Assessing cognitive functioning in ALS: A focus on frontal lobe processes

Gillingham, Susan; Yunusova, Yana; Ganda, Anoop; Rogaeva, Ekaterina; Black, Sandra E.; Stuss, Donald T.; Zinman, Lorne

doi:10.1080/21678421.2016.1248977

Cited by 22 publications

(21 citation statements)

References 45 publications

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“…While there is recent research indicating emotional empathy impairments in ALS (Cerami et al, 2014), this seems to be the first report of levels of Emotional apathy being associated with emotional recognition deficits. Similar such emotional processing deficits have been observed (Gillingham et al, 2017) which were proposed to be due to dysfunction of behavioral/emotional self-regulation (Stuss & Alexander, 2007, Stuss, 2011 This was also consistent with previous research showing ALS patients impairment in these domains of cognitive functioning (Zimmerman et al, 2007, Girardi et al, 2011, Cuddy et al, 2012. Previous research has shown that impairment in social cognition was associated with apathy as a behavioural change (Girardi et al, 2011).…”

Section: Discussionsupporting

confidence: 87%

“…In relation to current cognitive models of executive functions, Stuss and colleagues propose that the key frontal lobe functions are Energization, which relates to initiating and sustaining response, Monitoring and task setting which monitor and adjust performance, Behavioral/emotional self-regulation which integrates emotional and social aspects of behaviour and Metacognition/Integration relating to higher order, coordinative processing between the other functions of the frontal lobe. Specifically, Energization deficits are often observed as decreased output during verbal fluency tasks (Stuss & Alexander, 2007, Stuss, 2011, with recent research showing selective Energization deficits in ALS (Gillingham et al, 2017). The findings of our study suggest this may be a possible underlying process common to both Initiation apathy and verbal fluency.…”

Section: Discussionsupporting

confidence: 70%

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Multidimensional apathy and executive dysfunction in amyotrophic lateral sclerosis

Radaković

Stephenson

Newton

et al. 2017

Cortex

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Apathy and cognitive dysfunction are prominent symptoms of Amyotrophic lateral sclerosis (ALS). More specifically ALS patients show increased Initiation apathy-a lack of motivation for self-generation of thoughts as assessed by the Dimensional Apathy Scale. This study aimed to investigate the cognitive underpinnings of apathy subtypes in ALS. We hypothesized that increased Initiation apathy would be associated deficits on tests of intrinsic response generation, such as verbal fluency. We also explored the relationship of other apathy subtypes to cognitive processes, in particular emotional apathy with emotional and social cognition deficits and executive apathy with planning and goal management deficits. ALS patients, and their carers (N = 30), and healthy matched controls, and their informants (N = 29) were recruited. All participants completed self- and informant/carer-rated Dimensional Apathy Scale, to quantify apathy subtypes (Executive, Emotional and Initiation), along with standard apathy and depression measures. Patients and controls completed the Edinburgh Cognitive and behavioural ALS Screen, and a comprehensive neuropsychological battery including emotional recognition, social cognition, intrinsic response generation tasks (verbal fluency and random number generation) and a new ecologically valid, computerised measure of planning and goal management. The results demonstrated that increased Initiation apathy was the only significantly elevated subtype in ALS (self-rated p < .05, informant/carer-rated p < .01). Initiation apathy was found to be significantly associated with verbal fluency deficit, while Emotional apathy was significantly associated with emotional recognition deficits. No associations were found between apathy subtypes and depression or in controls. This is the first study to show specific associations between apathy subtypes (Emotional and Initiation) and executive and emotional cognitive dysfunction, indicating possible distinct underlying mechanisms to these demotivational symptoms.

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Section: Discussionsupporting

confidence: 87%

Section: Discussionsupporting

confidence: 70%

Multidimensional apathy and executive dysfunction in amyotrophic lateral sclerosis

Radaković

Stephenson

Newton

et al. 2017

Cortex

View full text Add to dashboard Cite

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“…[42,43], thought to reflect an impairment in initiation and sustainment of response. ALS patients show difficulties with performance on motor independent reaction time tasks[49], but most notably verbal fluency deficits (impaired word generation). Verbal (predominantly letter) fluency are the most striking and characteristic deficit of this disease[12] and shown to be related to intrinsic response generation and independent of motor disability[50].Our most recent work in ALS has demonstrated that Initiation apathy directly correlates with verbal fluency impairments, suggestive of shared underlying mechanisms of executive dysfunction and apathy subtypes [51**].…”

mentioning

confidence: 99%

Multidimensional apathy: evidence from neurodegenerative disease

Radaković

Abrahams

2018

Current Opinion in Behavioral Sciences

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Apathy is a demotivation syndrome common in neurodegenerative diseases and is fundamentally multidimensional in nature. Different methodologies have been used to identify and quantify these dimensions, which has resulted in multifarious concepts, ranging in the number and characteristics of apathy subtypes. This has created an ambiguity over the fundamental substructure of apathy. Here we review the multidimensional concepts of apathy and demonstrate that overlapping elements exist, pointing towards commonalities in apathy subtypes. These can be subsumed under a unified Dimensional Apathy Framework: a triadic structure of Initiation, Executive and Emotional apathy. Distinct cognitive processes may underlie these domains, while selfawareness interplays with all subtypes. Evidence from neurodegenerative diseases supports this distinction with differing apathy profiles in amyotrophic lateral sclerosis, Parkinson's disease and Alzheimer's disease.

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“…Large, multi-timepoint longitudinal studies invariably suffer from considerable attrition rates, but these are rarely explicitly reported in the manuscript abstracts (10). Detailed genotyping is only available in a minority of longitudinal studies (15, 77, 79, 94). The most widely utilized rating scale in longitudinal studies is the ALSFRS-r (70, 71, 128) which provides a composite score of bulbar, limb and respiratory dysfunction, and is invariably evaluated in clinical trials (72, 105).…”

Section: Resultsmentioning

confidence: 99%

Tracking a Fast-Moving Disease: Longitudinal Markers, Monitoring, and Clinical Trial Endpoints in ALS

et al. 2019

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Amyotrophic lateral sclerosis (ALS) encompasses a heterogeneous group of phenotypes with different progression rates, varying degree of extra-motor involvement and divergent progression patterns. The natural history of ALS is increasingly evaluated by large, multi-time point longitudinal studies, many of which now incorporate presymptomatic and post-mortem assessments. These studies not only have the potential to characterize patterns of anatomical propagation, molecular mechanisms of disease spread, but also to identify pragmatic monitoring markers. Sensitive markers of progressive neurodegenerative change are indispensable for clinical trials and individualized patient care. Biofluid markers, neuroimaging indices, electrophysiological markers, rating scales, questionnaires, and other disease-specific instruments have divergent sensitivity profiles. The discussion of candidate monitoring markers in ALS has a dual academic and clinical relevance, and is particularly timely given the increasing number of pharmacological trials. The objective of this paper is to provide a comprehensive and critical review of longitudinal studies in ALS, focusing on the sensitivity profile of established and emerging monitoring markers.

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Assessing cognitive functioning in ALS: A focus on frontal lobe processes

Cited by 22 publications

References 45 publications

Multidimensional apathy and executive dysfunction in amyotrophic lateral sclerosis

Multidimensional apathy and executive dysfunction in amyotrophic lateral sclerosis

Multidimensional apathy: evidence from neurodegenerative disease

Tracking a Fast-Moving Disease: Longitudinal Markers, Monitoring, and Clinical Trial Endpoints in ALS

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