Article
Deciphering amyotrophic lateral sclerosis: what phenotype, neuropathology and genetics are telling us about pathogenesis.
Amyotrophic lateral sclerosis & frontotemporal degeneration - 1 May 2013
Ravits John, Appel Stanley, Baloh Robert H, Barohn Richard, Brooks Benjamin Rix, Elman Lauren, Floeter Mary Kay, Henderson Christopher, Lomen-Hoerth Catherine, Macklis Jeffrey D, McCluskey Leo, Mitsumoto Hiroshi, Przedborski Serge, Rothstein Jeffrey, Trojanowski John Q, van den Berg Leonard H, Ringel Steven
Abstract excerpt
Amyotrophic lateral sclerosis (ALS) is characterized phenotypically by progressive weakness and neuropathologically by loss of motor neurons. Phenotypically, there is marked heterogeneity. Typical ALS has mixed upper motor neuron (UMN) and lower motor neuron (LMN) involvement. Primary lateral sclerosis has predominant UMN involvement. Progressive muscular atrophy has predominant LMN involvement. Bulbar and limb...
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