A comparative study of cognitive and behavioral profiles between sporadic and type 8 amyotrophic lateral sclerosis.
Muscle Nerve
; 68(3): 316-322, 2023 09.
Article
en En
| MEDLINE
| ID: mdl-37424512
ABSTRACT
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.Palabras clave
Texto completo:
1
Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Apatía
/
Esclerosis Amiotrófica Lateral
Tipo de estudio:
Diagnostic_studies
Límite:
Humans
/
Male
/
Middle aged
Idioma:
En
Revista:
Muscle Nerve
Año:
2023
Tipo del documento:
Article
País de afiliación:
Brasil