ACE-III and mini-ACE. Methods: ALS patients (n = 28) were recruited and AD patients (n = 26) were matched in age, sex, and education with ALS patients (n = 24). The normative data were derived from a random sample of controls (n = 52). Bayes correlation analysis was conducted to examine convergent validity. Bayes t-test was performed to assess between groups’ differences. Receiver operating characteristics (ROC) curve analyses and area under the curve (AUC) were implemented to appraise the sensitivity and specificity in the differentiation between the AD and non-demented ALS patients. Results: The ECAS and its sub-scores in addition to the behavior interview demonstrated robust correlations with the ALS-CBS. Impairment in language and verbal fluency were the most prominent deficits in the ALS patients. The most frequently reported change was apathy. The ROC analysis demonstrated that the ECAS-ALS nonspecific score (comprising memory and visuospatial domains) is the most sensitive and specific in differentiating the AD from ALS patients. The other measures expressed high sensitivity, yet a poor specificity. Conclusions: The ECAS is a multi-purpose screening tool. The ECAS-ALS specific appraises the whole spectrum of the highly prevalent cognitive impairments in ALS. The ECAS-ALS nonspecific (memory and visuospatial) is a sensitive score to detect AD related deficits and is able to differentiate the AD from the non-demented ALS patients better than the ACE-III and mini-ACE.

Cognitive impairment in amyotrophic lateral sclerosis: the hidden challenges

2016 ◽  
Vol 12 (1) ◽  
pp. 17-21
Author(s):  
Malabika Ghosh ◽  
Pauline Callagher ◽  
Robert Addison–Jones ◽  
Wendy Bennett ◽  
Amy Parkes ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Cognitive Impairment ◽  
Lateral Sclerosis

scholarly journals Cognitive reserve in amyotrophic lateral sclerosis (ALS): a population-based longitudinal study

2021 ◽  
pp. jnnp-2020-324992
Author(s):  
Emmet Costello ◽  
James Rooney ◽  
Marta Pinto-Grau ◽  
Tom Burke ◽  
Marwa Elamin ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Cognitive Impairment ◽  
Cognitive Decline ◽  
Cognitive Reserve ◽  
Population Based ◽  
Protective Role ◽  
Drop Out ◽  
Longitudinal Change ◽  
Activity Data ◽  
Lateral Sclerosis

BackgroundAmyotrophic lateral sclerosis (ALS) is often associated with cognitive and/or behavioural impairment. Cognitive reserve (CR) may play a protective role in offsetting cognitive impairment. This study examined the relationship between CR and longitudinal change in cognition in an Irish ALS cohort.MethodsLongitudinal neuropsychological assessment was carried out on 189 patients over 16 months using the Edinburgh cognitive and behavioural ALS screen (ECAS) and an additional battery of neuropsychological tests. CR was measured by combining education, occupation and physical activity data. Joint longitudinal and time-to-event models were fitted to investigate the associations between CR, performance at baseline and decline over time while controlling for non-random drop-out.ResultsCR was a significant predictor of baseline neuropsychological performance, with high CR patients performing better than those with medium or low CR. Better cognitive performance in high CR individuals was maintained longitudinally for ECAS, social cognition, executive functioning and confrontational naming. Patients displayed little cognitive decline over the course of the study, despite controlling for non-random drop-out.ConclusionsThese findings suggest that CR plays a role in the presentation of cognitive impairment at diagnosis but is not protective against cognitive decline. However, further research is needed to examine the interaction between CR and other objective correlates of cognitive impairment in ALS.

