3 Forms of PSP – RS, PSP-P, and PPFG

The Society for PSP had an International PSP Resarch Symposium on 11/17/05, right after the annual meeting of the Society for Neuroscience in Washington, DC.  The latest issues of the PSP Advocate has an interesting series of articles on some of the presentations made by scientists at the Symposium.

One scientific abstract I found worthwhile was written by David Williams, MBBS, FRACP, of the Queen Square Brain Bank (QSBB), the Sara Koe PSP Research Center, etc., London.

Dr. Williams said that clinicians can use some key differences between PD and some types of PSP in differentiating PD from some types of PSP in a clinical setting.  Specifically, the “timing of falls, the presence of visual hallucinations and testing of smell should be helpful in distinguishing PSP-P and PPFG from PD.”  (PD patients start falling later, nearly half have sustained visual hallucinations, and a decreased sense of smell.)

Also, he identified three distinct types of PSP:

  • Richardson’s Syndrome (RS):  early onset of postural instability and falls, gaze difficulties, and dementia.  54% of cases in the study.
  • PSP-parkinsonism (PSP-P):  non-symmetrical symptom onset, tremor, and a moderate initial therapeutic response to levodopa.  32% of cases in the study.  Obviously this type is often confused with Parkinson’s Disease.
  • Primary progressive freezing gait (PPFG):  early onset of gait freezing without other neurological signs. 4% of cases in the study.

Here are some excerpts from the scientific abstract about survival time and other symptoms:

“…We have recently identified two apparently distinct clinical types of PSP by analyzing clinical case notes of patients with a pathological diagnosis, archived at the Queen Square Brain Bank, London. We have named these two clinical types: Richardson’s syndrome (RS) and PSP-Parkinsonism (P).

Disease duration in RS was shorter (average 5.9 vs. 9.1 years), falls occurred earlier and age at death was younger (72.1 vs. 75.5 years) than in PSP-P. …

Primary progressive freezing gait (PPFG) … had longer disease duration (average 12.8 years) and late onset of gaze palsy and dementia compared with RS.

Other clinical analysis at QSBB has shown that sustained visual hallucinations are exceedingly rare in PSP (3%), but relatively common in Parkinson’s disease (PD, 49%). A decreased sense of smell is usual in Parkinson’s disease but not common in PSP. The time from symptom onset to first fall is early in PSP: 57% of patients with PSP fall within two years, 
compared to 6% with PD. The timing of falls, the presence of visual hallucinations and testing of smell should be helpful in distinguishing PSP-P and PPFG from Parkinson’s disease in the clinic…”

You can find this scientific abstract and the related lay abstract along with all the other articles online at:




Long Term Care Insurance- Some Useful Pubs/Resources

For those of you thinking about getting long-term care insurance…..

Over the last week, I’ve been looking into LTC insurance for my husband and myself. Local support group member Karen D. gave me some good materials on the subject, and I’ve found a few of my own. I thought I would pass on a list of the publications I recommend reviewing and resources available, in case some of you are thinking about getting this type of insurance:

“Taking Care of Tomorrow: A Consumer’s Guide to Long-Term Care,” CA Dept of Aging. This excellent booklet gives lots of useful statistics about long term care generally. Their website says: “To receive a free copy of this publication, you may contact Steve Miars, Department of Aging, at (916) 323-4356.”

The CA Dept of Aging brochure talks about the “California Partnership for Long-Term Care,” which is a program between CA and some insurance companies. The program takes into account MediCal. There’s good info on this program online at www.dhs.ca.gov/cpltc/. That website also lists the six insurance companies currently participating in the program. Note that not all policies from a participating insurer are CA Partnership for LTC policies.

“Do You Need Long-Term-Care Insurance,” Consumer Reports, Nov 2003, page 20+. This is an excellent article. One of the items Consumer Reports tells you to do is to go to www.ambest.com (or another financial rating company) to learn the financial strength of any insurance company you are considering.

“LTC Insurance May Not Be Worth Your Money,” Wall Street Journal, 10/9/03. This short article talks about the Consumer Reports study. It also provides the insurance industry’s response to the study. You can purchase this article for $5 from www.wsj.com.

“Guide to Long-Term Care Insurance,” America’s Health Insurance Plans, last revised July ’04. This excellent brochure describes how these policies work. There’s a very helpful “Long-term care policy checklist” on pages 14-15. You can read this publication online at: ahip.org/content/default.aspx?bc=41|329|450.

“A Shopper’s Guide to Long-Term Care Insurance,” National Association of Insurance Commissioners, Oct ’02. You can order a free copy by calling 816/842-3600, asking for “Publications,” and leaving your name and address in a phone message. You can also order a free copy online at: external-apps.naic.org/insprod/Consumer_info.jsp.

“The Lowdown on Long-Term Care Insurance,” Elder Law News, Summer 2003. You can read this short publication online at: www.elderlawanswers.com/ElderLawNews/Summer-2003.pdf.

