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      Multidimensional apathy in ALS: validation of the Dimensional Apathy Scale

      Author(s): , , , , ,
      Publication date Created:
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      Journal: Journal of Neurology, Neurosurgery & Psychiatry
      Publisher: BMJ

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          Abstract

          Apathy is a prominent symptom of amyotrophic lateral sclerosis (ALS), but measurement is confounded by physical disability. Furthermore, it has been traditionally measured as a unidimensional symptom despite research demonstrating a multifaceted construct. The new Dimensional Apathy Scale (DAS) has been specifically designed for patients with motor disability to measure 3 neurologically based subtypes of apathy: Executive, Emotional and Initiation. We aimed to explore this behavioural symptom by examining the substructure of apathy in ALS and to determine the reliability and validity of the DAS in patients and their carers.

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          Changes in cognition and behaviour in amyotrophic lateral sclerosis: nature of impairment and implications for assessment.

          Laura Goldstein, Sharon Abrahams (2013)
          Increased awareness of cognitive and behavioural change in amyotrophic lateral sclerosis has been driven by various clinic-based and population-based studies. A frontotemporal syndrome occurs in a substantial proportion of patients, a subgroup of whom present with frontotemporal dementia. Deficits are characterised by executive and working-memory impairments, extending to changes in language and social cognition. Behaviour and social cognition abnormalities are closely similar to those reported in behavioural variant frontotemporal dementia, implying a clinical spectrum linking amyotrophic lateral sclerosis and frontotemporal dementia. Cognitive impairment should be considered in clinical management, but few specialist assessment resources are available, and thus the cognitive status of most patients is unknown. Standard assessment procedures are not appropriate to detect dysfunction due to progressive physical disability; techniques that better measure the problems encountered by this group of patients are needed to further establish disease effects. Screening instruments are needed that are validated specifically for amyotrophic lateral sclerosis, encompass the heterogeneity of impairment, and accommodate physical disability. Copyright © 2013 Elsevier Ltd. All rights reserved.
          Article 0 comments Cited 151 times – based on 0 reviews     Review now
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            Is there a dysexecutive syndrome?

            Donald T Stuss, Roger Alexander (2007)
            The role of the frontal lobes has often been described as a 'paradox' or a 'riddle'. Ascribed to this region has been the loftiest of functions (e.g. executive; seat of wisdom); others contested that the frontal lobes played no special role. There has also been controversy about the unity or diversity of functions related to the frontal lobes. Based on the analysis of the effects of lesions of the frontal lobes, we propose that there are discrete categories of functions within the frontal lobes, of which 'executive' functioning is one. Within the executive category, the data do not support the concept of an undifferentiated central executive/supervisory system. The results are better explained as impairments in a collection of anatomically and functionally independent but interrelated attentional control processes. Evidence for three separate frontal attentional processes is presented. For each process, we present an operational description, the data supporting the distinctiveness of each process and the evidence for impairments of each process after lesions in specific frontal regions. These processes and their coarse frontal localizations are energization-superior medial, task setting-left lateral and monitoring-right lateral. The strength of the findings lies in replication: across different tasks; across different cognitive modalities (e.g. reaction time paradigms, memory); and across different patient groups. This convergence minimizes the possibility that any of the findings are limited to a specific task or to a specific set of patients. Although distinct, these processes are flexibly assembled in response to context, complexity and intention over real time into different networks within the frontal regions and between frontal and posterior regions.
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              Cognitive changes predict functional decline in ALS: a population-based longitudinal study.

              Marwa Elamin, Catherine Lynch, Niall Pender … (2013)
              To determine whether cognitive status in patients with amyotrophic lateral sclerosis (ALS) is a useful predictor of attrition and motor and cognitive decline. Cognitive testing was undertaken in a large population-based cohort of incident ALS patients using a longitudinal, case-control study design. Normative data for neuropsychological tests were generated using age-, sex-, and education-matched healthy controls who also underwent repeated assessments. Data were analyzed to generate models for progression/spread. One hundred eighty-six patients with ALS who had no evidence of C9orf72 hexanucleotide repeat expansion were enrolled. A second and third assessment were undertaken in 98 and 46 of the patients with ALS, respectively. Executive impairment at the initial visit was associated with significantly higher rates of attrition due to disability or death and faster rates of motor functional decline, particularly decline in bulbar function. Decline in cognitive function was faster in patients who were cognitively impaired at baseline. Normal cognition at baseline was associated with tendency to remain cognitively intact, and with slower motor and cognitive progression. Non-C9orf72-associated ALS is characterized by nonoverlapping cognitive subgroups with different disease trajectories. These findings have important implications for models of ALS pathogenesis, and for future clinical trial design.
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                Author and article information

                Journal
                Title: Journal of Neurology, Neurosurgery & Psychiatry
                Abbreviated Title: J Neurol Neurosurg Psychiatry
                Publisher: BMJ
                ISSN (Print): 0022-3050
                ISSN (Electronic): 1468-330X
                Publication date Created: May 13 2016
                Publication date Created: June 2016
                Publication date (Print): June 2016
                Publication date (Electronic): July 22 2015
                Volume: 87
                Issue: 6
                Pages: 663-669
                Article
                DOI: 10.1136/jnnp-2015-310772
                PubMed ID: 26203157
                SO-VID: d6473ef6-dbbb-4272-9160-643162ce190b
                Copyright © © 2015
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