More short stuff

Three more brief items:


Another case of paraneoplastic PSP has been reported, making 7 in the world’s literature.  This was a 62-year old woman with ductal breast cancer whose PSP-like features evolved over only 4 months to an advanced state of disability.  The brain MRI and spinal fluid were normal.  None of the known paraneoplastic antibodies were present in her blood.  A PET scan using fluorodopa was negative, a result expected with non-degenerative forms of parkinsonism.  Treatment with steroids and other immunomodulatory agents helped dramatically, restoring an independent gait and a “good quality of life” until the breast cancer became metastatic.  This case was reported in the Annals of Indian Academy of Neurology by Dr. Ajith Cherian and colleagues. The take-home: When any neurodegenerative disease, including a PSP-like syndrome, evolves rapidly to disability over a period of months rather than years, a strong possibility is a paraneoplastic syndrome.  That’s where the immune system’s fight against a tumor produces antibodies that also attack normal components of other organs such as the brain.  Not only may the neurological symptoms improve with immunoregulatory treatment, but if those symptoms appear before the tumor has been suspected, the workup could include a thorough search for a small tumor, removal of which could be lifesaving.  (Note: The information from the PET scan could have been obtained nearly as well from a dopamine transporter scan, which is far more widely available and is covered by insurance.)


Depression occurs in about 60% of people with PSP, according to a published review.    Now, an article in Journal of Neurology reports MRI scans in 40 patients with PSP, comparing the 21 with depression to the 19 without.  The research group works at the University of Bari, in Tricase, at the tip of the heel of Italy’s boot, and was led by Dr. Daniele Urso, a well-published expert in neuroimaging of neurodegenerative disease. In patients with PSP and depression, they found more thinning of the cerebral cortex in the temporal, parietal and occipital lobes, moderately worse on the right than the left.  (In the linked image, the colored areas are those where atrophy was greater in patients with depression than in patients without. The left cerebral hemisphere is shown on the left and the right on the right. The upper two images are the medial (inner) surface and the lower two the lateral surface.) This confirms once again that depression is part of the disease process in PSP and not (or not only) a normal psychological reaction to the overall disability.  The right/left asymmetry shows that it wasn’t just that the overall PSP was more severe in the patients with depression, as the cortical atrophy of PSP is generally symmetric. 


A few years ago, CurePSP created a network of 25 academic centers in the US and Canada with special expertise in care of patients with PSP and CBD, calling it the “CurePSP Centers of Care.”  I’ve been one of its organizers and leaders.  Last year, we published a “best-practices” article on medical management of the disorders.  This year, we have expanded to 28 centers, including 2 in Canada.  To keep things moving and accountable, we have doubled the size of our Steering Committee to include 4 Center directors and 4 representatives of the CurePSP Board of Directors and staff.  For the first time, we’ve drafted a list of projects designed to improve clinical care and to provide clinicians of many kinds with education and awareness of PSP and CBD (and for some centers, MSA as well).  Also for the first time, CurePSP is providing the Centers some modest financial support for expenses related to delivering first-rate clinical care. I’ll let you know how it’s going. 

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:

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.