Progressive supranuclear palsy

Progressive supranuclear palsy (PSP), also known as Steele-Richardson-Olszewski syndrome, is a rare neurodegenerative disorder characterized by the abnormal accumulation of tau protein in the brain. It primarily affects the brainstem, basal ganglia, cerebral cortex, and cerebellum, leading to progressive difficulties with balance, movement, vision, speech, and cognition. PSP belongs to the group of disorders known as tauopathies. The hallmark clinical feature of PSP is vertical supranuclear gaze palsy — a progressive inability to voluntarily move the eyes, particularly in the downward direction. Other key symptoms include early postural instability with frequent backward falls, axial rigidity (stiffness of the trunk and neck more than the limbs), bradykinesia (slowness of movement), dysarthria (slurred speech), dysphagia (difficulty swallowing), and a frontal-type dementia characterized by apathy, impulsivity, and executive dysfunction. Unlike Parkinson's disease, PSP typically does not respond well to levodopa therapy, and tremor is usually absent or minimal. The disease typically manifests in the sixth or seventh decade of life, with a median survival of approximately 6 to 9 years from symptom onset. Several clinical subtypes have been recognized, including Richardson syndrome (the classic form), PSP-parkinsonism, PSP-progressive gait freezing, and PSP-corticobasal syndrome, among others. There is currently no cure or disease-modifying treatment for PSP. Management is supportive and multidisciplinary, involving physical therapy to address gait and balance issues, speech therapy for swallowing and communication difficulties, occupational therapy, and pharmacological approaches for symptomatic relief. Levodopa may provide modest and transient benefit in some patients, particularly those with the PSP-parkinsonism subtype. Research into tau-targeted therapies is ongoing.

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