Accuracy of clinical and electrophysiological criteria for diagnosing amyotrophic lateral sclerosis

Except for the genetic forms, amyotrophic lateral sclerosis (ALS) is not a single disease but a syndrome. ALS is clinically characterised by the presence of progressive upper motor neuron (UMN) signs and lower motor neurone (LMN) signs in bulbar, upper limb, axial, and lower limb muscles [Riva et al. 2016]. Depending on the affected region at onset, bulbar-onset and limb-onset ALS is delineated [Morris, 2015]. At onset, affection of the limbs may be asymmetric. No specific test is available for diagnosing ALS and the golden standard is the neuropathological demonstration of the UMN and LMN lesion.

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