In this way, this review summarizes the origin and the role of insulin in the CNS, and discusses the relationship between insulin and autophagy in some neurodegenerative diseases, such as Alzheimer’s disease (AD), Parkinson’s disease (PD), Huntington’s disease (HD), amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), as well as in spinocerebellar ataxia type 3 (SCA3) and Lafora disease (LD). This evidence concerns the gene INS and Machado-Joseph disease.