Neurodegeneration strikes when we least expect it. Prime of life neurodegeneration is a spectrum of relatively rare diseases that often afflict people during productive, active years and lead to debilitating symptoms and early death. They are generally categorized as frontotemporal disorders and are characterized by progressive personality, behavior, language, and motor decline. These diseases create a level of functional impairment that significantly compromises the ability to carry out activities of daily living. There is currently no treatment and no cure, but there is hope. Research into these diseases may be a gateway to understanding and ultimately preventing.