PSP Research Study- NIH-Funded and Parkinson’s Institute is a Site

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I just received this email from the Society for PSP (in Maryland) about an important NIH grant given to Dr. Irene Litvan, one of the top researchers in the US on progressive supranuclear palsy. One of the eight sites is The Parkinson’s Institute (thepi.org) in Sunnyvale. The site coordinator there is our friend Dr. James Tetrud, a neurologist with wide knowledge of PSP. (He spoke at the Society’s recent PSP Family Conference. He mentioned the arctic ground squirrel. Most of you received an email from me about that.)

If you are interested in participating in this PSP research study, please contact Whitney Rogers, the Clinical Coordinator for the main study and for the University of Louisville site in particular. Her contact info is: 502-852-4612 or [email protected].

I have just spoken with Whitney to get some general info; she is very nice. She explained that every person with PSP who wants to be involved in the study needs to bring with them two controls. The controls should: not have PSP, not be a blood relative, and be similar in age to the person with PSP (plus or minus 5 years). One control must be the same gender as the person with PSP; this is usually an in-law. The other control can be a different gender as the person with PSP; this is usually the spouse.

Please let me know if you do contact Whitney and run into some issues that the rest of the group should know about. Also, I’d appreciate knowing who eventually signs up to participate in this study!

Thanks,
Robin

PS. The “Mayo Clinic” mentioned below is the one in Jacksonville, FL.

Subject: NIH Grant to Irene Litvan
Date: Mon, 22 May 2006 13:10:25 -0400
From: CurePSP
To: Support Group Leaders

Movement Disorder Program Awarded Novel NIH Clinical Research Grant to Study Rare Neurodegenerative Disease

Dr. Irene Litvan, Raymond Lee Lebby Professor of Neurology and Director of the Movement Disorder Program has been awarded a $3.4 million NIH grant to study the genetic and environmental risk factors for progressive supranuclear palsy. Progressive supranuclear palsy (PSP) is the most common atypical parkinsonian movement disorder. Dr. Litvan will lead a team of movement disorder specialists from 8 sites across the country (Baylor, Case Western, Emory, Mayo Clinic, Parkinson Institute, Massachusetts General Hospital, University of Maryland) and basic science researchers (Duke, Johns Hopkins, Mayo Clinic, Washington University, University of Louisville), in determining the role of genetic, occupational and environmental components in the development of PSP. Specifically, this large case/control study that will involve 500 PSP patients and 1000 controls will seek to determine if there is an association between PSP and specific H1 tau genotypes, alpha-synuclein polymorphisms, parkin gene deficits or other gene-gene interactions. The study will also explore whether there is an association between PSP and occupational and or environmental chemical exposures functionally or structurally similar to known parkinsonian toxicants. This is the first major research award to focus on PSP.

The Movement Disorder Program has begun recruiting patients with PSP for inclusion in the study. Interested parties should contact the Movement Disorder Program at 502-852-4612. It is hoped that this major effort will in turn help find ways to prevent or treat this devastating disease.

Journal article on MRIs to distinguish PSP, MSA, and PD

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This article in the Movement Disorder Society magazine (of the UK) is on using MRIs to distinguish between Progressive supranuclear palsy (PSP), Multiple system atrophy (MSA), and Parkinson’s disease (PD). Apparently the technique is most (only?) effective in later stages. This was published online on 4/6/06. The abstract is below.

Robin

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Regional brain volumes distinguish PSP, MSA-P, and PD: MRI-based clinico-radiological correlations
Dominic C. Paviour, MRCP, Shona L. Price, BSc, Marjan Jahanshahi, PhD , Andrew J. Lees, MD, FRCP , Nick C. Fox, MD, FRCP

Progressive supranuclear palsy (PSP) and multiple system atrophy (MSA) are neurodegenerative disorders, each with a prevalence of around 5 per 100,000. Regional brain atrophy patterns differ in the two disorders, however, and magnetic resonance imaging is sometimes helpful in distinguishing them in the later stages. We measured whole brain and regional volumes, including cerebellum, pons, midbrain, superior cerebellar peduncle (SCP), and ventricular volumes as well as frontal and posterior-inferior cerebral regions in 18 subjects with PSP, 9 with MSA-P (parkinsonian phenotype), 9 with Parkinson’s disease (PD), and 18 healthy controls. Associations between these volumes, cognitive profiles, and clinical measures of disease severity and motor disability were assessed. Mean midbrain volume was 30% smaller in PSP than in PD or controls (P < 0.001) and 15% smaller than in MSA-P (P = 0.009). The mean SCP volume in PSP was 30% smaller than in MSA-P, PD, or controls (P < 0.001). Mean cerebellar volumes in MSA-P were 20% smaller than in controls and PD and 18% smaller than in PSP (P = 0.01). Mean pontine volume in MSA-P was 30% smaller than in PD or controls (P < 0.001) and 25% smaller than in PSP (P = 0.01). Motor disability was most strongly associated with midbrain volume, and more severe executive dysfunction was associated with reduced frontal volume. These distinct patterns of cortical and subcortical atrophy, when considered together rather than independently, better differentiate PSP and MSA-P from each other and also from healthy controls. © 2006 Movement Disorder Society

“Applause sign” to diagnose PSP?