The consumer advocate Clark Howard, who has a show on Cox Radio, also has a website providing his advice on a range of subjects. The LTC insurance info is at: clarkhoward.com/shownotes/category/9/327/. He lists six insurance companies on his honor roll of those with a good financial rating: clarkhoward.com/topics/LTC_list.html. Four companies receive an honorable mention. If you were going to get quotes from three insurance companies, you might consider three of the six on his honor roll.

Good luck!

Treating incontinence by injecting muscle stem cells

This not-yet-available in the US treatment for urinary incontinence may be of interest to many in the group…

Beth (with multiple system atrophy)) had another interesting post recently on an MSA-related list. I’ve gotten her permission again to copy it below.

She includes an abstract from a 5/21/06 American Urological Association meeting. The abstract summarizes two clinical studies done in Austria and Canada on injecting muscle stem cells to treat urinary incontinence. Roger Dmochowski, MD, a professor with the Dept of Urology at Vanderbilt University in Nashville, moderated a press conference on urinary incontinence at the meeting. He said two interesting things:

* “The technique is minimally invasive compared to surgical treatments for urinary incontinence. Once it gets traction, it could go rapidly into the practice arena.”

* Some larger studies will be beginning very soon in the United States.

If you want to keep an eye out for possible trials in the US, you can: 1) watch and search on clinicaltrials.org, using “urinary incontinence” as the search term, 2) watch Medscape’s weekly newsletters, and 3) go to a medical library or go online to review announcements put out by major urology journals.


From: Beth Klitch
Date: Tue May 30, 2006 6:01pm(PDT)
Subject: Exciting new treatment for urinary incontinence – Injection of muscle stem cells

Urinary incontinence is another common condition that we MSA patients experience. Some of us respond to medications such as Flomax that reduce the bladder spasms that can cause urge incontinence. Some of us may undergo surgery to have devices such as Medtronic’s InterStim device implanted to send electrical signals to the sacral nerves that help stimulate the bladder to contract. Many more of us find ourselves isolated or fearful of incontinence accidents and thus we pass up opportunities to travel outside our homes or to visit friends.

Earlier this month, the American Urological Association met and heard reports about a wide range of topics that affect the urinary tract. I have included an extended summary of some exciting research that is underway to treat and actually cure urinary incontinence. Note that the first two clinical studies were performed in Austria and Canada and that larger trials are planned for the United States in upcoming months. This may be an opportunity to volunteer to take part in a clinical trial that has high potential to improve the quality of our lives. Also note that the results were significantly better for women than men and that there are some risks to any surgical procedures, even ones that are minimally invasive.


May 22, 2006 (Atlanta) ­ Injection of muscle stem cells into the suburethral mucosa may be effective for the treatment of urinary incontinence, according to findings from 2 clinical studies. Hannes Strasser, MD, from the Universitatsklinik fur Urologie, Medizinische Universitat Innsbruck, Austria, and colleagues reported their findings here at the American Urological Association 2006 Annual Meeting.

Injecting stem cells into the urethral submucosa is expected to alleviate atrophy of the submucosa by potentially boosting the contractility of the muscle tissue and improving urethral function. In the study, a total of 130 patients (45 men and 85 women) with stress or mixed urinary incontinence were treated with transurethral ultrasound­guided injections. Patients were aged 36 to 85 years. Changes in morphology and function of the urethra and rhabdosphincter and quality of life were evaluated.

While under local anesthesia, patients had small skeletal muscle biopsies taken from their upper arm. Cells from the sample were cultured, and fibroblasts were eventually mixed with about 2.5 mL of collagen, which served as carrier material. With the aid of a transurethral ultrasound probe and injection device, the fibroblasts were introduced into the urethral submucosa. The myoblasts were directly injected into the rhabdosphincter to reconstruct the muscle.

Urinary incontinence was cured after injection of stem cells in 111 (79 women, 32 men) of the 130 patients. Therapy was able to increase the thickness of the urethra and the rhabdosphincter as well as increase the activity and contractility of the rhabdosphincter. An additional 17 patients experienced an improvement but not complete relief of incontinence.

Significant improvements in quality of life were also observed after treatment, and the therapy appeared to be well tolerated. No adverse effects or complications were observed. Dr. Strasser noted that further follow-up indicated that 1 patient had a major complication ­ a perforation in a male patient who had undergone several surgeries and radiation therapy.

According to Dr. Strasser, the efficacy of this technique is better in women than men, possibly for 2 reasons: the injection is easier in women due to the shorter length of the urethra. The second is that after radical prostatectomy in men, changes such as scarring can take place in the urethra. “In females the efficacy rate is more than 90%, whereas in males the efficacy rate is about 72% to 73%,” he told Medscape.

