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Hemiplegic-type ALS shows a strong correlation between upper, lower motor neuron degeneration and pTDP-43 pathology
  1. Nilo Riva,
  2. Angelo Quattrini
  1. Department of Neurology, Institute of Experimental Neurology (INSPE), Division of Neuroscience, San Raffaele Hospital Institute of Experimental Neurology, Milano, Lombardia, Italy
  1. Correspondence to Dr Nilo Riva, Department of Neurology, Institute of Experimental Neurology (INSPE), Division of Neuroscience, San Raffaele Hospital Institute of Experimental Neurology, Milano, Lombardia, Italy; riva.nilo{at}hsr.it

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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 …

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Footnotes

  • Contributors NR conceptualised and designed the study, generated the first draft of the manuscript. All authors critically revised the manuscript for intellectual content and edited manuscript drafts; NR collated all authors’ comments for the final submitted version. The corresponding author had final responsibility for the decision to submit for publication. All authors approved the final version of the manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Commissioned; internally peer reviewed.

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    Makoto Sainouchi Hidetomo Tanaka Hiroshi Shimizu Takuya Mashima Takao Fukushima Yuya Hatano Tomohiko Ishihara Kunihiko Makino Osamu Onodera Akiyoshi Kakita