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

AI Article Synopsis

  • The study aimed to explore how cognitive and behavioral deficits develop in patients with ALS and bvFTD to better understand their differences.
  • Researchers analyzed profiles of 762 ALS and bvFTD patients, comparing them to 49 healthy controls using a cognitive screening tool.
  • Results showed that ALS patients experience different cognitive changes than those with bvFTD, suggesting distinct developmental patterns that might be influenced by genetic factors.

Article Abstract

Objective: 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.

Methods: 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.

Results: 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.

Conclusion: 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.

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Source
http://dx.doi.org/10.1136/jnnp-2018-318800DOI Listing

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