Skip to content
How It Works
Explore
Resources Ask a Question
Who It's For
Patients
Decode results, prep for appointments, weigh options.
Caregivers & advocates
Carry the research load for someone you love.
Providers & clinicians
Resources for patients in your practice.
Log In Ask a Question
PubMed This is a summary of 60 peer-reviewed journal articles Updated
Neurology · Progressive Supranuclear Palsy

Understanding Atypical PSP: First Steps After Diagnosis

At a Glance

An atypical PSP diagnosis means your symptoms differ from classic Richardson's syndrome and may progress more slowly. While not directly inherited, atypical variants like PSP-P require a specialized neurologist and care team to manage specific movement, speech, and balance changes.

Receiving a diagnosis of Progressive Supranuclear Palsy (PSP) is a significant life event, but hearing the word “atypical” alongside it can add another layer of confusion. It is important to know that in the world of PSP, “atypical” is not necessarily a more frightening label. In fact, for many variants, it can be a sign that the disease may move more slowly than the “classic” form of the condition [1][2].

What is PSP?

Progressive Supranuclear Palsy (PSP) is a rare brain disorder that affects movement, balance, vision, and sometimes speech or behavior [3]. It is known as a tauopathy, which means it is caused by the abnormal buildup of a protein called tau in certain parts of the brain [4]. When this protein clumps together, it disrupts the way brain cells communicate and eventually causes them to stop working [5].

A Reassuring Note on Genetics: Families newly diagnosed often worry about passing this condition to their children. It is important to know that PSP is almost always sporadic, meaning it occurs by chance and is not directly inherited [3].

While there is currently no cure, understanding the specific type of PSP you or your loved one are dealing with is the first step in managing the journey ahead [6].

Redefining “Atypical”

For decades, doctors only recognized one form of PSP, called Richardson’s syndrome (PSP-RS) [7]. This is the “classic” version characterized by early falls and significant eye movement issues. Today, medical guidelines recognize that PSP is actually a broad spectrum [8].

If you or your loved one has “atypical” PSP, it simply means the symptoms do not follow that classic Richardson’s pattern [8][9]. Common atypical variants include:

  • PSP-Parkinsonism (PSP-P): Often resembles Parkinson’s disease in the early stages, sometimes including a tremor [8][10].
  • PSP-Progressive Gait Freezing (PSP-PGF): Primary symptom is a “freezing” of gait, where the feet feel stuck to the floor [11].
  • PSP-Speech/Language (PSP-SL): Symptoms start primarily with changes in the ability to speak or find words [12].

Completeness Check: What to Focus on Now

Because atypical PSP is different for everyone, your focus should be on the specific symptoms present rather than a “one size fits all” checklist.

01

Biology and Subtypes: The Spectrum of Atypical PSP

Learn about atypical Progressive Supranuclear Palsy (PSP) subtypes, including PSP-P, PSP-CBS, and PSP-F. Understand how 4R-tau biology impacts your symptoms.

02

Getting the Right Diagnosis: Criteria and Imaging

Understand how atypical Progressive Supranuclear Palsy (PSP) is diagnosed. Learn about 2017 MDS criteria, the MRI Hummingbird sign, and PSP vs. Parkinson's.

03

Symptom Management and Building Your Care Team

Learn how to manage atypical progressive supranuclear palsy (PSP) symptoms. Discover physical therapy tips, medication options, and how to build a care team.

04

Prognosis and What to Expect Over Time

Learn about the prognosis and timeline for atypical progressive supranuclear palsy (PSP). Understand variant life expectancy, milestones, and complications.

Common questions in this guide

What does an atypical PSP diagnosis mean?
Atypical PSP means your symptoms do not follow the classic pattern of early falls and eye movement issues seen in Richardson's syndrome. Reassuringly, for many people, atypical variants progress more slowly than the classic form of the disease.
Is atypical progressive supranuclear palsy inherited?
Families newly diagnosed often worry about passing this condition to their children. However, PSP is almost always sporadic, meaning it occurs by chance and is not directly inherited.
What are the different types of atypical PSP?
Common variants include PSP-Parkinsonism (PSP-P) which often features tremors, PSP-Progressive Gait Freezing (PSP-PGF) where feet feel stuck to the floor, and PSP-Speech/Language (PSP-SL) which starts with changes in communication abilities.
Are there medications or treatments for atypical PSP?
While there is no cure, some atypical variants like PSP-Parkinsonism may show partial or temporary improvement with Parkinson's medications like levodopa. An integrated care plan involving physical and speech therapy is also essential for maintaining quality of life.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.Which specific variant of atypical PSP am I dealing with (e.g., PSP-P or PSP-PGF)?
  2. 2.Based on this diagnosis, what can we expect regarding the speed of progression compared to the more common 'classic' form?
  3. 3.Is it common for people with this variant to respond to Parkinson's medications like levodopa, even if only for a short time?
  4. 4.What specific clinical signs or imaging results led to the 'atypical' classification rather than Richardson's syndrome?
  5. 5.Can you refer us to a multidisciplinary care team (including physical and speech therapy) that has experience with these specific variants?

