A How-To Guide for Doctors

Educating health care providers about PSP and CBD has long been a goal of mine and of CurePSP.  Most of my patients relate unfortunate stories of bothersome or even disabling symptoms for years before any physician suspected the correct diagnosis.  During those years, they may have endured futile, expensive, and potentially harmful diagnostic tests and treatments.  Even after PSP or CBD is correctly diagnosed, attempts to manage the symptoms are often unsupported by evidence, prescribed at an inappropriate dosage, or continued after any benefit has disappeared — while their side effects continue.

All too often, the neurologist tersely informs the patient that no treatment is available for PSP or CBD and that they should just go home, do the best they can and maybe get some physical therapy.  While it’s true that there’s no “specific” treatment or way to slow the underlying disease process, there are treatments that ease most of the symptoms as symptoms.  This is called “palliative” or “symptomatic” management and it’s up to the neurologist and other clinicians to understand and offer it.

These management measures are not unique to PSP or CBD – they are standard drugs and therapies used for symptoms regardless of their underlying cause.  Having said that, it’s also true that patients with PSP may differ from others in their reactions to common medications. 

You may recall that in 2018 a brief single-author book appeared that described management of PSP for clinicians.  For better or worse, the author (that would be me) relied heavily on his own experience, his own reading of the literature and his own philosophical point of view to recommend diagnostic and therapeutic approaches.  That was great as far as it went, but it didn’t reach much of an audience.  The book’s cover price — $75 for the paperback or digital editions – deterred many, and the publisher didn’t advertise it at all.

But now we have a new resource – the CurePSP Centers of Care.  In 2017, when CurePSP organized this network of highly-qualified academic centers in the US and Canada, the mission was to have a list of geographically well-distributed centers providing first-rate care for PSP and CBD.  The network has now grown to 30 sites with plans for 10 more in the next few years.  But besides providing care, the CoC’s are also uniquely positioned to work collaboratively to improve care.  

So in 2019, I and the other three members of the CoC Steering Committee (Drs. Irene Litvan, Brent Bluett and Alexander Pantelyat) organized the other 21 (at the time) CoC site directors to write a “best practices” document on the symptomatic management of PSP and CBD.  We divided the topic into 12 section and for each, created a writing committee from the list of site directors and any institutional colleagues whom they chose to recruit as collaborators.  Each committee submitted a 2- or 3-page draft that the Steering Committee edited and stitched together into a coherent article.  We returned that to the whole group so that every co-author could have some input into the whole document and then submitted the result for publication.

We chose Frontiers in Neurology, an “open-access,” on-line journal, meaning that viewing and downloading articles does not require a subscription or a per-article fee.  Such journals cover their expenses by having advertising and by charging a fee to the authors; in our case CurePSP paid the $2,950 bill.

Here’s the link to the article and here’s the URL: https://www.frontiersin.org/articles/10.3389/fneur.2021.694872/full

Please consider sending the link (or a hard copy) to any clinician you know who takes care of people with PSP or CBD.  That’s not only neurologists, but also primary care physicians and nurse practitioners, ophthalmologists, optometrists, rehabilitation medicine specialists, neuropsychologists, physical therapists, speech/swallowing therapists, and occupational therapists.  Maybe keep a copy in your “go-bag” to provide to your doctors and nurses in a hospital or emergency room.  CurePSP will soon start a North America-wide campaign to distribute the link along with a series of videos of experts discussing and enlarging on points raised in the publication.

I think the authors of the paper did a great job, if I do say so myself.  But now begins the real work of broadcasting our advice so that clinicians can be competent and comfortable taking care of people with PSP and CBD.

Some prognostic help

Sooner or later, most patients with PSP or someone they rely on will ask the doctor, “What’s going to happen next, and when?”  Until now, that question has only been answerable by saying, “Well, the symptoms you have now will slowly get worse and you may develop some additional ones.” or “I don’t know; everyone’s different.”  If the question is, “How long will I survive?” the only available answer has been to quote the published averages for PSP, which have a wide variance. All too often, the answer is, “Don’t worry about that — just take it one day at a time.”

A long-gestating project of mine has finally seen the light of day.  It uses scores on my patients’ PSP Rating Scale (PSPRS) scores gathered from 1995 to 2016 to allow clinicians to predict how much longer it will take for a given patient to reach certain disability milestones and death.  It also proposes a new five-point clinical staging system that we used as some of the disease milestones.  It appears in the August 2020 issue of Movement Disorders Clinical Practice and is available here.

Assisting in the effort was my trusty statistician, Pam Ohman-Strickland, of the Rutgers School of Public Health.  She was also my co-author in the original validation of the PSPRS in 2007.  BTW, if you want to read that paper, here’s your chance. Since then I’ve refined the rules and instructions for administering the PSPRS and that’s available here.

Two undergrads helped out in the new project: Emily Beisser did most of the analysis for the new staging system and Francesca Elghoul helped with data wrangling.

The outcome milestones number 13 in total.  The first seven are severe difficulty with swallowing solids, swallowing liquids, speech, eye movement, general movement, balance and thinking. For each, “severe” is defined as exceeding a specific score on the relevant PSP Rating Scale item(s).  The next five are the stages on the proposed “PSP Staging System” and the last milestone is death.  

We created the five PSP stages by totaling four of the 28 items on the PSPRS: swallowing solids, swallowing liquids, gait, and the ability to return to one’s seat safely from a few steps away without using the hands.  They’re items 3, 13, 26 and 28 on the PSPRS.  The point total for those four items, each rated 0 to 4, are divided into five groups: 0 points, 1-4, 5-8, 9-12, 13-15 and the full 16.  Although this staging rubric uses only two of the many possible deficit areas in PSP, we found that the total of these those four items correlates very closely with the total PSP Rating score.  We chose swallowing and gait/balance as candidates because so many of the serious complications and disabilities of PSP lie in those areas. I’ll devote a future post to the issue of “stages” in PSP.

Tables 3 and 4 in the new paper show the meat of the matter.  You’ll see that the input data are gender, the total PSP Rating Scale score at the time of the visit, and the rate of progression to date.  The last one has to be calculated by dividing the current PSPRS score by the number of months since the onset of the first PSP symptom.

Just a quick caveat: Please don’t try this at home. Many of the exam items on the PSPRS require training and experience to administer correctly; the scale and its instructions are in technical language; and the dating of the onset of PSP symptoms may not be interpreted by the patient or family as an experienced neurologist would.

I hope that these new results, to quote myself from the paper’s introduction, “may influence decisions to retire from work, hire caregivers, alter the home environment, move to a seniors-oriented or institutional living arrangement, decide on a feeding gastrostomy and not least, prepare psychologically for advanced disability and death.”  Until we have a way to prevent or halt the progression of PSP, this will be an important part of how clinicians can help their patients.