Progressing supranuclear palsy
Last reviewed: 23.04.2024
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Progressing 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 paralysis and dementia.
Causes of the progressing supranuclear palsy
The cause of the disease is unknown. Degeneration of neurons of basal nuclei and brainstem is shown, neurofibrillary glomeruli containing phosphorylated tau protein are revealed. Lacunar cysts are possible in the basal ganglia and deep white matter.
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Symptoms of the progressing supranuclear palsy
A typical debut in late middle age. Usually there are difficulties when looking up (without neck extension), as well as when climbing and descending from the stairs. Although arbitrary eye movements, especially vertical movements, are difficult, but reflex are preserved. Movement slows down, muscles become rigid, axial dystonia develops, and a tendency appears to fall backwards. Dysphagia and dysarthria with emotional lability (pseudobulbar paralysis) are common, these disorders progress, as in multiple strokes. As a result, dementia develops.
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Treatment of the progressing supranuclear palsy
Treatment is unsatisfactory. Dopamine agonists and amantadine only partially reduce stiffness.