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Título

Assessment of Executive Functions in Patients with Amyotrophic Lateral Sclerosis

Resumo

Introduction: Rare neuromuscular diseases are chronic degenerative, presenting, in some cases, a motor and/or bulbar deficit. Some studies mention a significant presence of executive dysfunctions in patients with Amyotrophic Lateral Sclerosis (ALS). The Five Digit Test (FDT) aims to measure mental processing speed and the ability to direct and switch their attentional control, demanding executive function skills such as response inhibition and cognitive flexibility.
Objectives: The objective of this study is to describe executive functions in patients with ALS with a multilingual and non-reading test like FDT. Methods: Cross-sectional and descriptive study, in which patients treated at an outpatient clinic for rare neuromuscular diseases were selected. As inclusion criteria, having rare neuromuscular disease and over 18 years of age. We excluded patients whose medical conditions made it impossible to understand or perform the tests (such as acute confusional state, aphasia, reduced level of consciousness, dementia, and sensory deficits). The FDT test was applied to assess executive functions in patients with ALS. All percentiles below the 25th are considered “changed”, given that scores below the 25th percentile indicate mild difficulties in executive functioning and processing speed, even without necessarily having clinical significance, and scores below the 5th percentile are more indicative of prominent deficits.
Results: A total of 16 patients were included in the study; 10 patients (62.5%) were male and mean (SD) age was 56 (11.04) years. More than half of patients showed changes to FDT decoding and retrieving (75% and 68.75% respectively). However, the control processes are 56.25% not changed in Response Inhibition and 62.5% not changed in cognitive flexibility.
Conclusions: Patients with ALS can manifest executive dysfunction severe enough to classify them as cognitively impaired. However, in this sample, despite the automatic processes (reading and counting) being altered, the inhibition and cognitive flexibility, which are control processes and depend on executive function, responses remained preserved. Furthermore, more studies are needed to better understand and monitor executive functions in patients with ALS, as well as discuss an assessment more adapted to the progression of the disease.

Palavras Chave

Amyotrophic Lateral Sclerosis; Executive Functions; Neuropsychology

Área

Doenças Neuromusculares