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Over the past decade, evidence has emerged of unique pathophysiological processes, including glutamate\u2010mediated excitotoxicity, which has resulted in the development of novel diagnostic investigations and uncovered potential therapeutic targets. Advances in genetics, including the recently discovered <jats:styled-content style=\"fixed-case\"><jats:italic>C9orf72<\/jats:italic><\/jats:styled-content> gene, have radically changed the pathological mindset, from <jats:styled-content style=\"fixed-case\">MND<\/jats:styled-content> being classified as a neuromuscular disease to one that <jats:styled-content style=\"fixed-case\">MND<\/jats:styled-content> forms a continuum with other primary neurodegenerative disorders, including frontotemporal dementia. 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