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Propagation: Prion-like mechanisms can explain spreading of motor neuronal death in amyotrophic lateral sclerosis?
  1. Satoshi Kuwabara1,
  2. Takanori Yokota2
  1. 1Department of Neurology, Graduate School of Medicine, Chiba University, Chiba, Japan
  2. 2Department of Neurology and Neurological Science, Tokyo Medical and Dental University, Tokyo, Japan
  1. Correspondence to Dr Satoshi Kuwabara, Department of Neurology, Graduate School of Medicine, Chiba University, 1-8-1 Inohana, Chuo-ku, Chiba 260-8670, Japan; kuwabara-s{at}faculty.chiba-u.jp

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Although amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease diffusely affecting the human motor system, there is a large variability in the clinical expression of the disease (site of onset, speed of progression, involvement of lower- or upper-motor neuron). The phenotypes, particularly at the site of onset, are highly heterogeneous. Bulbar muscles are first affected in some patients, and upper- or lower-limb muscles are initially involved in others; the manifestations are focal and discrete at clinical onset and then progress contiguously outward over time to become diffuse and complex.1 2

In other common neurodegenerative diseases, such as Alzheimer's disease and Parkinson's disease, the molecular pathogenesis is characterised by the misfolding of proteins that assemble into ordered aggregates in affected neurons. So far, it has been considered that the diseases affect the most vulnerable neurons to form aggregates and that non-cell …

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