Extra-motor abnormalities in amyotrophic lateral sclerosis: another layer of heterogeneity.
Expert Rev Neurother
; 17(6): 561-577, 2017 06.
Article
en En
| MEDLINE
| ID: mdl-27983884
INTRODUCTION: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease defined by the presence of muscle weakness. The motor features of disease are heterogeneous in site of onset and progression. There are also extra-motor features in some patients. The genetic basis for extra-motor features is uncertain. The heterogeneity of ALS is an issue for clinical trials. Areas covered: This paper reviews the range and prevalence of extra-motor features associated with ALS, and highlights the current information about genetic associations with extra-motor features. Expert commentary: There are extra-motor features of ALS, but these are not found in all patients. The most common is cognitive abnormality. More data is required to ascertain whether extra-motor features arise with progression of disease. Extra-motor features are reported in patients with a range of causative genetic mutations, but are not found in all patients with these mutations. Further studies are required of the heterogeneity of ALS, and genotype/phenotype correlations are required, taking note of extra-motor features.
Palabras clave
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Esclerosis Amiotrófica Lateral
/
Mutación
Tipo de estudio:
Risk_factors_studies
Límite:
Humans
Idioma:
En
Revista:
Expert Rev Neurother
Asunto de la revista:
NEUROLOGIA
/
TERAPEUTICA
Año:
2017
Tipo del documento:
Article
País de afiliación:
Australia
Pais de publicación:
Reino Unido