Acquired non-Wilsonian hepatocerebral degeneration

Objective : analysis of clinical features of non-Wilsonian hepatocerebral degeneration. Material and methods : a systematization and analysis of data from domestic and foreign literature on the pathogenesis, clinical picture, diagnosis and treatment of a rare variant of chronic encephalopathy against the background of metabolic disorders — non-Wilsonian hepatocerebral degeneration were carried out. Results . Our own clinical observation is presented. The main aspects of pathogenesis and risk factors are listed. The conditions and diseases that require differential diagnostics in case of suspected hepatocerebral degeneration are systematized: Wilson-Konovalov disease, non-Wilsonian hepatocerebral degeneration associated with nitrogenous base metabolism disorder, chronic manganese intoxication. Conclusion . Due to the rare occurrence and non-specific clinical manifestations, the availability of information on the pathogenesis, clinical picture, differential diagnostics and treatment of hepatocerebral degeneration of various etiologies remains low. Improvement of the differentiated choice of diagnostic methods by a specialist, in particular magnetic resonance imaging, laboratory methods for assessing metabolic disorders, as well as gene diagnostics options contribute to the improvement of detection and differentiation of these conditions.