Brain, Progressive supranuclear palsy

Progressive supranuclear palsy (PSP) is a neurodegenerative disease characterized by accumulation of phosphorylated tau protein in neurons and glia (“tauopathy”). This results in neuron loss and gliosis. Clinically, PSP classically presents as the Steele-Richardson-Olszewski syndrome of postural instability, supranuclear gaze palsy, pseudobulbar palsy, and axial rigidity. Other clinical presentations such as corticobasal syndrome, behavioural variant frontotemporal dementia, and primary progressive aphasia are also possible. Pathologically, the basal ganglia, diencephalon, and brainstem nuclei are most typically affected by neuron loss. The characteristic protein inclusions are all composed of 4-repeat tau and include neurofibrillary tangles, neuropil threads, tufted astrocytes (most specific), and oligodendroglial coiled bodies. In this case, there is an unusual abundance of tufted astrocytes seen in the cortex in addition to the other types of inclusions (see Related Content). On average, patients live about 7 years after diagnosis.