Intro for Patients and Caregivers

Welcome to PSP Blog and Registry!

Non-Technical Summary

If you’re having trouble reading this, try enlarging the type.  Hold down “Ctrl” and press “+”.

Progressive supranuclear palsy is a rare disorder where cells in some areas of the brain gradually sicken and die off, causing the individual great difficulty in movement and, later, in thinking.  Its cause is unknown, but research is active.  I’m a medical school neurologist who has been doing research in PSP for nearly 30 years.  This blog is both for professionals and for patients with PSP and their families.  An important feature is the email address registry, which will allow me to bring special news to your attention quickly, including opportunities to participate in research.

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What Is PSP?

Progressive supranuclear palsy is a “neurodegenerative disease,” meaning that it starts gradually and worsens slowly and its cause is a poorly-understood loss of brain cells.  PSP is similar to Parkinson’s disease in its general slowness, poor balance and slurred speech.  The two most common neurodegenerative diseases are Alzheimer’s disease and Parkinson’s disease, each of which has things in common with PSP and some important differences.  For more info on PSP, click here.

Unlike Parkinson’s, PSP is rare, with 5-6 cases per 100,000 population, of whom only 1 or 2 carry the correct diagnosis at the time.  This compares to 150-200 per 100,000 for Parkinson’s.  Relative to Parkinson’s, PSP’s response to medication is poor to fair, it rarely has tremor, it commonly includes important loss of thinking ability and it almost always causes difficulty moving the eyes.  (The “palsy” part refers to this last feature.)   PSP starts at age 63, on average, compared to 59 for Parkinson’s.  The most common first symptom is unexplained falls or personality changes.

OK, So What’s the Good News?

The good news is that there’s lots of research in progress on PSP.  Some more good news is that while PSP outwardly looks very different from Alzheimer’s disease, the abnormality in the brain cells in PSP is very similar to one of the abnormalities in Alzheimer’s.  Many scientists believe that the route to a cure or prevention for Alzheimer’s goes through PSP.  So PSP commands far more attention from researchers and the biotech and pharmaceutical industries than its rarity would suggest.

This Blog’s Relationship to CurePSP

Even more good news is that you can find plenty of reliable information on PSP at www.psp.org, the website of “CurePSP” (the Foundation for PSP | CBD and Related Brain Diseases).  You might like to start directly on their Education page.

CurePSP also offers educational videos, Webinars, pamphlets, meetings and symposia and a Web forum for patients and caregivers.

Full disclosure: I have volunteered with CurePSP for many years and serve as its Director of Research and Clinical Affairs.  I wrote many of the things on its Website.  Part of my reason for starting PSP Blog is to help CurePSP do its job.

Who Am I? 

I’m a neurology professor at Rutgers Robert Wood Johnson Medical School in New Brunswick, New Jersey.   I’ve been doing research in PSP since the 1980s and I have a large referral practice of people with PSP and related movement disorders.

What This Blog Hopes to Accomplish

PSP Blog will keep you updated on what I see as important and interesting things related to PSP.  Some posts will discuss research findings or new treatments.  Others will announce research projects starting to recruit participants.  Such posts will include links for people with PSP or their caregivers to learn more and possibly to volunteer.  There may be news about PSP clinical centers, opinions of medical products, and interesting scientific ideas in non-technical language.

Some posts on PSP Blog will be in non-technical language and others will be written for professionals.  The terms “technical” or “non-technical” will appear in the keywords for each post.  But you don’t have to upload a diploma in neuroscience to gain access to the technical-language stuff.  Have a go at it, but keep in mind that some technical terms may look like ordinary English words but mean something very different.

The blog’s Comments function is not for medical advice.  If you’d like to participate in a forum with other patients or caregivers, CurePSP has a good one.  Click here.

I’ve always believed that I learn something new from each of my patients, and the power of the Web can magnify that.  I also hope that the exchange of ideas will teach things to caregivers, researchers, medical inventors and pharmaceutical executives as well as to people with PSP themselves.

The Optional Email Registry

Finally, PSP Blog allows you to register for an email list: a registry.  That would allow me to send you brief notices about new blog posts on PSP Blog and news flashes about clinical trials and other appeals for volunteers.  I am the only person who can send emails using the list.  I am sworn by a very serious committee at Rutgers University responsible for such things (ominously called the “Institutional Review Board”) never to reveal your email or any other identifying information to anyone.  Nor can I allow anyone else access to the list.  In fact, the software doesn’t allow me to do so.

