Fact-sheet: Progressive supranuclear palsy - PSP


Updated on 11/17/2025 at 6:29 PM

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Definition

Rare, late-onset neurodegenerative disease secondary to accumulation of 4R tau protein, characterized by:

  • supranuclear oculomotor involvement
  • postural instability,
  • then progressive rigidity and cognitive decline.

Also known as Steele-Richardson-Olszewski disease.

Clinical features

Atypical parkinsonian syndrome (little or no response to dopaminergic treatment, faster progression and reduced survival compared with Parkinson disease).

The disease begins with:

  • a symmetric axial akinetic-hypertonic syndrome, with instability and falls.
  • little resting tremor.
  • vertical gaze palsy.
  • cognitive impairment (dementia), dysarthria, cerebellar syndrome, pyramidal syndrome, dysautonomia and sphincter disturbances develop rapidly.

MRI

  • midbrain atrophy (penguin sign or hummingbird sign) on midline sagittal (thin: 3 mm) T1 and T2 sequences. Measurements on a midline sagittal T2 slice, see image below
  • Midbrain area / pons area < 0.2 (or <0.16 according to Oba et al., Neurology 2005);
  • Anteroposterior diameter of the midbrain / anteroposterior diameter of the pons < 0.52

MRI progressive supranuclear palsy versus normal midbrain

  • morning glory sign on axial T1 and T2 sequences = widening of the interpeduncular cistern, concavity of the posterolateral midbrain surface.
  • Mickey Mouse sign (cerebral peduncles resembling Mickey Mouse ears)
  • no significant pontine atrophy.
  • T2 FLAIR hyperintensity and atrophy of the superior cerebellar peduncles.

Management

Symptomatic treatment.

Occasionally, partial response to L-dopa.

Differential diagnosis

  • Parkinson's disease
  • Parkinsonian-type multiple system atrophy
  • Other atypical parkinsonian syndromes (corticobasal degeneration)
  • Frontotemporal dementias
  • Niemann-Pick disease type C