title: Amyotrophic lateral sclerosis and frontotemporal dementia (ALS-FTD).
abstract: There is increasing clinical, imaging and neurophatological evidence that amyotrophic lateral sclerosis (ALS) represents a multisystem neurodegenerative disease. Neurodegeneration is not restricted to motor neurons, but also includes parts of the brain other than the motor cortex, especially the prefrontal and/or anterior temporal lobe, that contribute to the clinical syndrome. In some cases an evident dementia that resembles frontotemporal degeneration (FTD) was observed. It is now suggested that ALS and FTD are closely related conditions with overlapping clinical, pathological, radiological, and genetic characteristics. The presence of a frontal dementia in ALS has also crucial practical consequences for management of the patients, whose disorder requires critical life decisions for enteral nutrition and respiratory complications. It is our intent to provide a brief overview of the relationships between ALS and FTD.
authors.affiliation: Department of Neurological Sciences, Università degli Studi di Milano, Medical School IRCCS, Fondazione Ca' Granda Ospedale Maggiore Policlinico, IRCCS Istituto Auxologico Italiano, Milano, Italy. stefano.zago@unimi.it
authors.forename: Stefano
authors.initials: S
authors.lastname: Zago
authors.forename: Barbara
authors.initials: B
authors.lastname: Poletti
authors.forename: Claudia
authors.initials: C
authors.lastname: Morelli
authors.forename: Alberto
authors.initials: A
authors.lastname: Doretti
authors.forename: Vincenzo
authors.initials: V
authors.lastname: Silani
mesh_terms: D000690:Amyotrophic Lateral Sclerosis / Q000150:complications / Q000175:diagnosis; D001526:Behavioral Symptoms / Q000209:etiology; D003072:Cognition Disorders / Q000209:etiology; D003952:Diagnostic Imaging; D057180:Frontotemporal Dementia / Q000150:complications / Q000175:diagnosis; D006801:Humans; D009482:Neurophysiology; D009483:Neuropsychological Tests