It has been reported that ATXN2 induces an increase of phosphorylated TDP-43 in the spinal anterior horn but not in motor cortex neurons of patients with ALS.19 Interestingly, our data show that the spinal phenotype is more common in patients with ATXN2 polyQ intermediate number of repeats than those without expansion. Here, ATXN2 is linked to amyotrophic lateral sclerosis.