Article

ALS is now understood to be a complex multisystem neurodegenerative disease
because areas other than the motor cortices of the brain undergo degeneration.
Frontotemporal dementia (FTD) may be associated with motor neuron disease, and
the transactive response DNA-binding protein 43 (TDP-43) is a major pathological
substrate underlying both diseases. The recent discovery of a gene that can cause
both FTD, ALS and FTD-ALS, C9ORF72, has modified the way for considering these
two pathologies. These findings would allow the development of potential
biomarkers and therapeutic targets for these devastating diseases. This review
summarizes the key points leading up to our current understanding of the genetic,
clinical and neuropathological overlap between FTD and ALS.
CI - Copyright (c) 2017 Elsevier Masson SAS. All rights reserved.

Langue : ANGLAIS