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Story of the ALS-FTD continuum retold: rather two distinct entities

<h3>Objective</h3> <p>To determine the evolution and profile of cognitive and behavioural deficits in amyotrophic lateral sclerosis (ALS) and behavioural variant frontotemporal dementia (bvFTD) to disentangle the development of FTD in ALS and vice versa.</p><h3>Methods&...

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Main Authors: Lulé, Dorothée (Author) , Aho-Özhan, Helena E. A. (Author) , Vázquez, Cynthia (Author) , Weiland, Ulrike (Author) , Weishaupt, Jochen H. (Author) , Otto, Markus (Author) , Straub, Sarah (Author) , Semler, Elisa (Author) , Uttner, Ingo (Author) , Ludolph, Albert C. (Author)
Format: Article (Journal)
Language:English
Published: 2019
In: Journal of neurology, neurosurgery, and psychiatry
Year: 2019, Volume: 90, Issue: 5, Pages: 586-589
ISSN:1468-330X
DOI:10.1136/jnnp-2018-318800
Online Access:Verlag, lizenzpflichtig, Volltext: https://doi.org/10.1136/jnnp-2018-318800
Verlag, lizenzpflichtig, Volltext: https://jnnp.bmj.com/content/90/5/586
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Author Notes:Dorothée E Lulé, Helena EA Aho-Özhan, Cynthia Vázquez, Ulrike Weiland, Jochen H Weishaupt, Markus Otto, Sarah Anderl-Straub, Elisa Semler, Ingo Uttner, Albert C Ludolph
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Summary:<h3>Objective</h3> <p>To determine the evolution and profile of cognitive and behavioural deficits in amyotrophic lateral sclerosis (ALS) and behavioural variant frontotemporal dementia (bvFTD) to disentangle the development of FTD in ALS and vice versa.</p><h3>Methods</h3> <p>In a prospective design, cognitive and behavioural profiles of 762 patients with motor predominant ALS (flail arm/leg syndrome, primary lateral sclerosis, pseudobulbar palsy, ALS) and behavioural predominant FTD (bvFTD, ALS-FTD) were determined and caregivers of patients with ALS were asked on the evolution of behavioural symptoms. Data were compared with 49 healthy controls. Cognition was measured with the Edinburgh Cognitive and Behavioral ALS Screen.</p><h3>Results</h3> <p>Evolution and features of cognitive profile of patients with motor predominant ALS were distinctly different from patients with behavioural FTD with regard to number and degree of affected cognitive domains. Also, in ALS mostly minus symptoms evolved after physical symptom onset whereas in ALS-FTD plus and minus symptoms were reported with an onset before physical degradation.</p><h3>Conclusion</h3> <p>Evolution of cognitive and behavioural profile in patients with motor predominant ALS is distinctly different from those psychocognitive findings in patients with behavioural variant dementia. This may support the hypothesis that (possibly genetic) triggers decide in the preclinical phase on either motor or psychocognitive phenotypes.</p>
Item Description:Zuerst online veröffentlicht: 26. September 2018
Gesehen am 08.07.2021
Physical Description:Online Resource
ISSN:1468-330X
DOI:10.1136/jnnp-2018-318800

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