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The “applause sign” test is useful in diagnosing progressive supranuclear palsy (PSP), according to recent research.  The “applause sign” is where you ask someone who might have PSP to clap.  While clapping, you tell them to stop.  The person with PSP continues to clap; it takes them awhile to stop.

In a study done by Dubois, 30 out of 42 patients diagnosed with PSP could not stop applauding immediately after being told to stop.  None of those with frontotemporal dementia (FTD) or Parkinson’s Disease (PD) had trouble stopping.

So I guess the part of the brain that controls this process is not affected by PD or FTD…?  The “applause sign” is an indication of “motor perseveration.” Perhaps this is controlled by the frontal lobe so interesting that it’s not affected in FTD.

I’ll ask the neurologist to do this in Dad’s next appt.  I’m interested in seeing this.

Copied below is part of the abstract of the Dubois article (published 6/05 in Neurology) from PubMed (pubmed.gov).

Robin

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“Applause sign” helps to discriminate PSP from FTD and PD

The “applause sign” is a simple test of motor control that helps to differentiate PSP from frontal or striatofrontal degenerative diseases. It was found in 0/39 controls, 0 of 24 patients with frontotemporal dementia (FTD), 0 of 17 patients with Parkinson disease (PD), and 30/42 patients with progressive supranuclear palsy (PSP). It discriminated PSP from FTD (p < 0.001) and PD (p < 0.00). The “three clap test” correctly identified 81.8% of the patients in the comparison PSP and FTD and 75% of the patients in the comparison of PSP and PD.

NIH/NINDS Creatine Study Press Release

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If you are interested in research going on in the Parkinson’s world that might affect the atypical parkinsonism disorders, this post may be of value to you.

The National Institute of Neurological Disorders and Stroke (NINDS) of the National Institutes of Health (NIH) has organized a nationwide effort called NET-PD (Neuroprotection Exploratory Trials in Parkinson’s Disease), a randomized, double-blind futility trial, to study compounds that may slow the clinical decline of Parkinson’s disease.  Creatine and the antibiotic minocycline were identified as agents worthy of preliminary study.

In a press release on the study that was released in conjunction with the World Parkinson Congress in Feb ’06, the lead investigator said that a “clinical trial with 200 Parkinson’s disease patients has shown that creatine and minocycline may warrant further consideration for study in a large trial.”

Here’s a link to the press release titled
“Preliminary Results Shows Creatine and Minocycline May Warrant Further Study in Parkinson’s Disease”:

www.ninds.nih.gov/news_and_events/press_releases/pressrelease_parkinsons_20060223.htm

The bottom line is this:

“The trial investigators note that while encouraging, this pilot study does not have sufficient numbers of patients or duration of follow-up to recommend that patients with Parkinson’s take either agent.  In fact, the investigators caution Parkinson’s patients and their physicians not to interpret the results of this study as suggesting such a course of treatment.  Further study is required before the researchers can conclude whether creatine or minocycline is in fact helpful, harmful or has no significant impact.”

Robin

Arctic ground squirrel clears out tau during hibernation

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The PSP Family Conference, put on by the Society for PSP (Progressive Supranuclear Palsy), was held last Saturday near SFO.

As I had attended the ’04 PSP Family Conference and had heard Dr. James Tetrud speak before, I had different expectations of what he would say at this year’s conference.  He is the neurologist at The Parkinson’s Institute who probably knows the most about Atypical Parkinsonism disorders, such as PSP, LBD, MSA, and CBGD.  I compared his slides from ’06 to ’04, and was disappointed that there was nothing new to report in the area of research with one exception:  at last Saturday’s conference he mentioned the Arctic ground squirrel.

During a 7-month hibernation, the squirrel’s brain loses many of the nerve-cell connections that govern how it operates.  Tau protein accumulates in the brain — just like with Alzheimer’s Disease and PSP.  (I’m sorry to say that I don’t know the pathology of the other Atypical Parkinsonism diseases.)  Within 2 to 3 hours of emerging from hibernation, there is a wave of neuronal growth and tau is eliminated.  Thus, there appears to be a mechanism to clear tau.

Dr. Tetrud referred to a recent Economist magazine article on the subject.  I consider myself to have excellent follow-through and research skills but I was outdone on both of these fronts by Sam, a support group member who also attended the Saturday conference.  He found the article in the Feb. 4th issue (page 72).  Here’s a link to the article:

www.economist.com/node/5466196

Dementia
Sleeping on it
Similarities between dementia and hibernation suggest a treatment
The Economist
Feb 2nd 2006

It’s interesting reading!  Let’s hope something comes of it…

Robin