Progressive Supranuclear Palsy for clinicians

Progressive Supranuclear Palsy (PSP or Steele-Richardson-Olseweski syndrome) is a rapidly degenerative neurological disorder.

Prevalence

  • The prevalence of PSP is 6.5 per 100,000 (similar to Motor Neuron Disease)
  • average age of onset is 60 to 65 years
  • average life expectancy is six to seven years from diagnosis.

Cause

PSP belongs to the family of tauopathies. Abnormalities in the protein tau lead to damage in both cortical and subcortical areas of the brain.

Diagnosis

  • PSP is difficult to diagnose.
  • Patients may remain undiagnosed for approximately half of the natural history of their disease.
  • Patients with PSP present with parkinsonian symptoms of slowness of movement, difficulty initiating movement, and rigidity with or without resting tremor. PSP differs from Parkinson’s Disease with rapid progression of their disorder, absent/poor/waning response to dopaminergic medications, early instability or falls, early dysphagia and/or dysarthria.

Symptoms

Symptoms may include:

  • mobility difficulties - falls (often backwards), balance, postural instability, slow movements, rigidity, dystonia
  • vision difficulties - vertical gaze palsy (may not be present in early stages), photophobia, diplopia (blurred vision), involuntary eyelid spasms, dry eyes
  • swallowing difficulties - dysphagia, impulsive feeding, excess saliva/drooling, needing PEG Feeding (late stages)
  • communication difficulties - speech problems, occasionally stuttering
  • cognition/mood difficulties - apathy, depression, anxiety, disinhibition, irritability, emotional lability, executive dysfunction, slowness of thought.
  • bowel and bladder difficulties - constipation, incontinence
  • sleep disturbances - insomnia, REM sleep behaviour disorder, excessive daytime sleepiness, restless legs syndrome.

Cures or treatments

Possible cures for PSP are being researched. Currently, there are a number of symptomatic therapies available which can help to improve quality of life.

Medications

  • Appropriate medications for symptomatic management continue to evolve and it may be beneficial to consult with a Neurologist and/or Geriatrician
  • high levodopa in some circumstances has limited effect
  • atropine eye drops or atrovent nasal spray administered under the tongue may assist in drying excess secretions/assist with drooling, medications to assist with constipation
  • medications may assist with constipation.

Non-pharmacological treatments

  • Constipation: daily movement, sufficient fluid and fibre intake.

Management of symptoms

For a list of management symptoms, see the Progressive Supranuclear Palsy fact sheet for GPs and Healthcare Providers (PDF 325KB). Some of these include:

  • allied health care
  • psychology/counselling
  • and a number of specialised clinics.

Useful references/associations

Further information

For further information, contact:

Speech Pathology
Repatriation General Hospital
Daws Road, Daw Park SA 5041
Telephone: (08) 8275 1861