A-151 Cognitive Impairment in Amyotrophic Lateral Sclerosis

2021 ◽  
Vol 36 (6) ◽  
pp. 1205-1205
Author(s):  
Etiane Navarro ◽  
Charles J Golden
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Cognitive Impairment ◽  
Literature Review ◽  
Cognitive Impairments ◽  
Empirical Literature ◽  
Sporadic Als ◽  
Neuropsychological Assessments ◽  
Familial Als ◽  
Als Patients ◽  
Lateral Sclerosis

Abstract Objective Amyotrophic Lateral Sclerosis (ALS) is a progressive neurodegenerative disease caused by degeneration of the upper and lower motor neurons. This literature review examines the recurring etiology of cognitive impairments in ALS through empirical literature. The current study explores ALS across different subtypes and potential cognitive impairments. Two classifications are primarily examined ALS, and ALS with frontotemporal dementia (ALS-FTD). Involving three categories: familial inheritance pattern, genetic mutation, or sporadic. Neuropsychological studies affirm cognitive impairments in individuals diagnosed with ALS and ALS-FTD. Data Selection Data was culled from the American Psychological Association (PsycInfo), PubMed, Google Scholar. Terms used in this literature review include cognitive impairment in ALS and ALS-FTD, executive function deficiencies in ALS, neuropsychology in ALS, neuropsychological deficits in ALS, neuropsychological assessments for ALS, cognitive impairments in familial ALS, genetic ALS, and sporadic ALS, familial ALS, sporadic ALS, genetic mutations involved in ALS. Search dates December 20–23 of 2020 and March 3–4 of 2021. A total of 40 studies were examined. Data Synthesis ALS-patients demonstrate a significant cognitive impairment. However, influencing comorbidities accompanying the disease may be contributing to these impairments. Researchers employed neuroimaging and neuropsychological batteries to further understand influencing factors involved in ALS and cognition. Conclusions Researchers now understand ALS as a multi-symptomatic disorder and acknowledge the presence of cognitive impairments at various encased levels. There are limitations in neuropsychological batteries that accommodate for executive dysfunctions observed in ALS patients. Future studies should explore neuropsychological assessments that accommodate for motor deficits and dysarthria when assessing cognitive impairment in ALS patients.

Amyotrophic lateral sclerosis diagnostic index

Neurology ◽  
2019 ◽  
Vol 92 (6) ◽  
pp. e536-e547 ◽  
Author(s):  
Nimeshan Geevasinga ◽  
James Howells ◽  
Parvathi Menon ◽  
Mehdi van den Bos ◽  
Kazumoto Shibuya ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Confidence Interval ◽  
Diagnostic Accuracy ◽  
Neuromuscular Disorders ◽  
Area Under The Curve ◽  
Disease Process ◽  
Diagnostic Utility ◽  
Training Cohort ◽  
Diagnostic Index ◽  
Lateral Sclerosis

ObjectiveThe aim of the study was to assess the utility of a novel amyotrophic lateral sclerosis (ALS) diagnostic index (ALSDI).MethodsA prospective multicenter study was undertaken on patients presenting with suspected ALS. The reference standard (Awaji criteria) was applied to all patients at recruitment. Patients were randomly assigned to a training (75%) and a test (25%) cohort. The ALSDI was developed in the training cohort and its diagnostic utility was subsequently assessed in the test cohort.ResultsA total of 407 patients were recruited, with 305 patients subsequently diagnosed with ALS and 102 with a non-ALS mimicking disorder. The ALSDI reliably differentiated ALS from neuromuscular disorders in the training cohort (area under the curve 0.92, 95% confidence interval 0.89–0.95), with ALSDI ≥4 exhibiting 81.6% sensitivity, 89.6% specificity, and 83.5% diagnostic accuracy. The ALSDI diagnostic utility was confirmed in the test cohort (area under the curve 0.90, 95% confidence interval 0.84–0.97), with ALSDI ≥4 exhibiting 83.3% sensitivity, 84% specificity, and 83.5% diagnostic accuracy. In addition, the diagnostic utility of the ALSDI was confirmed in patients who were Awaji negative at recruitment and in those exhibiting a predominantly lower motor neuron phenotype.ConclusionThe ALSDI reliably differentiates ALS from mimicking disorders at an early stage in the disease process.Classification of evidenceThis study provides Class I evidence that for patients with suspected ALS, the ALSDI distinguished ALS from neuromuscular mimicking disorders.