The email Registry offers two options at the time you register.  One would accept all of my emails, which would be at most once a week.  The other would accept only emails that offer opportunities to volunteer in clinical trials.

Your Privacy

Another note about privacy:  This blog’s email Registry is maintained not by the company hosting the blog itself (WordPress), but my own medical school.  The registration process does not ask for your name or any information other than an email address, and you don’t have to register at all to use the Blog.  That address would be protected with the same rules of confidentiality required by Federal law for our medical school’s own patients’ medical records.  No blog hosting company offers that level of confidentiality, which is why I’m using my med school’s facilities for the email list.  Keep in mind that if you somehow provide your email address or other information to WordPress, it may not be kept confidential.

Your Feedback

Comments feature to provide feedback on my posts, to comment on on others’ comments, to run your own ideas up the flagpole, and to suggest how I can improve the blog.

20 thoughts on “Intro for Patients and Caregivers

  1. Hello. I believe my mother and two of her sisters (and nieces now too) have this disease. In addition to common bloodline (genetics?) they all grew up in Arkansas in their early years and started showing symptoms around 62-63 yrs of age. The presentations are strikingly similar and one of the biggest “aha” moments for me was when you mention the behavioural changes. I have read everything I could find regarding PSP and other neurodegenerative syndromes etc., and I am convinced more than ever this is the diagnosis we have been looking for (going on 12 years or so now). The age of onset, balance issues, choking, difficulty swallowing and slurred speech, short-term memory loss, behavioural changes, difficulty reading or writing…but no tremors to speak of, it’s all there and she has been to anyone who will take an interest, but now we are expat Americans living in New Zealand so access to resources can be a challenge. Looking forward to more information.

    • There is a genetic component to the cause of PSP, but it’s not strong enough to make the disease appear in multiple family members except in a few rare cases. A person may have to be unlucky enough to have the abnormal version of multiple genes plus a toxic exposure or two to develop PSP, and the chance that more than one person in a family has all those things is low.

    • No connection is known, but this provides a genetic clue. Assuming that both PSP (in this family) and hemochromatosis (ditto) are each caused by a different single gene mutation, those two genes could be close together on the same chromosome, both victims of a deletion or transposition of a short stretch of DNA. I’ll bring your post to the attention of a geneticist colleague.

      • My mother has PSP with non fluent aphasia and unlike most people with PSP her first symptom was with her speech. At this time she is unable to talk and her writing is being affected. She is able to walk and is generally able to take care of herself, but communication is rapidly disappearing. Her ability to process what she reads is now being affected as well. Her eyes are also affected. So far none of the progressions of this disease follow my mother’s pattern of decline. Just wanted to put my 2 cents worth in. It is heartbreaking to watch her deteriorate as it is for most people, but on top of that her career field was in editing….

    • Hi,

      My dad is 73 and was diagnosed with PSP this past February, though his symptoms started about 3 years ago with a foggy head. He has been declining rapidly in the past few months.

      We went through much testing before finally landing at the diagnosis. His slow eye movement is what finally gave it away. But one early test that was run on him was a heavy metal test, which showed a dangerously high level of lead in his body. The weird thing about it was that he had no exposure to lead…..or so we thought. The only exposure he had was in the hair dye he used, Grecian Formula. Which he had used for years.
      Is it possible that his PSP could be a result of a toxic substance like lead in his system, from hair dye? I know lead in hair dye is banned in other countries. I’d love to know if anyone else suffering from PSP also used Grecian Formula.

      This is the saddest disease, today my father said he is disappearing. I’m not sure he meant it in the sense I understood it, but it’s very true. His loud, outgoing, smart, friendly personality is just a shadow of what it once was.

      My best,
      Theresa

      • Grecian Formula’s active ingredient is lead acetate, which would only get into the body if there’s some break in the skin or if the hands aren’t washed well after applying the dye. Of course, either of these could have been the case for your father. The relationship of PSP and hair dye has never been studied to my knowledge and I’m not aware of any other reports of lead being associated with PSP. Furthermore, the type of brain damage that lead can do does not look that the damage of PSP. However, the cluster of PSP in northern France may well be the result of multiple metals that we know were processed in that local area over many years. Perhaps lead was one of them and perhaps it can, in combination with other metals, cause PSP. I’m working right now on a project to understand the cause of the cluster and will definitely include lead in the inquiry. Thank you for bringing this to my attention!

