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In amyotrophic lateral sclerosis, the deadliest form of motor neuron diseases, physicians observe the spread of non-painful paralysis with intact sensory function often presenting as asymmetric muscle atrophy and brisk or maintained reflexes. Using Awaji criteria to estimate upper and lower motor neuron signs in bulbar, cervical, thoracic and lumbar regions, and the highly sensitive electromyography (EMG), they diagnose amyotrophic lateral sclerosis (ALS) in a majority of the cases. The spread of symptoms is attributed to spinal motor neuron pathology, but experienced ALS neurologists will agree that a slowing of movement, loss of fine motor control and occurrence of mass or mirror …
Funding European Union Joint Programming for Neurodegenerative Diseases (JPND) grant SOPHIA.
Competing interests None.
Provenance and peer review Commissioned; externally peer reviewed.
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