Here’s my soapbox message today… I’m finding lots of things tedious about these CurePSP webinars. Two hours is too long, and the repeated info from Janet Edmunson and Kathy Speca is unnecessary. My “control panel” showed the attendee count varied between 160 and 190. Can’t CurePSP determine if a majority of these are repeat visitors, and skip the duplicate stuff? The Parkinson’s Disease Foundation had a great webinar last week that was one-hour long; that was perfect. The other thing that is a total waste of time is that the questions are read verbatim and with all the introductory comments. Can’t CurePSP skip this, and consolidate the questions?
And here are my comments about Dr. Hermanowicz’s presentation… I liked the fact that his message to spouse caregivers was so strong: spouse caregivers should not be caregiving 7×24. This is not a good situation for the caregiver. And I thought the format he used to describe PSP, CBD, and MSA was very effective. He seemed to be very up on the O’Sullivan and Williams research but I thought it was unfortunate that he didn’t take the opportunity to explain that there are two common forms of PSP — one with dementia and one without. Those dealing with the non-dementia form of PSP don’t appreciate hearing that a primary symptom of PSP is dementia.
Enough of my layperson opinions. What follows are the PSP-related and general notes I took from the webinar.
Background Info on Presenter – Dr. Neal Hermanowicz
He’s a Board certified neurologist with fellowship training in Movement Disorders at the Univ of Michigan. His mentors there were Drs. Anne Young, Sid Gilman, and the late Jack Penney.
Since 1999, he’s been at the University of California Irvine to establish a clinical program in Movement Disorders (in the Institute for Memory Impairments and Neurological Disorders). Clinical trials in treatment development for PD, dystonia, Huntington’s Disease and, soon, MSA.
Since 1999, he’s been the Medical Director for the Traub Center for Movement Disorders at the Eisenhower Medical Center in Rancho Mirage, CA.
He just attended the UCI atypical parkinsonism support group meeting on 10/7..
Presentation by Dr. Neal Hermanowicz
Topic: Fundamentals & Diagnosis of PSP, CBD, MSA, and Related Disorders
These three disorders have common features — slowness and stiffness of movement (Parkinsonism)
Similar ages of onset
Progressive (and gradual)
Unknown cause (which is not unusual in neurology)
Uncommon (so it’s hard for clinicians to make a diagnosis)
No specific treatment for the diseases
The diagnoses of these three disorders is established by clinical assessment, ie, history and physical examination.
No tests confirm the diagnosis.
There is always some level of uncertainty, even among experienced clinicians.
Patient #1 – PSP
68 year old man referred for possible PD. He has been falling recurrently and without warning for the past year and a half. Gravelly voice (noticed by wife). He coughs when eating and drinking. He is losing weight. He complains of vision problems. He acknowledges that he is slower in his movement and his neck feels stiff.
History of PSP: Dr. Richardson, in Toronto, saw a patient in 1955. He called the disease by the name “progressive supranuclear palsy.” Steele, Richardson, and Olszewski published on PSP in 1964. This 1964 included brain pathology. Seemed reminiscent of post-encephalitic parkinsonism.
Prevalence is 1 to 6 in 100,000
Jankovic: of 100 PD patients he sees in his clinic, actually 5 have PSP [Robin’s note: not sure I got that right] Symptom onset average age 63.5
Diagnosis average age 66.5
More men than women affected at a ratio of around 2:1. (We don’t know why. This is similar to PD.)
Rarely runs in families
PSP Initial Symptoms:
Unsteady gait or falls: unpredictable falls
PSP Diagnosis: consolidated several criteria here, including the SPSP-NINDS criteria
Onset 40 or older, and gradually progressive
Impaired balance and falls in the first year
Eye movement abnormalities (difficulty looking downward or side to side is characteristic of PSP)
Early speech and swallowing difficulty
Symmetric (both right and left side of body) slowness and stiffness
Cognitive impairment (different from the type we see in Alzheimer’s)
Difficulty looking upward is very common, even in normal aging
PD is asymmetric
PSP Pathology: described by Richardson; brain cell loss in characteristic areas; neurofibrillary tangles within brain cells (tau); locations correlate to symptoms and exam
The cause is unknown. Research on environmental and genetic factors is currently underway, enrolling at 10 sites within the US. This is not a treatment study but a gathering-of-information study.
PSP Prognosis: (according to O’Sullivan et al 2008)
Measure of time from onset to milestones:
* Frequent falls: 3.9 +/- 2.5 years
* Wheelchair: 6.4 +/- 2.7 years
* Severe swallowing difficulties: 6.4 +/- 2.4 years
* Disease duration: 8.0 +/- 4.1 years
These are averages, statistics. More severely impaired people may be seen at these centers that were part of the study.
