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There has been a fundamental revolution of our understanding of ALS in recent years. It is a multisystem neuromuscular disease that has a neurodegenerative basis which, along with the frontotemporal dementias, represents a spectrum of conditions. Cognitive and behavioural disorders are now an integral part of the diagnostic criteria, and initial diagnostic biomarkers have been established. This has significant implications not only for the diagnosis, treatment and care of patients, but also for our understanding of the pathophysiology of the disease. Rapidly advancing technical developments, such as eye control&based communication, are creating novel opportunities for therapists, patients and their relatives, which are increasingly influencing borderline decisions for and against life-prolonging measures. This book takes account of these new developments and develops novel concepts ranging from basic science to the classification and diagnosis of the disease to the opportunities and limitations of current and future treatment options and approaches to care.