FUS and Juvenile onset: Two patients, with mutations located in the nuclear localization signal (NLS)—R495X and a likely de novo P525L—exhibited aggressive, juvenile-onset ALS with multiple neuronal cytoplasmic FUS inclusions distributed in the brain and spinal cord, supporting previously reported (classical) FUS neuropathology [9, 10] (Fig. 1B–E, Fig. 2B, D–F).