A systematic review of behavioural changes in motor neuron disease

@article{Raaphorst2012ASR,
  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},
  year={2012},
  volume={13},
  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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References

SHOWING 1-10 OF 55 REFERENCES
How common are behavioural changes in amyotrophic lateral sclerosis?
TLDR
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.
Behaviour in amyotrophic lateral sclerosis
TLDR
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
TLDR
In a case series of patients with FTD-MND, language-dominant FTD -MND was associated with bulbar-onset MND and a shorter survival, and there was evidence that the dominant FTD,MND type is related to differences in brain atrophy patterns.
The cognitive profile of amyotrophic lateral sclerosis: A meta-analysis
TLDR
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.
Survival in frontotemporal dementia
TLDR
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. Portet, C. Cadilhac, J. Touchon, W. Camu
  • Medicine
    Amyotrophic lateral sclerosis and other motor neuron disorders : official publication of the World Federation of Neurology, Research Group on Motor Neuron Diseases
  • 2001
TLDR
This study adds further arguments for a cognitive impairment in MND/ALS and reinforces the hypothesis that there is continuum between MND /ALS and fronto-temporal lobe dementia.
Neurobehavioral features in frontotemporal dementia with amyotrophic lateral sclerosis.
TLDR
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?
TLDR
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
TLDR
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.
The syndrome of cognitive impairment in amyotrophic lateral sclerosis: a population-based study
TLDR
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.
...
1
2
3
4
5
...