The interplay among education, brain metabolism, and cognitive impairment suggests a role of cognitive reserve in Amyotrophic Lateral Sclerosis

2021 ◽  
Vol 98 ◽  
pp. 205-213
Author(s):  
Antonio Canosa ◽  
Francesca Palumbo ◽  
Barbara Iazzolino ◽  
Laura Peotta ◽  
Francesca Di Pede ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Cognitive Impairment ◽  
Cognitive Reserve ◽  
Brain Metabolism ◽  
Lateral Sclerosis

scholarly journals Non-invasive measurement of fasciculation frequency demonstrates diagnostic accuracy in amyotrophic lateral sclerosis

2020 ◽  
Vol 2 (2) ◽  
Author(s):  
Arina Tamborska ◽  
James Bashford ◽  
Aidan Wickham ◽  
Raquel Iniesta ◽  
Urooba Masood ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Confidence Interval ◽  
Biceps Brachii ◽  
Delayed Diagnosis ◽  
Area Under The Curve ◽  
Motor Neuropathy ◽  
Healthy Individuals ◽  
Non Invasive ◽  
Lateral Sclerosis ◽  
Invasive Measurement

Abstract Delayed diagnosis of amyotrophic lateral sclerosis prevents early entry into clinical trials at a time when neuroprotective therapies would be most effective. Fasciculations are an early hallmark of amyotrophic lateral sclerosis, preceding muscle weakness and atrophy. To assess the potential diagnostic utility of fasciculations measured by high-density surface electromyography, we carried out 30-min biceps brachii recordings in 39 patients with amyotrophic lateral sclerosis, 7 patients with benign fasciculation syndrome, 1 patient with multifocal motor neuropathy and 17 healthy individuals. We employed the surface potential quantification engine to compute fasciculation frequency, fasciculation amplitude and inter-fasciculation interval. Inter-group comparison was assessed by Welch’s analysis of variance. Logistic regression, receiver operating characteristic curves and decision trees discerned the diagnostic performance of these measures. Fasciculation frequency, median fasciculation amplitude and proportion of inter-fasciculation intervals <100 ms showed significant differences between the groups. In the best-fit regression model, increasing fasciculation frequency and median fasciculation amplitude were independently associated with the diagnosis of amyotrophic lateral sclerosis. Fasciculation frequency was the single best measure predictive of the disease, with an area under the curve of 0.89 (95% confidence interval 0.81–0.98). The cut-off of more than 14 fasciculation potentials per minute achieved 80% sensitivity (95% confidence interval 63–90%) and 96% specificity (95% confidence interval 78–100%). In conclusion, non-invasive measurement of fasciculation frequency at a single time-point reliably distinguished amyotrophic lateral sclerosis from its mimicking conditions and healthy individuals, warranting further research into its diagnostic applications.

Inside minds, beneath diseases: social cognition in amyotrophic lateral sclerosis-frontotemporal spectrum disorder

2020 ◽  
Vol 91 (12) ◽  
pp. 1279-1282
Author(s):  
Patricia Lillo ◽  
Paulo Caramelli ◽  
Gada Musa ◽  
Teresa Parrao ◽  
Ricardo Hughes ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Cognitive Impairment ◽  
Social Cognition ◽  
Digit Span ◽  
Short Version ◽  
The Social ◽  
Significant Difference ◽  
Similar Disease ◽  
Post Hoc ◽  
Lateral Sclerosis

ObjectiveTo compare social cognition performance between patients with amyotrophic lateral sclerosis (ALS) and those patients with behavioural variant frontotemporal dementia (bvFTD).MethodsWe 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 faux pas 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).ResultsNo 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 faux pas (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.DiscussionOur findings support a cognitive continuum between ALS and bvFTD and shed light on the cognitive heterogeneity of ALS, expanding its possible neuropsychological profiles.

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