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Progressive Supranuclear Palsy (PSP)

Atypical parkinsonism with vertical gaze palsy and early falls

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 (PSP)?

PSP is a sporadic 4R-tauopathy with characteristic pathology in the basal ganglia, brainstem and cerebellum.

Estimated prevalence is 5–7 per 100,000; mean onset age is 60–65 years.

Several clinical phenotypes are recognized: PSP-Richardson syndrome (classic), PSP-parkinsonism, PSP-progressive gait freezing, PSP-corticobasal syndrome, PSP-frontal, PSP-speech/language.

Diagnosis is clinical, supported by MRI showing midbrain atrophy ('hummingbird sign'), and the 2017 MDS-PSP criteria.

Median survival is 6–9 years from symptom onset; no disease-modifying therapy is currently available.

Symptoms

Vertical supranuclear gaze palsy (especially downgaze) — pathognomonic when present
Postural instability with backward falls within the first year
Symmetric parkinsonism with axial rigidity, poor levodopa response
Behavioral and cognitive changes: apathy, disinhibition, executive dysfunction, frontal-subcortical pattern
Pseudobulbar features: dysarthria, dysphagia, emotional incontinence
Eyelid abnormalities: blepharospasm, apraxia of lid opening or closure
Procerus sign (worried frown), reduced blinking, applause sign
Falls leading to fractures and head trauma — early disability

Risk Factors

Sporadic disease in the great majority — modest age-related risk
Rare familial forms with MAPT (tau) mutations
Specific MAPT haplotype (H1/H1) overrepresented in PSP
Possible environmental contributions (atypical parkinsonism clusters in Guadeloupe linked to annonacin)
No clear protective or modifiable risk factors identified

When to See a Doctor?

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

  • Recurrent falls within first year of parkinsonism, especially backward falls
  • Early difficulty with downward gaze, frequent meal/drink spillage from impaired downgaze
  • Symmetric parkinsonism poorly responsive to levodopa
  • Rapid cognitive decline with prominent apathy and behavioral change
  • Frequent choking on food or drink (early dysphagia warning)

Treatment Methods

01
Detailed neurologic and ophthalmologic assessment; brain MRI for midbrain atrophy
02
Symptomatic levodopa trial (high doses up to 1000–1500 mg/day) — usually limited but worth attempting
03
Botulinum toxin for blepharospasm, apraxia of eyelid opening, dystonia
04
Multidisciplinary rehabilitation: physical therapy emphasizing fall prevention, occupational therapy, speech and swallow therapy
05
Antidepressants (SSRIs) for mood symptoms; cautious use of atypical antipsychotics for severe behavioral problems
06
Walking aids (weighted walkers preferred to prevent backward falls), home modifications, fall-proofing
07
Advance care planning, palliative care involvement; PEG feeding decisions when dysphagia advances

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.

Learn About Nöroloji Department

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You can make an appointment with our specialists or contact us for your concerns.

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.