The cognitive profile of ALS: a systematic review and meta-analysis update

  title={The cognitive profile of ALS: a systematic review and meta-analysis update},
  author={Emma Beeldman and Joost Raaphorst and Michelle Klein Twennaar and Marianne de Visser and Ben Schmand and Rob J. de Haan},
  journal={Journal of Neurology, Neurosurgery \& Psychiatry},
  pages={611 - 619}
Cognitive impairment is present in approximately 30% of patients with amyotrophic lateral sclerosis (ALS) and, especially when severe, has a negative impact on survival and caregiver burden. Our 2010 meta-analysis of the cognitive profile of ALS showed impairment of fluency, executive function, language and memory. However, the limited number of studies resulted in large confidence intervals. To obtain a more valid assessment, we updated the meta-analysis and included methodological… 

The cognitive profile of Friedreich ataxia: a systematic review and meta-analysis

Background Study the cognitive profile of individuals with Friedreich ataxia (FRDA) and seek evidence for correlations between clinical, genetic and imaging characteristics and neuropsychological

The cognitive profile of behavioural variant FTD and its similarities with ALS: a systematic review and meta-analysis

The Cognitive profile of bv-FTD consists of deficits in social cognition, verbal memory, fluency and executive functions and shows similarities with the cognitive profile of ALS, providing evidence for the existence of a cognitive disease continuum encompassing amyotrophic lateral sclerosis and bv -FTD.

An overview of screening instruments for cognition and behavior in patients with ALS: selecting the appropriate tool for clinical practice

The BBI appears to be the best option to screen for behavioral changes in ALS, since all relevant domains are assessed, motor-related problems are considered, and has a satisfactory validity.

Cognition in the course of ALS-a meta-analysis.

Objective: The goal of this meta-analysis is to improve insight into the development of cognition over the course of ALS and to assess predictors of cognitive performance.Method: A literature search

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The Edinburgh Cognitive and Behavioural ALS Screen (ECAS) had an excellent AUC and high sensitivity and specificity, indicating that it is a valid screening instrument for cognitive impairment in ALS.

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The screening test, ECAS and the social cognition test, RME, may have some advantages over other tests that have been used for assessing cognitive change in ALS patients, and recommendations of ALS-specific tests with sound psychometric properties are urgently needed.

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Cognitive and behavioral impairment is observed in a substantial number of patients with ALS and mostly presented with moderate executive and social cognitive dysfunction, as well as apathy and less often disinhibition.



The cognitive profile of amyotrophic lateral sclerosis: A meta-analysis

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Prevalence and patterns of cognitive impairment in sporadic ALS

These data confirm the presence of cognitive impairment in 50% of patients with ALS and particularly implicate executive dysfunction and mild memory decline in the disease process and have features consistent with FTD.

An observational study of cognitive impairment in amyotrophic lateral sclerosis.

Demented patients with ALS showed predominant impairment in free recall, executive function, and naming, with relative preservation of attention, psychomotor speed, and visuospatial function, as well as increasing ALS severity and declining verbal fluency performance.

A prospective cohort study of neuropsychological test performance in ALS

  • P. GordonR. Goetz H. Mitsumoto
  • Psychology, Medicine
    Amyotrophic lateral sclerosis : official publication of the World Federation of Neurology Research Group on Motor Neuron Diseases
  • 2010
The strongest neuropsychological predictors of cognitive status were measures of executive, episodic memory and language function, and older age and more severe ALS were associated with impairment.

The effects of executive and behavioral dysfunction on the course of ALS

Survival is significantly shorter among patients with ALS-FTLD than with classic ALS, and patients with FTLD are twice as likely to be noncompliant.

[Cognitive impairment in amyotrophic lateral sclerosis].

A comparison of estimates by different scores of emotional state and quality of life (the Hamilton depression scale, CNSLS, ALSAQ40) allows to conclude that CC had no influence on the emotional state of ALS patients and their totalquality of life.

Cognition, Behavior, and Respiratory Function in Amyotrophic Lateral Sclerosis

Results suggest that the respiratory insufficiency of ALS patients may potentially produce irreversible deficits in executive functioning; yet once treated, impairments in more basic cognitive abilities may be less evident.

Subcortical structures in amyotrophic lateral sclerosis

Consensus criteria for the diagnosis of frontotemporal cognitive and behavioural syndromes in amyotrophic lateral sclerosis

  • M. StrongG. Grace D. Figlewicz
  • Psychology, Medicine
    Amyotrophic lateral sclerosis : official publication of the World Federation of Neurology Research Group on Motor Neuron Diseases
  • 2009
Recommendations arising from an international research workshop on frontotemporal dementia (FTD) and ALS address the requirement for a concise clinical diagnosis of the underlying motor neuron disease, defining the cognitive and behavioural dysfunction, and identifying the presence of disease modifiers.