Neurológia pre prax 3/2023

Autoimmune leukoencephalopatis with predominant brain involvement

Leukoencephalopathies can be difficult to diagnose. Diagnosis must focus on treatable causes. However, in addition to vascular, infectious and autoimmune causes, there are an increasing number of treatable genetic disorders. In the case of autoimmune encephalopathies, diagnosis and treatment have improved in recent years, allowing patients to be treated more quickly and effectively. Although there are still many unanswered questions about the mechanism by which the immune system attacks brain tissue, a better understanding of this mechanism will enable targeted immunotherapy to be chosen and prevent the possible worsening of the disease if the wrong treatment is applied. The aim of this article is to highlight the rarer inflammatory autoimmune leukoencephalopathies with predominant brain involvement, focusing on the targets and mechanisms of the immune response, and to help in the often difficult diagnosis of these entities.

Keywords: autoimmune leukoencephalopathy, differential diagnosis, CNS vasculitis, astrocytopathy, inflammatory vasculopathies, demyelinating diseases