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The diagnosis as well as the anatomy and physiology of the condition amyotrophic lateral sclerosis is one of the most completely understood conditions in the realm of clinical neurology. —Jean-Martin Charcot (1825–1893)
In an hubristic statement, Charcot was correctly observing that the dominant phenotype of the clinical syndrome known in the UK as motor neurone disease (MND), is among the most recognisable to the neurologist and often unmistakeable in written descriptions published even before his own.1 Neurologists can probably agree on a core statement that MND is a progressive syndrome involving motor neuronal cell death, but there is undoubtedly a challenging clinical heterogeneity coupled with recognition that it overlaps clinically, histopathologically and genetically with frontotemporal dementia. The typically drastically life-shortening nature of MND encourages the pursuit of so-called mimics, long lists of which pervade the literature.2 The concern is that this contributes to the average diagnostic latency from symptom onset of 1 year, occasionally resulting in unnecessary spinal surgery, and …
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