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Progressive Supranuclear Palsy - Richardson Syndrome

Atypical parkinsonian syndrome characterised by vertical gaze palsy and early postural instability.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Nöroloji department. Book Appointment →

What is Progressive Supranuclear Palsy - Richardson Syndrome?

Progressive supranuclear palsy (PSP)-Richardson syndrome is a 4R-tauopathy neurodegenerative disorder defined by Steele, Richardson and Olszewski in 1964. Tau protein accumulation occurs in the basal ganglia, brainstem and cerebellum. Mean age of onset is 60-65; mean survival 6-10 years.

Cardinal features: (1) supranuclear vertical gaze palsy (especially downward gaze; saccade abnormalities), (2) postural instability and unprovoked backward falls (within first year), (3) symmetrical akinetic-rigid parkinsonism (poorly L-dopa responsive), (4) bulbar findings (dysarthria, dysphagia), (5) frontal cognitive findings (apathy, apraxia, executive dysfunction).

MDS-PSP 2017 criteria define different subtypes: PSP-RS (Richardson), PSP-P (parkinsonism), PSP-PGF (progressive gait freezing), PSP-CBS (corticobasal syndrome), PSP-SL (speech-language), PSP-F (frontal). Diagnosis is supported by MRI (midbrain atrophy, 'hummingbird' sign, MR-Parkinson Index). Symptomatic treatment only; tau-targeted therapies in clinical trials. Multidisciplinary care (physiotherapy, speech-swallowing, palliative) is critical.

Symptoms

Vertical gaze palsy (especially downward)
Recurrent backward falls
Postural instability
Symmetrical bradykinesia and rigidity
Axial rigidity (neck > limbs)
Wide-based, slow gait
Astonished facial expression
Dysarthria, dysphagia
Apathy, irritability
Executive dysfunction
Apraxia of eyelid opening
Photophobia, blepharospasm

Risk Factors

Advanced age (>60)
Male gender (slight)
MAPT H1 haplotype
Family history (rare; sporadic)
Environmental tau triggers (Guadeloupean PSP)
Head trauma (controversial)

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Recurrent backward falls
  • Vertical eye movement difficulty
  • Bradykinesia + early falls
  • L-dopa unresponsive parkinsonism
  • Apathy, frontal behaviour change
  • Dysphagia (recurrent aspiration)
  • Speech disturbance
  • Visual symptoms (photophobia, blepharospasm)

Treatment Methods

01
Brain MRI (midbrain atrophy assessment)
02
MDS-PSP 2017 diagnostic criteria
03
Tauopathy-specific PET (in research)
04
Limited symptomatic L-dopa trial (300-1500 mg)
05
Amantadine (parkinsonism, freezing)
06
Donepezil (cognitive)
07
Botulinum toxin (blepharospasm, sialorrhoea)
08
Physiotherapy and balance training
09
Fall prevention precautions
10
Speech-language and swallowing therapy
11
PEG feeding (advanced dysphagia)
12
Palliative care and family support
13
Tau-targeted therapies (in clinical trials)
14
Multidisciplinary follow-up

Which Department to Visit?

You can visit our Nöroloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.