Neuropathological evidence from a small number of patients is consistent with the hypothesis of FUS pathology spreading within the central nervous system (CNS), including 1) clinical symptoms often start focally and spread as disease progresses [30–32] and 2) FUS cytoplasmic inclusions have been observed in several regions of the CNS of ALS and FTLD-FUS patients with similar spatial patterns as in FTLD-Tau or FTLD-TDP-43 forms [2, 33–35]. This evidence concerns the gene FUS and amyotrophic lateral sclerosis.