  2. My Husband has this awful psp.it’s so hard to watch him struggle now his throat is bothering him I want to breath for him.we have hospice in now they don’t know as much as we do cause they have never had anyone with this diease.what can I do to help his throat

    • I’m sorry to hear about your husband’s illness. You don’t provide enough information for me to give advice, and in any event, I can’t give medical advice to patients I’ve never met or examined. But your husband’s doctor or hospice director is welcome to email his/her contact information to me at (golbe@rutgers.edu).

  3. I’ve been searching for a current blog site. This is great. My husband has had PSP going on four years but , of course like so many, I saw something wasn’t right about three years before that.
    I just want to say that his grandfather had a neurological problem, his father had Alzheimer’s and his mother had something that affected her balance. I definitely think Tau protein is genetic.
    My husband is now 68.
    Continue the good work.

    • It’s a lot of work to write a blog and I’m very glad to get positive feedback from you and others. Thank you.
      You could well be right that there’s a genetic condition running in your husband’s family, but you can’t go so far as to say that tau protein (abnormalities) are genetic in general. At least 98% of people with PSP have no known relative with PSP and the studies attempting to find a greater prevalence of Alzheimer’s in the families of people with PSP have been contradictory. So let’s not frighten the families of people with PSP unnecessarily.
      You might want to speak with a genetics counselor to see if any of the available genetic tests might be appropriate for your husband to have.

    • The average survival of people with PSP is 7.4 years from the onset of symptoms. For those with the (more common) Richardson’s syndrome form, it’s about 6 years and for the (less common) PSP-parkinsonism form, it’s about 10 years. Keep in mind that these are only averages. Close attention to avoiding complications like falls and aspiration of food and drink will tend to land you on the good side of the average. Then there’s the possibility that one or more of the new “neuroprotective” treatments being tested now will work. That could improve these average figures significantly.

  4. I am happy to have found this blog. I love the curepsp website! I printed off a big chunk of info from that site when my mother was given the diagnosis 4 years ago. She had symptoms that were most closely identifiable as Parkinson’s and yet the dr was never satisfied with that Dx as she never had resting tremors. She has had motor symptoms (balance,falling) for at least 10 years before diagnosis with PSP. Her diagnosis came after she suddenly had speech difficulties and they found her abnormal eye movement (nystagmus I think). Her lifetime of singing opera helped delay some of her vocal symptoms they (university of Utah) said. One thing she does not appear to have is major personality changes. She is still very loving and agreeable, never combative or difficult. The only thing she ever had in that regard was a lack of ability to understand her own decreasing abilities. She has been on hospice for 2.5 years now. I haven’t brought her to see the docs at the U in a few years. Do you think it would be worthwhile? It is very hard to take her places due to her almost complete immobility. Do all PSP pts have the personality changes?
    Thank you,

    • I can’t give individual, specific medical advice here, but in general, when you have a rare disease, it’s good to touch base with an academic specialist regularly. Fourteen years is a very long time to have PSP, so maybe the diagnosis needs to be reconsidered, and general neurologists are not usually trained for that.
      As for whether all people with PSP develop “personality” change, the answer is no, but almost all do eventually develop some sort of behavioral and/or cognitive change. Close family members are often oblivious to such changes because they see the patient every day and may have “redefined normal.”

    • The classical form of PSP that was described in 1963 is now called Richardson’s syndrome and accounts for about half of all autopsy-proven cases. Next, with about 30% of the total, is PSP-parkinsonism, which starts out looking more like Parkinson’s disease and includes more tremor, levodopa responsiveness and asymmetry; and less dementia, falls, gaze palsy and swallowing problems. PSP-P has a more prolonged course than PSP-RS. The others each account for less than 5%. The most common of these rare presentations is PSP-corticobasal syndrome. The much rarer ones are PSP-pure akinesia with gait freezing, PSP-postural instability, PSP-behavioral variant frontotemporal dementia, PSP- promary progressive aphasia, PSP-progressive apraxia of speech and PSP-cerebellar. All have the same appearance under the microscope, but in slightly different parts of the brain.

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