title: Frontotemporal Dysfunction and Dementia in Amyotrophic Lateral Sclerosis.
abstract: Although amyotrophic lateral sclerosis (ALS) is classically considered a disorder exclusively affecting motor neurons, there is substantial clinical, neuroimaging, and neuropathologic evidence that more than half of patients have an associated syndrome of frontotemporal dysfunction. These syndromes range from frontotemporal dementia to behavioral or cognitive syndromes. Neuroimaging and neuropathologic findings are consistent with frontotemporal lobar degeneration that underpins alterations in network connectivity. Future clinical trials need to be stratified based on the presence or absence of frontotemporal dysfunction on the disease course of ALS.
authors.affiliation: Forbes Norris MDA/ALS Research Center, California Pacific Medical Center, 2324 Sacramento Street, Suite 111, San Francisco, CA 94115, USA.
authors.forename: Susan C
authors.initials: SC
authors.lastname: Woolley
authors.affiliation: Department of Clinical Neurological Sciences, Schulich School of Medicine & Dentistry, Western University, London, Ontario, Canada. Electronic address: Michael.Strong@Schulich.uwo.ca.
authors.forename: Michael J
authors.initials: MJ
authors.lastname: Strong
mesh_terms: D000690:Amyotrophic Lateral Sclerosis / Q000150:complications* / Q000235:genetics / Q000473:pathology*; D003072:Cognition Disorders / Q000209:etiology*; D004252:DNA Mutational Analysis; D004268:DNA-Binding Proteins / Q000235:genetics; D057180:Frontotemporal Dementia / Q000209:etiology*; D057174:Frontotemporal Lobar Degeneration / Q000209:etiology*; D006801:Humans; D007806:Language Disorders / Q000209:etiology; D008297:Male; D008875:Middle Aged; D009483:Neuropsychological Tests; D012720:Severity of Illness Index; D016875:tau Proteins / Q000235:genetics
chemical_list: D004268:DNA-Binding Proteins; D016875:tau Proteins
keywords: Cognitive impairment; Frontotemporal dementia; Frontotemporal lobar degeneration; Neural network; TDP-43; Tau; Theory of mind