Questions For You

Tap a prompt to share your answer — we'll use it plus this page's context to start a tailored conversation.

References

References (14)
  1. 1

    Subtypes of PSP and Prognosis: A Retrospective Analysis.

    Mahale RR, Krishnan S, Divya KP, et al.

    Annals of Indian Academy of Neurology 2021; (24(1)):56-62 doi:10.4103/aian.AIAN_611_20.

    PMID: 33911380
  2. 2

    The clinical spectrum and natural history of pure akinesia with gait freezing.

    Owens E, Josephs KA, Savica R, et al.

    Journal of neurology 2016; (263(12)):2419-2423 doi:10.1007/s00415-016-8278-x.

    PMID: 27624121
  3. 3

    Advances in progressive supranuclear palsy: new diagnostic criteria, biomarkers, and therapeutic approaches.

    Boxer AL, Yu JT, Golbe LI, et al.

    The Lancet. Neurology 2017; (16(7)):552-563 doi:10.1016/S1474-4422(17)30157-6.

    PMID: 28653647
  4. 4

    Motor and non-motor features in progressive supranuclear palsy: the impact of microtubule associated protein tau haplotypes among a Tunisian cohort.

    Abida Y, Sghaier I, Souissi A, et al.

    Journal of neural transmission (Vienna, Austria : 1996) 2026; (133(3)):423-432 doi:10.1007/s00702-025-03004-z.

    PMID: 40810960
  5. 5

    4R-tau seeding activity reveals molecular subtypes in progressive supranuclear palsy.

    Martinez-Valbuena I, Lee S, Santamaria E, et al.

    Nature communications 2025; (17(1)):1006 doi:10.1038/s41467-025-67744-y.

    PMID: 41476155
  6. 6

    Which ante mortem clinical features predict progressive supranuclear palsy pathology?

    Respondek G, Kurz C, Arzberger T, et al.

    Movement disorders : official journal of the Movement Disorder Society 2017; (32(7)):995-1005 doi:10.1002/mds.27034.

    PMID: 28500752
  7. 7

    Sensitivity and Specificity of Diagnostic Criteria for Progressive Supranuclear Palsy.

    Ali F, Martin PR, Botha H, et al.

    Movement disorders : official journal of the Movement Disorder Society 2019; (34(8)):1144-1153 doi:10.1002/mds.27619.

    PMID: 30726566
  8. 8

    Graphic Summary of Movement Disorders Society Criteria for Progressive Supranuclear Palsy and Multiple Allocations eXtinction Rules.

    Ogawa T, Hatano T, Oyama G, et al.

    Movement disorders clinical practice 2020; (7(2)):240-242 doi:10.1002/mdc3.12894.

    PMID: 32071951
  9. 9

    Clinical diagnosis of progressive supranuclear palsy: The movement disorder society criteria.

    Höglinger GU, Respondek G, Stamelou M, et al.

    Movement disorders : official journal of the Movement Disorder Society 2017; (32(6)):853-864 doi:10.1002/mds.26987.

    PMID: 28467028
  10. 10

    Development and Validation of Automated Magnetic Resonance Parkinsonism Index 2.0 to Distinguish Progressive Supranuclear Palsy-Parkinsonism From Parkinson's Disease.

    Quattrone A, Bianco MG, Antonini A, et al.

    Movement disorders : official journal of the Movement Disorder Society 2022; (37(6)):1272-1281 doi:10.1002/mds.28992.

    PMID: 35403258
  11. 11

    Association of PSP phenotypes with survival: A brain-bank study.

    Guasp M, Molina-Porcel L, Painous C, et al.

    Parkinsonism & related disorders 2021; (84()):77-81 doi:10.1016/j.parkreldis.2021.01.015.

    PMID: 33581485
  12. 12

    An Evaluation of the Progressive Supranuclear Palsy Speech/Language Variant.

    Whitwell JL, Stevens CA, Duffy JR, et al.

    Movement disorders clinical practice 2019; (6(6)):452-461 doi:10.1002/mdc3.12796.

    PMID: 31392246
  13. 13

    Tau in Atypical Parkinsonisms: A Meta-Analysis of in Vivo PET Imaging Findings.

    Mena AM, Chen R, Graff-Guerrero A, et al.

    Movement disorders clinical practice 2023; (10(12)):1725-1737 doi:10.1002/mdc3.13885.

    PMID: 38094644
  14. 14

    Multidisciplinary care use in neurodegenerative complex diseases: The example of progressive supranuclear palsy and advanced Parkinson's disease in real-life.

    Fabbri M, Ledda C, Schirinzi T, et al.

    Parkinsonism & related disorders 2024; (125()):107047 doi:10.1016/j.parkreldis.2024.107047.

    PMID: 38964017

This page explains atypical PSP variants and symptoms for educational purposes. Always consult a neurologist to interpret your specific diagnosis, variant, and comprehensive care plan.

Get notified when new evidence is published on Atypical progressive supranuclear palsy syndrome.

We monitor PubMed for new peer-reviewed studies on this topic and email a short summary when something meaningful changes.