Decline of cognitive and behavioral functions in amyotrophic lateral sclerosis: a longitudinal study

Enrica Bersano, Maria Francesca Sarnelli, Valentina Solara, Barbara Iazzolino, Laura Peotta, Fabiola De Marchi, Alessio Facchin, Cristina Moglia, Antonio Canosa, Andrea Calvo, Adriano Chiò, Letizia Mazzini

Research output: Contribution to journalArticlepeer-review


Background: A cognitive impairment, ranging from frontotemporal dementia (FTD) to milder forms of dysexecutive or behavioral dysfunction, is detected in 30–50% of patients affected by amyotrophic lateral sclerosis (ALS) at diagnosis. Such condition considerably influences the prognosis, and possibly impacts on the decision-making process with regards to end-of-life choices. The aim of our study is to examine the changes of cognitive and behavioral impairment in a large population of ALS from the time of diagnosis to a 6-month follow-up (IQR 5.5–9.0 months), and to examine to what extent the progression of cognitive impairment affects survival time and rate of disease progression. Methods: We recruited 146 ALS patients classified according to revised criteria of ALS and FTD spectrum disorder. In a multidisciplinary setting, during two subsequent visits we examined clinical features with ALSFRS-r score, FVC% and BMI, and cognitive status with an extensive neuropsychological evaluation. Results: At second examination, one-third of patients showed a worsening of cognitive impairment, namely 88% of ALSbi, 27% of ALSci, 40% of ALScbi, and, interestingly, also 24% of cognitive normal ALS developed a significant cognitive dysfunction. We find that those who changed their cognitive status presented a lower ALSFRS-r score at t1 and a shorter survival time compared to those who did not change, regardless of the type of cognitive impairment. Conclusion: We show how cognitive disorders in ALS patients can not only be present at diagnosis, but also manifest during disease and influence the progression of motor deficit and the prognosis.

Original languageEnglish
Pages (from-to)373-379
Number of pages7
JournalAmyotrophic Lateral Sclerosis and Frontotemporal Degeneration
Issue number5-6
Publication statusPublished - Jul 2 2020


  • Amyotrophic Lateral Sclerosis
  • cognitive impairment
  • fronto-temporal dementia
  • longitudinal study

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology


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