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Progressive supranuclear palsy.
Last reviewed: 04.07.2025
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Progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome) is a rare degenerative disease of the central nervous system characterized by loss of voluntary eye movements, bradykinesia, muscle rigidity with progressive axial dystonia, pseudobulbar palsy and dementia.
Causes of progressive supranuclear palsy.
The cause of the disease is unknown. Degeneration of neurons in the basal ganglia and brainstem is shown, neurofibrillary tangles containing phosphorylated tau protein are detected. Lacunar cysts are possible in the basal ganglia and deep white matter.
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Symptoms of progressive supranuclear palsy.
Onset is typical in late middle age. Difficulty in looking up (without extending the neck) and in going up and down stairs is common. Although voluntary eye movements, especially vertical ones, are difficult, reflex movements are preserved. Movements slow down, muscles become rigid, axial dystonia develops, and a tendency to fall backwards appears. Dysphagia and dysarthria with emotional lability (pseudobulbar palsy) are common; these disorders progress as in multiple strokes. Dementia eventually develops.
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Treatment of progressive supranuclear palsy.
Treatment is unsatisfactory. Dopamine agonists and amantadine only partially reduce rigidity.