Progressive Supranuclear Palsy (PSP)

Progressive Supranuclear Palsy (PSP) is the second most common of the four atypical parkinsonisms and exists in two variants: Richardson’s syndrome (PSP-RS) and PSP-parkinsonism (PSP-P).   Depending on whom you are talking to, it is classified as an atypical parkinsonism disorder, a non-Alzheimer’s dementia, a type of frontotemporal degeneration, or a sub-cortical dementia.

PSP Symptoms

The key symptoms of PSP are bradykinesia (slow movement), rigidity (stiffness), and postural instability…in other words, parkinsonism. As a result, we need to look deeper for distinguishing symptoms.

Since 2005, PSP experts refer to two main types of PSP:  Richardson’s syndrome (PSP-RS) and PSP-parkinsonism (PSP-P).

  • Richardson’s syndrome: early onset of postural instability and falls, supranuclear vertical gaze palsy, and cognitive dysfunction
  • PSP-parkinsonism: asymmetric onset, tremor, a moderate initial therapeutic response to levodopa

The symptoms of these two main types are so different that they seem to be entirely different disorders. The PSP-RS disorder described by Drs. Richardson, Steele, and Olszewski in 1964 can be called “classic PSP.” The diagnostic criteria of PSP are focused on this classic type:  supranuclear gaze palsy (inability to voluntarily look downward or upward), early falls, and dementia. The classic type is referred to in an important 2005 paper as “Richardson’s syndrome” (after Dr. Clifford Richardson, one of the early describers). See  “Characteristics of two distinct clinical phenotypes in pathologically proven progressive supranuclear palsy: Richardson’s syndrome and PSP-parkinsonism,” Brain 2005, Williams, et al  This is the more common type and the more easily recognized by neurologists.

The second type, PSP-parkinsonism, is much harder for neurologists to diagnose accurately. Someone with this type of PSP can look like he/she has Parkinson’s Disease.  Because a third of people may have this second type, it’s important to understand the differences.

Over time, most of those with PSP-parkinsonism do experience falls and supranuclear gaze palsy. Only about half experience cognitive loss. This means that PSP-parkinsonism patients can become more easily diagnosed with PSP when they develop the more classic symptoms of the disease. On the other hand, those with Richardson’s syndrome do not develop tremor or have a response to levodopa therapy.

Other common symptoms that many with PSP develop are dysarthria (slurred, inarticulate speech), pseudobulbar palsy (also called emotional incontinence – laughter or crying for no apparent reason), retrocollis (abnormal neck posture where the head is flexed backwards), akinesia (absence of movement), apathy (loss of interest), personality changes such as sudden anger, blurred vision, double vision, blepharospasm (involuntary closing of eyelids), infrequent blinking (and dry eyes), decrease in speech (even mutism), urinary incontinence, etc.

The falls of PSP are often called “unexplained falls” because the person seems to fall out of the blue, for no obvious reason. Prior to being diagnosed, sometimes the person who falls explains that his/her shoes don’t fit properly, he/she is in an unfamiliar place, or he/she became dizzy.

Those with PSP can have a very awkward gait. Friends or strangers might suspect the person is drunk because of the wide stance and staggering gait.

Eventually those with PSP develop swallowing problems (dysphagia). The most common causes of death are related to these problems: development of aspiration pneumonia or the person loses the ability to swallow.

In a sense, there’s a long list of possible symptoms. No two people with PSP look exactly alike. Plus there are other types of PSP that are not as common — such as pure akinesia with gait freezing. Richardson’s syndrome and PSP-parkinsonism are only the two most common types.

Survival time varies greatly based upon the type of PSP. Survival time in Richardson’s syndrome is just under six years, while the survival time in PSP-parkinsonism is just over nine years. These are averages. And these figures are based on the Brain 2005 paper mentioned above.

PSP Treatments

There is remarkably limited treatment for PSP. The most effective treatments are the therapies: physical therapy, occupational therapy, and speech therapy. Therapies are pinpointed on prevention of falls and aspiration pneumonia.

Exercise is key for fall prevention and maintaining mobility. A member of our local support group was told by his wife’s movement disorder specialist that she lived an additional two years due to exercise on a special treadmill.

