The Spectrum of Cognitive Dysfunction in Amyotrophic Lateral Sclerosis: An Update.

ABSTRACT: Cognitive dysfunction is an important non-motor symptom in amyotrophic lateral sclerosis (ALS) that has a negative impact on survival and caregiver burden. It shows a wide spectrum ranging from subjective cognitive decline to frontotemporal dementia (FTD) and covers various cognitive domains, mainly executive/attention, language and verbal memory deficits. The frequency of cognitive impairment across the different ALS phenotypes ranges from 30% to 75%, with up to 45% fulfilling the criteria of FTD. Significant genetic, clinical, and pathological heterogeneity reflects deficits in various cognitive domains. Modern neuroimaging studies revealed frontotemporal degeneration and widespread involvement of limbic and white matter systems, with hypometabolism of the relevant areas. Morphological s

SUBMITTER: Jellinger KA 

PROVIDER: S-EPMC10572320 | biostudies-literature | 2023 Sep

REPOSITORIES: biostudies-literature