The guide is devoted to the diagnosis and treatment of patients with hereditary diseases of the nervous system. The guide "Hereditary Diseases of the Nervous System," written with the participation of a team of authors of the MNII Pediatrics and Pediatric Surgery of the Ministry of Health of the Russian Federation and the Institute of Neurology of the Russian Academy of Medical Sciences, is timely and, of course, useful for a wide range of specialists who face neurogenetics problems in everyday practice. For the first time, diseases are described, accompanied by characteristic disorders of bioenergetics processes due to mutations of both mitochondrial and nuclear DNA, diagnostic criteria and recommendations for therapy are presented.