The medication that can be prescribed and any other treatments are focused on a few symptoms that may respond to medication or treatments such as depression, urinary incontinence, and dry eyes. Those with PSP-parkinsonism do have some response to levodopa medication. There is no evidence that any of the Alzheimer’s medications are helpful with the dementia in Richardson’s syndrome.

Go-To Organizations for PSP Education

A handful of organizations consistently deliver quality information about PSP. Click the link for each organization to review their web sites:

Organization NameCountryFocusWeb Address
CurePSPUSAIncrease awareness, fund research, educate health professionals, support patients & familieshttp://www.psp.org
PSP AssociationUKSupport patients & families, provide educationhttp://www.pspassociation.org.uk

PSP Top Resources

Brain Support Network continually reviews most of the resources available on PSP. In our opinion, these web pages, documents, and videos are the most helpful resources for families:

A Physician’s Guide to PSP

Though this video was produced by CurePSP and the PSP Association for MDs, most of it is understandable to laypeople. This 18-minute video features some of the world’s top experts in PSP including Dr. John Steele and Dr. Lawrence Golbe.
https://www.youtube.com/watch?v=IXMv919LOWE
Brain Support Network notes on the video guide:
https://www.brainsupportnetwork.org/physicians-diagnostic-video-on-psp/

Exploring diagnosis, management and best practice interventions in relation to PSP, MSA, and CBD

This November 2009 conference for allied health professionals (RNs, PTs, OTs, STs, social workers, etc) in Melbourne, Australia was recorded. One of the world’s top researchers of PSP, Dr. David Williams, based in Melbourne, is featured. The multi-hour conference is broken into 10-minute online video segments.
https://www.youtube.com/watch?v=XuPyLTip53w

Notes on the videos: www.brainsupportnetwork.org/psp-cbd-and-msa-conferenceextensive-notes-nov-2009-australia/

Your Personal Guide to PSP

The first section of this excellent 110-page introduction to PSP and CBD focuses on symptoms such as cognition/mood, mobility/falls, fatigue/sleep, pain, swallowing, vision, and bladder/bowel. Published April 2013 by The PSP Association (UK).
http://www.pspassociation.org.uk/app/uploads/2017/01/Personal-Guide-to-PSP-Section-1.pdf

UCSF PSP/CBD Research Update

This February 2014 conference focused on what UCSF Memory and Aging Center researchers have learned thus far about PSP and CBD, and what research is underway. Most of the eight talks are about 14 minutes in length. There’s also an hour-long question-and-answer period with researchers.
http://www.youtube.com/watch?v=56JAzPba59E

Falls and Dysphagia in PSP

This excellent 6-page report was published for healthcare professionals in Summer
2015 by The Association for Frontotemporal Degeneration.  The composite story of “Jackie Riddle” is actually an amalgam of two Brain Support Network volunteers’ loved ones — Phil Myers’s wife Jackie Myers and Robin Ridddle’s father Larry Riddle.
http://www.theaftd.org/wp-content/uploads/2015/07/PinFTDcare_Newsletter_summer_2015.pdf

PSP: Some Answers

12-page document from CurePSP was last updated in July 2016.
http://www.psp.org/wp-content/uploads/2016/08/PSP-SOME-ANSWERS-BROCH_web.pdf

National Institute of Neurological Disorders and Stroke Fact Sheet on PSP

NINDS is part of the NIH. This web page was last updated in September 2015.
http://www.ninds.nih.gov/disorders/psp/detail_psp.htm

Atypical Parkinsonism Symposium

This symposium featured Dr. Irene Litvan and Adam Boxer. Litvan, one of the world’s top experts on PSP, gives the keynote on atypical parkinsonism. Boxer, leader of a worldwide clinical trial in PSP, talks at the break-out session on PSP. This October 2012 Stanford University (SF Bay Area) event was recorded. Brain Support Network was one of the co-organizers.
https://www.youtube.com/channel/UCmbEn5aJoUT_sXo6CSSKw9Q

CurePSP Family Conferences and Webinars

CurePSP records many of its family conferences (available on DVD or online) and webinars (available online). The best neurologist talks at family conferences on PSP are from the Denver event (Dr. Kumar, October 2013), the Boston event (Dr. Golbe, April 2013), and the LA event (Dr. Boxer, May 2011). Stream videos on YouTube (links on the psp.org page) or order a DVD from CurePSP.

