A comparative study of cognitive and behavioral profiles between sporadic and type 8 amyotrophic lateral sclerosis.

Autor: de Alcântara C; Programa de Pós-graduação em Neurociências, Universidade Federal de Minas Gerais, Belo Horizonte, Brazil., Cruzeiro MM; Departamento de Clínica Médica, Universidade Federal de Juiz de Fora, Juiz de Fora, Brazil., França MC Jr; Departamento de Neurologia, Universidade Estadual de Campinas, Campinas, Brazil., Alencar MA; Departamento de Fisioterapia, Universidade Federal de Minas Gerais, Belo Horizonte, Brazil., Jaeger A; Programa de Pós-graduação em Neurociências, Universidade Federal de Minas Gerais, Belo Horizonte, Brazil.; Departamento de Psicologia, Faculdade de Filosofia e de Ciências Humanas, Universidade Federal de Minas Gerais, Belo Horizonte, Brazil., de Araújo CM; Programa de Pós-graduação em Neurociências, Universidade Federal de Minas Gerais, Belo Horizonte, Brazil., da Gama NAS; Programa de Pós-graduação em Neurociências, Universidade Federal de Minas Gerais, Belo Horizonte, Brazil., Camargos ST; Programa de Pós-graduação em Neurociências, Universidade Federal de Minas Gerais, Belo Horizonte, Brazil.; Departamento de Clínica Médica, Faculdade de Medicina, Universidade Federal de Minas Gerais, Belo Horizonte, Brazil., de Souza LC; Programa de Pós-graduação em Neurociências, Universidade Federal de Minas Gerais, Belo Horizonte, Brazil.; Departamento de Clínica Médica, Faculdade de Medicina, Universidade Federal de Minas Gerais, Belo Horizonte, Brazil.
Jazyk: angličtina
Zdroj: Muscle & nerve [Muscle Nerve] 2023 Sep; Vol. 68 (3), pp. 316-322. Date of Electronic Publication: 2023 Jul 10.
DOI: 10.1002/mus.27927
Abstrakt: Introduction/aims: Amyotrophic lateral sclerosis (ALS) type 8 (ALS8) is caused by VAPB gene mutations. The differences between neuropsychological and behavioral profiles of patients with sporadic ALS (sALS) and those with ALS8 are unclear. We aimed to compare cognitive performance and behavioral aspects between sALS and ALS8 patients.
Methods: Our study included 29 symptomatic ALS8 patients (17 men; median age 49 years), 20 sALS patients (12 men; median age 55 years), and 30 healthy controls (16 men; median age 50 years), matched for sex, age, and education. Participants underwent neuropsychological assessments focused on executive functions, visual memory, and facial emotion recognition. Behavioral and psychiatric symptoms were evaluated using the Hospital Anxiety and Depression Scale and the Cambridge Behavioral Inventory.
Results: Clinical groups (sALS and ALS8) exhibited lower global cognitive efficiency and impaired cognitive flexibility, processing speed, and inhibitory control compared with controls. ALS8 and sALS showed similar performance in most executive tests, except for poorer verbal (lexical) fluency in those with sALS. Apathy, anxiety, and stereotypical behaviors were frequent in both clinical groups.
Discussion: sALS and ALS8 patients demonstrated similar deficits in most cognitive domains and had comparable behavioral profiles. These findings should be considered in the care of patients.
(© 2023 Wiley Periodicals LLC.)
Databáze: MEDLINE