This highlights a high degree of heterogeneity and complexity in the neuropathological changes associated with myotonic dystrophy type 1.15,16 Given the interdependence between muscle and nervous system, it is plausible that muscle defects in myotonic dystrophy type 1 may influence spinal cord function and splicing mechanisms.17-20 The HSALR mouse model, which expresses a human skeletal actin (HSA) gene containing a CTG repeats expansion of ∼220 repeats, is well established for studying myotonic dystrophy type 1. This evidence concerns the gene ALB and myotonic dystrophy type 1.