Tag Archives: parkinsons-disease

Jesse Jackson

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The Rev. Jesse L. Jackson, Sr., the civil rights leader and political activist, has announced that his 2017 diagnosis of Parkinson’s disease has been changed to PSP.

I have no inside information on the details of Rev. Jackson’s condition, but I can tell you that this is a common scenario for people with the second-most-common type of PSP, called PSP-Parkinsonism (PSP-P).  It accounts for about 15% to 35% of all PSP, depending on the source of the data, and starts out looking like Parkinson’s disease (PD), typically with asymmetric stiffness of the limbs and often a tremor, without much cognitive, eye movement or balance difficulty.   The symptoms, like those of PD, usually respond to levodopa, though not quite as well or for as many years. 

PSP-P progresses at a rate typical for PD, not the faster rate for most types of PSP.  The average lifespan of PSP-Richardson syndrome (PSP-RS), which accounts for about 45% to 55% of PSP, is about 6 years, while for PSP-P, it’s 9 years and for PSP overall, 7 years.  (The lifespan of people with PD receiving modern treatment averages about 16 years, but many neurologists today don’t know that before levodopa became available in 1970, it was about 9 years, like PSP-P.)

The typical journey of someone with PSP-P starts with a satisfactory response to levodopa.  But the dosage requirement increases more rapidly and balance problems develop sooner than in PD.  After a few years, the neurologist may suspect one of the “atypical Parkinsonian disorders,” examine the patient more closely than at the previous follow-up visits, find the eye movement problem and frontal cognitive issues of PSP, and change the diagnosis.  Often, this re-evaluation is performed by a second neurologist whose opinion was sought by the patient or family member worried about the atypical symptom course and unsatisfactory levodopa response.

So, if Rev. Jackson does have PSP-P, he would now be at about the average (i.e., the median) survival duration.  Of course, about half of all people with PSP-P survive past the median, some for as long as a total of 20 years or even more.

A scientific comment: Keep in mind that PSP-P wasn’t described in the medical literature until 2005 and formal criteria to distinguish among the various (now 10) different PSP subtypes didn’t appear until 2017.  Before then, most people with PSP-P would not have fulfilled the existing formal criteria for PSP, which were published back in 1996.  Therefore, pre-2017 statistics on the prevalence or lifespan of people with PSP refer mostly to PSP-RS, which is the name given in 2005 to what was defined as “PSP” by the 1996 criteria.

Another scientific comment: PSP-RS, PSP-P and the other 8 subtypes all have the same set of microscopical changes in the brain, featuring neurofibrillary tangles of tau protein and tufted astrocytes.  It’s just the sets of locations around the brain that differ to some extent.  We don’t yet know what causes this “cell-type specificity,” but the best theory is that it’s slight differences in the mis-folding pattern of tau, caused by slightly different locations of abnormal attachment of phosphate groups on the protein.

And finally, an editorial comment: Almost all clinical drug trials in PSP only accept participants with PSP-RS.  There are several good reasons for that, but that policy excludes half of all those with PSP, raising the theoretical possibility that any resulting treatment may work only in that sub-type.  I’ll discuss potential solutions in a future post.

Meanwhile, my deepest gratitude to Rev. Jackson for sacrificing some degree of personal medical privacy in the service of increasing public awareness of PSP and my best wishes to him in the journey forward.

Yes, Congress can accomplish something

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Here’s a great step forward: The Energy and Commerce Committee of the US House of Representatives has just approved the “National Plan to End Parkinson’s Act.”  Thanks in part to advocacy by CurePSP and other organizations devoted to the atypical Parkinsonisms, the bill includes not only PD but also PSP, multiple system atrophy, Lewy body dementia, corticobasal degeneration and Parkinson’s dementia. 

For the remaining required approvals, the bill will now proceed to the full House itself, then the Senate, then the President. (I recited that route for my international readers and for my US readers who doodled through civics class.)  Crucially, the bill was passed with full bipartisan support in the committee, which bodes well for its chances the rest of the way.

Here’s video of the committee’s session.

The bill directs the Department of Health and Human Services to create an advisory commission with representation from all relevant Federal agencies and some advocacy and research organizations as well as patients and caregivers.  Each year, the commission would assess the state of research and clinical care and formulate recommendation on how the various relevant Federal agencies could formulate and coordinate further research plans.  It would also interact with similar organizations internationally. A similar bill for Alzheimer’s disease was enacted in 2011 and has been working successfully by all accounts.

The commission would recommend spending levels for the Federal Government to advance these efforts, but the bill provides no funding for the work of the commission itself.  That would have to be absorbed by the existing budget of the Department of HHS.  (This is standard practice when Congress is interested in a specific medical cause.) 

The bill was first taken up by the committee in March, nine months ago.  One of its major advocates has been Congresswoman Jennifer Wexton of Virginia, who was diagnosed with PSP herself last summer and has been working with CurePSP and others to improve awareness of PSP nationally and to raise funding for research.  Here’s a press release from her office.   The lead sponsor of the bill is Congressman Gus Bilirakis of Florida, who has three close relatives with PD, but 167 other House members signed on as co-sponsors.  The Michael J. Fox Foundation has been working tirelessly for the bill.

I know you’ve been waiting for my editorial commentary.  Here it is:  This is great publicity for PSP, and it sure needs it. 

Congresswoman Wexton is the highest-profile celebrity with PSP since Dudley Moore, the British-American comic actor best known for the movies “10” and “Arthur,” announced his diagnosis of PSP in 1998.  His friends organized a star-studded benefit at Carnegie Hall in New York that raised $50,000 for CurePSP but he declined to become an activist in other ways.  (I was his neurologist, and he told me, “I’ll help out, but don’t want to be the poster child for PSP.”) Linda Ronstadt, the popular singer and Rock and Roll Hall of Fame member, announced in 2019 that her longstanding diagnosis of Parkinson’s had been changed to PSP.  She has not yet supported PSP-related activities of which I’m aware.  A few other less-famous celebrities with PSP have advanced awareness and fundraising, and we’re grateful to them and their families.  But when “progressive supranuclear palsy” is mentioned on the floor of the US House of Representatives and included in press releases, that’s rare and valuable publicity.