Classification and clinical features of motor neurone diseases and motor neuropathies in adults

The term motor neurone disease encompasses combined upper and lower motor n eurone disorders (amyotrophic lateral sclerosis), pure lower motor neurone disorders (spinal muscular atrophies, multifocal motor neuropathies, post i rradiation lumbosacral radiculopathy, postpolio syndrome, hereditary bulbar palsy) and pure upper motor neurone disorders (primary lateral sclerosis, hereditary spastic paraplegia, neuro-lathyrism, Konzo). The chief clinical and electrophysiological criteria for these different disorders are discuss ed, with particular attention to diagnostically distinctive characteristics of each. Age of onset, and inheritance are considered as additional diagno stic features.