Causes of progressive supranuclear palsy

Causes of progressive supranuclear palsy

The cause of progressive supranuclear palsy is unknown.

Radiologic and pathologic evidence indicates that a multi-infarct state can cause a picture identical to progressive supranuclear palsy.

Idiopathic progressive supranuclear palsy is pathologically characterized by marked neuronal cell loss in subcortical structures, such as the nucleus basalis of Meynert, pallidum, STN, SN, locus ceruleus, and superior colliculi.

Other pathologic features include neurofibrillary tangles, granulovacuolar degeneration, and gliosis.

Atrophy, generalized or focal (midbrain or cerebellum), is the most common neuroradiologic finding in idiopathic progressive supranuclear palsy.

Because of disproportionate atrophy of the dorsal midbrain compared to the pons, the sagittal view of magnetic resonance imaging (MRI) often gives the appearance of a “penguin” or “hummingbird,” and these “birds of progressive supranuclear palsy” are characteristic radiological features.

However, up to 25% of the patients with progressive supranuclear palsy have no abnormality on computed tomography and/or MRI of the brain.

Growing evidence suggests linkage disequilibrium between a progressive supranuclear palsy gene and allelic variants of the tau gene.

progressive supranuclear palsy is classified as a form of tauopathy.


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