Numerous mis-splicing events have been described in genes expressed in the myotonic dystrophy type 1 brain implicated in its CNS symptomatology, including MAPT exons 2–3 and 10, GRIN1 exons 5 and 21, APP exon 8, MBNL1/2 exon 7 and CAMK2D exons 14–15.16,57-59 Previous research has revealed that spinal motor neurons in myotonic dystrophy type 1 exhibit more pronounced splicing abnormalities compared to cortical neurons, while the cerebellum exhibits fewer mis-splicing events. Here, APP is linked to myotonic dystrophy type 1.