Neuropsychological Sub‐Phenotypes in Amyotrophic Lateral Sclerosis

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Abstract
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.
Funding Information
  • Fondazione Regionale per la Ricerca Biomedica (2015‐0023)
  • Horizon 2020 Framework Programme (101017598)

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