The loss of striatal efferent neurons has been described in Huntington disease and X-linked recessive dystonia parkinsonism (Reiner et al., 1988; Goto et al., 2005); however, striatal efferent projections can be involved in the TDP-43-related FTLD/ALS disease (Riku et al., 2016). This evidence concerns the gene TARDBP and juvenile Huntington disease.