Progression rate is variable from one person to another.
http://brain.oxfordjournals.org/cgi/con … 131/5/1362 ]
PSP Treatment: (from Burn & Warren 2005)
Nothing specific for the disease process itself
Involuntary eyelid closure (blepharospasm or apraxia of eyelid opening) can be treated with botulinum toxin injections
Double vision can be treated with prisms in eyeglass lenses
Dry eyes can be treated with artificial tears
Speech and swallow problems should be evaluated by a speech pathologist
Excessive saliva can be treated with atropine drops or botulinum toxin injections into salivary glands
Cognitive symptoms treatment is under investigation. Mixed reports on Aricept (donepezil). Exelon (rivastigmine) is being looked at and is potentially beneficial. He tends not to use Alzheimer’s medications.
Mobility/fall risk reduction through physical therapy.
PD medications such as levodopa and amantadine are reasonable to try in PSP. These medications have the potential for unpleasant side effects.
Brain surgery: not yet beneficial; under investigation
Support: support groups and websites give people a feeling of empowerment
Other related brain disorders include:
Dementia with Lewy Bodies
Questions and Answers: (All answers were given Dr. Hermanowicz, unless indicated otherwise.)
Three Disorders (note: though some of the questions are specific to a disorder, the answers are more general)
Q: Is there anything new on stem cell therapy?
A: Stem cell therapy presents great hope to lots of people. Transplantation is further away. Another way stem cell therapy can be helpful is to model the disease. UCI is doing this in Huntington’s Disease.
Stem cell treatment is being tried in MSA-C (“the ataxia type”) in South Korea. These stem cells are being injected into veins, not the brain. But the stem cells find their way to the brain.
Q: What is the relationship between alpha-synuclein and tau, and the effect on them by hsp70?
A: Alpha-synuclein is causing abnormal clumps in MSA while tau is causing abnormal clumps in PSP and CBD. hsp70 is a protein that might help reduce this clumping. It has been looked at in the lab. This may perhaps open up a new avenue of treatment, or something like hsp70. Not yet in clinical trials.[Robin’s note: You can read the research findings on Hsp70 here:
Personally, I think this research will only be of interest to those dealing with PSP and CBD, as the discovery is about tau. And the research was in mice, so we are years away from any clinical trials.]
Q: My wife, with PSP, had an MRI that was inconclusive. What part do MRIs play in diagnosing PSP?
A: A brain MRI is not helpful in diagnosing any of these three disorders. No imaging study is helpful. There are sometimes hints or clues. I would never make a conclusive diagnosis from an imaging study.
Q: My husband, with PSP, fell and fractured his hip. With his impulsivity, how can we help him to understand the safety precautions he must now take to prevent further falls?
A: No medication can help with impulsivity. This requires ongoing discussion and reinforcement with the person with PSP. In some cases, a caregiver is needed 24×7. It doesn’t always need to be the spouse. Perhaps it can be a hired caregiver.
The risk of a fracture or subdural hematoma is always present with falls.
Q: My mother, with PSP, lives in Montana and has never seen a movement disorder specialist. Should we travel to see the leading experts in the US?
A: One does want to be certain about the diagnosis. These disorders are uncommon. If an MD or NP has never seen one of these disorders before, it’s hard to recognize them. I hate to encourage people to travel great distances but it might be at least worth at least one visit with someone in the western US with movement disorder training. Try Boise, ID, or Seattle, WA. This is a problem. It can be gratifying for people to get a diagnosis but it can be frustrating as there’s very little for treatment. Telemedicine is being developed as a technology for diagnosis. I think this could be diagnosed remotely via a camera.
Q: What medicine is the best to treat muscle stiffness?
A: Levodopa is a reasonable medication to try. Baclofen is another possible choice. If it’s localized, injection with botulinum toxin might be helpful.
Q: My wife, with PSP, has a problem with bed wetting. She is in diapers, doesn’t drink liquid after 7pm, and takes medication. What else can I do?
A: Elevating the head of the bed by 30 degrees can reduce urinary urgency. She should be seen by gynecologist or urologist so it’s clear what is causing the problem, though it’s probably related to PSP. Be sure she doesn’t have a UTI. Void the bladder before going to bed. Sometimes incontinence briefs are the best resolution. Consider a bed-side commode or bed-side urinal (for men).
Q: My relative has PSP. Does CoQ10 help? What is it used for?
A: CoQ10 tries to encourage brain cells to get more energy for their operation. UC Irvine is participating in a couple of CoQ10 trials in PD and Huntington’s Disease. But the jury is still out on CoQ10. It’s not known to be effective but it’s not known to be harmful either. I don’t discourage people from taking it.
Q: Is lithium an effective treatment?
A: Lithium is being studied, but it hasn’t been proven to be effective. This medication has potential toxicities. It can have neurological side effects. It has to be monitored closely and used with caution. It is primarily for mood disorders, bipolar disorders.[Robin’s note: The NIH-funded trial into the safety of lithium in PSP and CBD has ended. Many patients reported unacceptable side effects. To my knowledge, lithium has not been studied in MSA.]
