Neuropsychological Sub‐Phenotypes in Amyotrophic Lateral Sclerosis
Barbara Poletti, Edoardo Nicolò Aiello, Monica Consonni, Barbara Iazzolino, Silvia Torre, Veronica Faltracco, Alessandra Telesca, Francesca Palumbo, Beatrice Curti, Giulia De Luca, Arianna Moreschi, Francesca Frisco, Eleonora Dalla Bella, Enrica Bersano, Nilo Riva, Federico Verde, Stefano Messina, Alberto Doretti, Alessio Maranzano, Claudia Morelli, Stefano Francesco Cappa, Andrea Calvo, Michael J. Strong, Vincenzo Silani, Giuseppe Lauria, Adriano Chiò, Nicola TicozziABSTRACT
Background
This study aimed at identifying neuropsychological sub‐phenotypes in amyotrophic lateral sclerosis (ALS) within the mild cognitive impairment (MCI) and mild behavioral impairment (MBI) frameworks.
Methods
We used individual task−/item‐level data from the cognitive and behavioral sections of the Edinburgh Cognitive and Behavioral ALS Screen (ECAS) from 901 non‐demented ALS to derive neuropsychological sub‐phenotypes pursuant to classical MCI and MBI frameworks and in accordance with an expanded version of Strong's criteria, which also addressed memory and visuo‐spatial measures.
Results
The prevalence of MCI and MBI was 39% and 37%, respectively in this retrospective review. The following MCI sub‐phenotypes were identified: dysexecutive MCI—single‐ and multiple‐domain (dMCI‐sd: 63%; dMCI‐md: 24%, respectively); non‐dysexecutive MCI—single‐ and multiple‐domain (ndMCI‐sd: 12%; ndMCI‐md: 1%, respectively). MBI was classified as follows: apathetic MBI—single‐ and multiple‐domain (aMBI‐sd: 40%; aMBI‐md: 20%, respectively); apathetic‐disinihibited/perseverative MBI—multiple domain (ad/pMBI‐md: 21%); disinihibited/perseverative MBI—multiple domain (d/pMBI‐md: 7%); psychotic MBI—single‐ and multiple‐domain (psyMBI‐sd: 2%; psyMBI‐md: 3%, respectively); unclassifiable MBI—multiple domain (uMBI‐md: 1%). 143 (16%) of patients exhibited mild cognitive and behavioral impairment (MCBI).
Conclusions
This study delivers a provisional, ECAS‐based classification for the neuropsychological sub‐phenotyping of non‐demented ALS patients, which, with further validation, might be useful for both research and clinical purposes.