Dados do Trabalho

Título

Amyotrophic Lateral Sclerosis type 8 has less cognitive deficits and distinct behavioural profile than the sporadic form: a comparative study

Resumo

Objective: To compare the cognitive performance and the behavioural profile of patients with sporadic Amyotrophic Lateral Sclerosis (sALS) and patients with ALS type 8 (ALS8), a familial form of ALS caused by mutations on VAPB gene.
Methods: We included 29 symptomatic ALS8 patients (17 men; median age = 49 years-old), 20 sALS (12 men; median age = 55 years-old) and 30 healthy controls (16 men; median age = 50 years-old), matched for sex, age, and educational level. Participants underwent a neuropsychological assessment focused on executive functions, visual memory, and facial emotion recognition. Behavioural and psychiatric symptoms were investigated with the Hospital Anxiety and Depression Scale and the Cambridge Behavioural Inventory.
Results: Clinical groups (sALS and ALS8) had lower global cognitive efficiency than controls, and also impaired performance in cognitive flexibility and inhibitory control. Verbal fluency deficits were not found in ALS8 patients, but they seem to have impairments in working memory subcomponents. On the psychiatric and behavioural aspects, apathy was shown to be frequent in both clinical groups. ALS8 patients presented more eating changes and more stereotypical behaviours.
Conclusions: Overall, sALS and ALS8 had similar deficits in most cognitive domains, but ALS8 patients exhibit milder degree of executive dysfunction than sALS, besides distinctive pattern of behavioural changes. ALS8 has a specific cognitive-behavioural profile, which should be considered in the clinical care of patients.

Palavras Chave

Amyotrophic Lateral Sclerosis, VAPB, cognition