Hemiplegic-type ALS shows a strong correlation between upper, lower motor neuron degeneration and pTDP-43 pathology

The study of ALS restricted phenotypes as an opportunity to unravel ALS focality and spread

Several lines of evidence shoe that motor neuron degeneration starts as a focal process in amyotrophic lateral sclerosis (ALS), subsequently spreading through the complex anatomy of the motor system. ALS may present with considerable phenotypic heterogeneity, reflecting the varied degree of involvement of both motor and extramotor areas.1 Even though, historically, clinicpathological studies have provided an unevaluable contribution to the identification of ALS as a nosological entity, to unravel the central pathogenetic role of the phosphorylated 43 kDa transactive response DNA-binding protein (pTDP-43) as well as to conceptualise disease spreading, few reports have been focused on ALS restricted phenotypes so far.

Sainouchi et al present an intriguing clinicopathological study of two cases of hemiplegic ALS. Both patients presented at onset with upper motor neuron (UMN) and lower motor neuron (LMN) unilateral leg signs that spread…

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