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The expanding syndrome of amyotrophic lateral sclerosis: a clinical and molecular odyssey

OPEN Journal of neurology, neurosurgery, and psychiatry | 4 Feb 2015

MR Turner and M Swash
Abstract
Recent advances in understanding amyotrophic lateral sclerosis (ALS) have delivered new questions. Disappointingly, the initial enthusiasm for transgenic mouse models of the disease has not been followed by rapid advances in therapy or prevention. Monogenic models may have inadvertently masked the true complexity of the human disease. ALS has evolved into a multisystem disorder, involving a final common pathway accessible via multiple upstream aetiological tributaries. Nonetheless, there is a common clinical core to ALS, as clear today as it was to Charcot and others. We stress the continuing relevance of clinical observations amid the increasing molecular complexity of ALS.
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Concepts
Death, Superoxide dismutase, Lou Gehrig, Stephen Hawking, Jean-Martin Charcot, Medicine, Molecular biology, Amyotrophic lateral sclerosis
MeSH headings
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