A systematic review of behavioural changes in motor neuron disease

  title={A systematic review of behavioural changes in motor neuron disease},
  author={Joost Raaphorst and Emma Beeldman and Marianne de Visser and Rob J. de Haan and Ben Schmand},
  journal={Amyotrophic Lateral Sclerosis},
  pages={493 - 501}
Motor neuron disease (MND) and the behavioural variant of frontotemporal dementia (bvFTD) are thought to be part of a disease spectrum. There is uncertainty about the frequency and characteristics of behavioural changes in MND, and similarly, about a relation between bvFTD and the site of onset of MND. Our aim was to perform a systematic review of the publications on behavioural changes in MND. An extensive search for articles on behavioural changes in MND patients was performed. First, cohort… 

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An up-to-date state of the art of neuropsychological alterations in ALS is sought, tests used to detect cognitive and behavioral impairment are described, and promising non-invasive biomarkers to detect pre-clinical cognitive decline are focused on.

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Results showed that reduced motivation was reported in more than 80% of the ALS cases, with almost 41% of them having moderate-severe apathy, and apathy was the most prominent feature in ALS patients.

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Behavioural changes of the type seen in FTD may be present even in a small consecutive cohort of ALS patients, as seen in a semi‐structured behavioural interview administered to carers of 16 consecutive patients attending a motor neuron disease clinic.

Predicting survival in frontotemporal dementia with motor neuron disease

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In conclusion, the diversity of cognitive problems in ALS seems greater than was previously thought, and patients may suffer from cognitive impairment in multiple domains, including memory dysfunction.

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Failure to establish survival in patients with pathologically confirmed frontotemporal dementia (FTD) and to determine whether clinical or pathologic subtype affects prognosis is a malignant disorder with limited life expectancy is established.

Cognitive impairment in motor neuron disease with bulbar onset

  • F. PortetC. CadilhacJ. TouchonW. Camu
  • Psychology, Biology
    Amyotrophic lateral sclerosis and other motor neuron disorders : official publication of the World Federation of Neurology, Research Group on Motor Neuron Diseases
  • 2001
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Neurobehavioral features in frontotemporal dementia with amyotrophic lateral sclerosis.

The occurrence of delusions in the context of behavioral FTD should lead to an early search for ALS features, and delusions are particularly common in patients who develop FTD/ALS.

Motor neurone disease, dementia and aphasia: coincidence, co-occurrence or continuum?

This review demonstrates that the association between MND and dementia was described and recognised as an entity as early as 1929, and its close relationship to Pick's disease was postulated in 1932.

Prevalence and patterns of cognitive impairment in sporadic ALS

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The syndrome of cognitive impairment in amyotrophic lateral sclerosis: a population-based study

Cognitive impairment, predominantly but not exclusively in the form executive dysfunction, is present in more than 40% of ALS patients who have no evidence of dementia, and its manifestations may be more heterogeneous than previously recognised.