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This 75-year-old woman has proven

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Steele Richardson-Olszewski syndrome,

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also known as progressive supranuclear palsy.

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This is another disorder that affects the

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nigrostriatal dopaminergic system,

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which has pathologic features

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that include neuronal loss.

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This patient does have cerebral

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atrophy and astrocytosis,

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but unlike corticobasal degeneration,

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one of its lookalikes,

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the cerebral cortical component,

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occurs much later and is not as severe.

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It is also considered a tauopathy with neuronal

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accumulation of abnormal tau protein.

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Tauopathies encompass a number of diseases,

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including Alzheimer's disease.

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Like many other neurodegenerative disorders,

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there are argyrophilic silver staining aggregates

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of abnormal tau in the brain

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and throughout the brain.

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Many of these affect the substantia nigra.

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So, the substantia nigra is affected

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as it is in Parkinson's disease,

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although the loss of the zona compacta

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hyperintense area is not affected,

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or it's not affected early

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or in the mid-stage of the disease.

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The clinical features include symmetric akinesia,

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which this patient had with rigidity,

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difficulty walking that simulates classic

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Parkinson's disease.

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The abnormalities are most marked axially with

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early disequilibrium and very frequent falls.

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They have supranuclear ophthalmoplegia, dysarthria,

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dysphasia, and because it's so midbrain centric,

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the patients often die from aspiration pneumonia

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at about six or seven years.

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Progressive supranuclear palsy demonstrating

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the hummingbird sign with some anatomy,

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by the way.

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There's your cerebellar vermis,

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there's your primary fissure,

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here's your posterolateral fissure.

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And then as we come up,

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the superior medullary velum,

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the inferior caliculus,

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the very atrophic superior caliculus,

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the habenula posterior commissure,

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then the straight sinus,

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the region of the vein of galen and the internal

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cerebral vein region.

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Here's the massa intermedia.

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There's the splenium, body, genu,

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rostrum, and lamina terminalis of the corpus callosum.

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Here is the midbrain,

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the interpeduncular cistern

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and the mammillary body.

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All anatomic components well seen in the midline

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due to the presence of atrophy

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involving the midbrain in PSP.