Mutations in the ATP13A2 gene cause Kufor-Rakeb syndrome (KRS), a juvenile-onset pallido-pyramidal neurodegenerative disorder characterized by slowly progressive levodopa-responsive parkinsonism often with additional features including supranuclear gaze palsy, pyramidal dysfunction, dystonia and dementia [4], [5], [6]. This evidence concerns the gene ATP13A2 and Parkinson disease.