Q: I have been diagnosed with PSP. Can I remain at home (my preference) or must I have to an assisted living facility. Money is not an issue.
A: For many people, money is an issue. Insurance doesn’t cover facility-based care. If you can financially sustain it, the best care is at home. But I discourage the spouse to be the 7×24 caregiver. There is burnout associated with that.
Q: My husband has early-stage PSP, with his full mental capacities. We’ve been discussing the pros and cons of a feeding tube. He insists he doesn’t want one. I and my children would like him to have one. What should we do?
A: His wishes are clear. I do not encourage him to get a feeding tube.
Q: My MD first said I had CBD. Then the diagnosis changed to “slow moving PD.” What is this?
A: I don’t know. This is not a conventional term.
Q: My husband has MSA. There is some 1990 research on the effect of the iron binding protein ferritin. Is there any current info on this?
A: Iron deposits have been associated with neurodegenerative diseases. There’s lots of interest in this topic though nothing has panned out.
Q: I’m a Korean war vet. Would toxin exposure have caused PSP?
Carbon monoxide and manganese exposure can lead to PD. But the great majority of PD, PSP, and MSA cases have no toxin identified yet.
Q: Should levodopa be taken 1 hour before or 2 hours after a meal rather than with meals? If a PSP sufferer is not responsive to levodopa, should the dosage be increased to 2000mg/day?
A: I hate to have people orchestrating medication in relationship with their meals because it’s upsetting to people’s lives.
I don’t usually take people up to 2000mg/day. That’s a huge dose. I might take people up to 600mg/day, perhaps 800mg/day.
Q: My husband has been told by different neurologists that he has MSA or PD. Do I really have to wait for an autopsy report to find out what he has?
A: Yes. Even PD, a far more common disorder, can be hard to diagnose.
If it’s Parkinson’s-like, we use PD medications — whether it’s MSA or PD. No definitive test or clinical exam during life is available.
Q: My mom has one of these disorders and has a problem accepting it. What can I do to make my mother accept her condition?
A: Denial isn’t necessarily a bad thing. There’s nothing specific that can be done to force someone to accept her condition. Perhaps this will change over time.
Q: I have had PSP since December 2008. I have impaired voice and minor walking difficulty. How long do you guess that I have to live?
A: I don’t know. People ask me this. The data says 8 years +/- 4.1 years, but the data may be biased towards severe cases.
Q: My relative has PSP. Why is there such a difference in survival time for different people?
A: According to one study, the average is 8 years, though there is some variability. We don’t know why there is such variability. Perhaps it is connected to why people get the disease in the first place.
Q: What progress has there been in finding out the causes of PSP?
A: This is the thrust of the national study being organized by Dr. Irene Litvan. One of the participating sites is UCLA. This study is underway presently. In terms of progress, there hasn’t been enough. In terms of research, it’s ongoing.
Q: Is there any progress in finding a treatment for PSP?
A: There has been some but not enough. There are studies underway to identify the cause and brain mechanisms involved in PSP. This will lead to better understanding and better treatments.
Q: My husband, with PSP, is currently taking Sinemet, Namenda, and nortriptyline. Are there any new medications effective for treating PSP?
A: Nothing new has been proven to be effective.
Q: My mother died of PSP. What is the incidence of heredity in PSP?
A: I’m guessing that it’s less than 5% of all cases. It’s rare. In some families there is PSP showing up from generation to generation.
Q: My mother passed away due to PSP. My brother has multiple sclerosis. Is there a connection between PSP and MS?
A: Not that we know of. MS is an autoimmune disorder. PSP is not an autoimmune disorder, though there is an immune system component.
Q: My mother has PSP. She cannot walk with assistance. How does the disease progress in later stages?
A: More advanced stages of PSP is “more of the same.” More impairment of speech, more problems walking, more swallowing problems, more cognitive problems. Aspiration can occur with swallowing problems.
Q: I am a urologist (John B in Houston). One of my patients has PSP. What are the urinary problems in PSP?
A: The overwhelming majority of people with PSP have urinary problems (urgency, frequency or incontinence).
Q: I’ve been diagnosed with cerebellar ataxia but it sounds a lot like PSP. What’s the difference?
A: Cerebellar ataxia = sense of incoordination; feeling like you are drunk; irregular speech; poor coordination of walking, arms, and legs
PSP = stiffness of trunk and neck; problems with balance and falling; not the same type of incoordination
They look different.
Q: Can I get a copy of today’s webinar?
Answer by Larry Schenker: A summary will be posted to forum.psp.org. Get a copy of the presentation by emailing Kate DeSantis, [email protected].
Q: What is the latest clinical research that is being done for PSP?
A: Dr. Yvette Bordelon is doing a future webinar on PSP, CBD, and MSA research. I will defer to her.
10/22 – Robert Hutchman – Interventions
11/5 – Yvette Bordelon – Latest Research
11/19 – Lawrence Golbe – Research for Dummies
12/3 – Jerome Lisk and a panel of movement disorder specialists