Cognitive impairment in amyotrophic lateral sclerosis
: exploring its relationship with functional incapacity

  • Ana Rita Valente Santiago (Student)

Student thesis: Master's Thesis

Abstract

Amyotrophic Lateral Sclerosis (ALS), traditionally associated with motor dysfunction, also presents cognitive impairments in approximately half of the patients. Significant cognitive and behavioural deficits in ALS affects executive functioning and could have critical implications for prognosis and disease management. Assessing these changes in ALS patients is challenging because their motor disabilities can mask the real extent of their ability to perform daily activities. This study aimed to explore the cognitive impairment in ALS and their relationship with functional incapacity in Activities of Daily Living (ADLs). In a comparative cross-sectional analysis, twenty-two ALS patients were divided into Low Severity and High Severity groups (n=11 each) based on their ALSFRS-R (Amyotrophic Lateral Sclerosis Functional Rating Scale Revised) scores and compared with twenty-two Healthy Controls. Cognitive, behavioural, emotional, and functional performance were assessed. The results revealed significant cognitive deficits in ALS patients, particularly with High Severity disease. The Edinburgh Cognitive and Behavioural ALS Screen (ECAS), a multidomain evaluation specifically developed for ALS/Motor Neuron Disease, showed pronounced deficits including language, verbal fluency, executive function, and visuospatial abilities. The Mini Mental State Examination (MMSE) was more discriminative between levels of ALS severity, while the Frontal Assessment Battery (FAB) proved beneficial for comparisons with healthy individuals. Correlation analysis indicated a significant negative association between Executive Function and Advanced Instrumental Daily Living tasks. The results corroborate previous research and emphasize the importance of regular cognitive assessments in ALS patients, and also add value by assessing functional incapacity. Beyond the diagnostic cost, they pave the way for cognitive stimulation programs,, especially those targeting executive functions, which may delay cognitive decline and support complex daily living activities, essential for decision-making and developing coping strategies for the disease. Furthermore, the cognitive decline in a higher level of severity disease accentuates the need for early and comprehensive healthcare planning involving ALS patients and their families.
Date of Award15 Dec 2023
Original languageEnglish
Awarding Institution
  • Universidade Católica Portuguesa
SupervisorFilipa Ribeiro (Supervisor) & Simão Cruz (Co-Supervisor)

Keywords

  • Amyotrophic lateral sclerosis
  • Cognitive impairment
  • Neuropsychological assessment
  • Functional incapacity

Designation

  • Mestrado em Neuropsicologia

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