NEUROLOGY OF AGING (KS MARDER, SECTION EDITOR) Amyotrophic Lateral Sclerosis: An Aging-Related Disease Giancarlo Logroscino 1,2 & Rosanna Tortelli 2 & Giovanni Rizzo 2 & Benoît Marin 3,4 & Pierre Marie Preux 3,4 & Andrea Malaspina 5 Published online: 15 April 2015 # Springer Science+Business Media New York 2015 Abstract Amyotrophic lateral sclerosis (ALS) is a rapidly progressive disorder characterized primarily by selective neu- rodegeneration of the motor neurons. The basis of diagnosis is the presence of signs of both upper and lower motor neuron involvement in more than one body region. ALS was consid- ered until a few years ago a disease of young adults but re- cently population-based studies have consistently shown that ALS is an age-related disease with age incidence curve similar to other age-dependent neurodegenerative diseases like Parkinson’ s disease and Alzheimer’ s disease. The peak of in- cidence is in the eighth decade and declines thereafter, first in men and then in women. In the majority of cases, ALS is sporadic while 5–10 % of the disease is familiar. After the identification of C9ORF72, it is now recognized that the def- inition of familial ALS can be enlarged to include family members with frontotemporal dementia considering the tight link between the two nosological entities. Keywords Amyotrophic lateral sclerosis . Motor neuron disease . Aging . Neurodegenerative disorder . Diagnosis . Staging . Prognosis . Biomarkers . Nutrition . Respiratory insufficiency . Epidemiology . Incidence . Sex ratio . Population-basedstudies . Genetics . Superoxide dismutase 1 . Frontotemporal dementia . Phenotype . Ethnicity . Therapy . Randomized controlled trial Introduction Amyotrophic lateral sclerosis (ALS) is a rapidly progressive disorder characterized primarily by selective neurodegenera- tion of motor neurons. Clinical features important for diagno- sis and prognosis have been defined largely in tertiary centers [1]. ALS leads to death in 15 to 20 months after diagnosis in 50 % of patients [2••], generally due to respiratory failure. An This article is part of the Topical Collection on Neurology of Aging * Giancarlo Logroscino giancarlo.logroscino@uniba.it Rosanna Tortelli rosannatortelli79@gmail.com Giovanni Rizzo g.rizzo@unibo.it Benoît Marin benoit.marin@unilim.fr Pierre Marie Preux Maipierre-marie.preux@unilim.fr Andrea Malaspina a.malaspina@bartshealth.nhs.uk 1 Department of Basic Medical Sciences, Neuroscience and Sense Organs, University of Bari BAldo Moro^, Bari, Italy 2 Unit of Neurodegenerative Diseases, Department of Clinical Research in Neurology, University of Bari BAldo Moro^, Pia Fondazione Cardinale G. Panico, 73039 Tricase, Lecce, Italy 3 Univ. Limoges, UMR_S 1094, Tropical Neuroepidemiology, Institute of Neuroepidemiology and Tropical Neurology, CNRS FR 3503 GEIST, F-87000 Limoges, France 4 INSERM, U1094, Tropical Neuroepidemiology, Limoges, France 5 Blizard Institute, Institute of Cell and Molecular Science, Neuroscience and Trauma Centre, Barts Health, 2 Newark Street, London E1 2AT, UK Curr Geri Rep (2015) 4:142–153 DOI 10.1007/s13670-015-0127-8