The therapeutic effect appeared to be long-lasting. “The full effect of the therapy takes about 3 to 4 weeks to achieve, whereas other injectable therapies can take effect immediately,” he said. “However, the vast majority of patients who do well 3 months after therapy remain stable. These patients are still continent, meaning they don’t need pads.”

A smaller, North American study evaluating a similar procedure was reported by Lesley K. Carr, MD, from the University of Toronto, Ontario, Canada, and colleagues. Six women, aged 41 to 66 years, with stress urinary incontinence, were treated with either a trans- or periurethral injection technique; one of the patients was reinjected after 6 months.

Skeletal muscle tissue was taken from each patient with a needle biopsy technique. Muscle-derived cells were then isolated and expanded in culture. After at least 1 month of follow-up, no improvement was observed in patients treated with a smaller (8 mm) cystoscopic injection needle. However, 2 subsequent transurethral injections using a longer needle (10 mm) and the 2 periurethral injections did result in improvement in 4 patients, who all reported an improvement in quality of life.

“We hypothesize that the injected muscle-derived cells differentiated into new muscle fibers and improved muscle function, but the exact mechanisms of these actions are still being investigated,” note Dr. Carr and colleagues in their abstract. “Improvements to the delivery technique may have contributed to a greater success rate in the most recently injected patients.” Again, no adverse effects were noted.

Roger Dmochowski, MD, a professor with the Department of Urology at Vanderbilt University in Nashville, Tennessee, noted that the results seen with this technique are “very encouraging,” although he pointed out that various injection techniques and different types of cells are being studied, “making it difficult to compare results.”

Several centers in Europe are experimenting with autologous muscle stem cell injection, and some larger studies will be beginning very soon in the United States, noted Dr. Dmochowski, who moderated a press conference on urinary incontinence at the meeting. “The technique is minimally invasive compared to surgical treatments for urinary incontinence,” Dr. Dmochowski added. “Once it gets traction, it could go rapidly into the practice arena.”

AUA 2006 Annual Meeting: Abstracts 328 and 1284. Presented May 21, 2006.

Drug treatment for orthostatic hypotension

This will likely be of interest to the MSA folks or those with orthostatic hypotension as a symptom….

Beth Klitch has MSA. She lives in the Midwest. She posted this recently on an MSA digest; it’s about the success seen by Japanese researchers using an FDA-approved diabetes drug to treat post-prandial hypotension. I asked her permission to copy the post here because I think it may be useful to some of our group members.

To find the three-sentence Neurology journal article abstract the post below refers to, go to www.neurology.org and search for “voglibose.”


From: Beth Klitch
Date: Tue May 30, 2006 5:44pm(PDT)
Subject: Exciting new use of an existing drug to treat hypotension after meal

Since many of us with MSA suffer from severe orthostatic hypotension, me included, I keep a close eye on research reports about medications and other treatments that may be available to help us treat this condition. Just a few days ago, an interesting report surfaced in Neurology 2006;66:1432-1434 about a drug primarily used to treat diabetes, but which has profound implications to help treat the hypotension that occurs right after eating a meal, also called post-prandial hypotension. Note that MSA patients were included in this research along with Parkinson’s patients and patients with diabetes.

I have shared the article summary below for those who are interested. I think this is potentially great news for us because it is another example of an existing drug that can be extended to treat an additional condition and has already met the FDA’s safety and efficacy standards. The drug is called Voglibose, is marketed as Volix, and is considered one of the most important alpha-glucosidase inhibitors. It works in treating diabetes by delaying the digestion and absorption of carbohydrates, thereby decreasing the rise in glucose levels and insulin levels that typically occur after eating a meal.


NEW YORK (Reuters Health) May 24 – The alpha-glucosidase inhibitor voglibose can help reduce postprandial hypotension, Japanese researchers report in the May 9th issue of Neurology. Lead investigator Dr. Takahiro Maruta of Kanazawa University and colleagues note that reduced blood pressure after a meal is common in the elderly and in those with conditions such as Parkinson’s disease and diabetes mellitus. It can increase the risk of falls and coronary events.

To examine the effect of voglibose on postprandial hypotension, the researchers studied 48 elderly subjects including those with Parkinson’s disease, multiple system atrophy and diabetes as well as elderly and younger controls with no autonomic disorder. Within 2 hours of 75-g glucose loading, blood pressure fell by more than 20 mm Hg in 72.7% of the Parkinson’s patients, all of those with multiple system atrophy, 27.3% of the patients with diabetes, 23% of the elderly controls and none of the younger controls.

Following voglibose administration, there was a significant reduction in this drop in blood pressure. Without voglibose the mean drop was 41.5 mm Hg; with voglibose, it was 21.0 mm Hg. There was also a reduction in the duration of postprandial hypotension under the two conditions (52.3 minutes versus 17.3 minutes).

Summing up Dr. Maruta told Reuters Health, “Many people suffer from symptoms due to hypotension. Our research should cast some light on ways to help them.”