Family Conferences Archive: https://www.youtube.com/playlist?list=PLID1PzLG_zrezbfj20Q4iNlK1_TpIzfLO

https://www.youtube.com/user/CurePSP/playlists

A Guide for People Living with PSP, CBD, and Other Atypical Parkinsonian Disorders

122-page booklet from CurePSP was last updated no earlier than September 2012.
https://web.archive.org/web/20150227023317/http://www.psp.org/file_download/bdbad4d5-6215-4624-9aec-6f909f459fb5

PSP: A Primer for the Newly Diagnosed

14-minute video full of facts from PSP expert neurologist Dr. Larry Golbe, and full of hopeful messages from a person with PSP, his wife, a volunteer, and Dr. Golbe.
https://www.youtube.com/watch?v=eAlLKni9K9E
Brain Support Network notes on video:
https://www.brainsupportnetwork.org/psp-a-primer-for-the-newly-diagnosed-video/

Atypical Parkinsonian Disorders

This excellent 60-minute tutorial is for general neurologists, by CurePSP. Most of the video is understandable for laypeople. www.youtube.com/watch?v=BtEiNlivgeI

Notes on video: www.brainsupportnetwork.org/educational-video-for-neurologistsdiagnosing-mostly-and-treating-psp-cbs-msa-and-dlb/

PSP Blog and Registry

This blog, begun in March 2014 and last udpated in May 2016 by Dr. Lawrence Golbe, a PSP expert, is oriented towards clinicians and researchers, but public is welcome. “Registry” is open to all.
http://psp-blog.org

PSP Diagnostic Criteria

The National Institute of Neurological Disorders and Stroke and the Society for Progressive Supranuclear Palsy (NINDS-SPSS) are the source of the diagnostic criteria reported on this page.  See Litvan I, et al. “Clinical research criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome): report of the NINDS-SPSP international workshop, Neurology. 1996 Jul;47(1):1-9.

These criteria describe the classic type of PSP, Richardson’s syndrome. We do not list criteria for PSP-parkinsonism or the less common types, consistent with the fact that they are much more difficult to diagnose.

PSP-RS is characterized by a set of symptoms.  In the NINDS-SPSS report, symptoms were assigned to one of two categories:

  • Supportive
  • Exclusionary

The diagnosis of the disease depends on  the symptoms present.  The report delivered three classes of diagnosis:

  • Definite
  • Probable
  • Possible
Supportive Symptoms of PSP
Exclusionary Symptoms of PSP
  • Symmetric akinesia or rigidity, proximal more than distal
  • Abnormal neck posture, especially retrocollis
  • Poor or absence of response of parkinsonism to levodopa therapy
  • Early dysphagia and dysarthria
  • Early onset of cognitive impairment including at least two of the following: apathy, impairment in abstract thought, decreased verbal fluency, use or imitation behavior, or frontal release signs
  • Recent history of encephalitis
  • Alien limb syndrome, cortical sensory deficits, focal frontal or temporoparietal atrophy (These symptoms are suggestive of corticobasal degeneration.)
  • Hallucinations or delusions unrelated to dopaminergic therapy (These symptoms are suggestive of Lewy body dementia.)
  • Cortical dementia of Alzheimer type
  • Prominent early cerebellar symptoms or prominent early unexplained dysautonomia (These symptoms are suggestive of multiple system atrophy.)
  • Severe, asymmetric parkinsonian signs
  • Neuroradiologic evidence of relevant structural abnormalities (These symptoms are suggestive of stroke or vascular parkinsonism.)
  • Whipple’s disease, confirmed by polymerase chain reaction
Definite Diagnosis of PSP-RSProbable Diagnosis of PSP-RSPossible Diagnosis of PSP-RS
  • Clinically probable or possible PSP and confirming brain autopsy
  • Gradually progressive disorder
  • Onset at age 40 or later
  • Vertical supranuclear palsy and prominent postural instability with falls in the first year of disease onset
  • No evidence of other diseases that could explain the foregoing features, as indicated by mandatory exclusion criteria
  • Gradually progressive disorder
  • Onset at age 40 or later
  • Either vertical supranuclear palsy or both slowing of vertical saccades and prominent postural instability with falls in the first year of disease onset
  • No evidence of other diseases that could explain the foregoing features, as indicated by mandatory exclusion criteria