Morphological characteristics of midbrain structures in progressive supranuclear palsy

Progressive supranuclear palsy (PSP) is a heterogeneous, rapidly progressive neurodegenerative disease that leads to disturbances of eye movement, postural instability, akinesia, and cognitive impairment. It is considered a sporadic disease, affecting patients of both sexes equally, with a genetic and familial predisposition marked on chromosome 17q21. PSP is the second most common form of neurodegenerative parkinsonism after Parkinson’s disease. It represents a tauopathy characterized by deposits of neurofibrillary tangles in neurons and glial cells, as well as the presence of neuropil threads and tufted astrocytes in various brain regions. A similar pathohistological picture is observed in other tauopathies, which makes the pathological diagnosis of PSP complicated. In recent years, it has been shown that the disease can manifest through several distinct phenotypes and that symptoms overlap with other neurodegenerative disorders, particularly Parkinson’s disease. There are no biological markers for antemortem diagnosis of PSP. The disease progresses rapidly, with a progressive increase in functional disability, and ends fatally within five to ten years after symptom onset. Despite ongoing clinical studies, there is currently no therapy or medication that can modify the course of PSP.