Gait and Balance Webinar Notes (5-28-09)

Did anyone attend this webinar on gait and balance webinar on May 28, 2009 and take notes (especially during the Q&A and when some photos were shown) that you can share?  It was at 2am in the timezone where I was that day, so I was unable to call in.

Though CurePSP was the organizer of this webinar, it applied to all the disorders in our group.

Today, as a registered attendee of the webinar, I received a copy of the slides.  I don’t think these have been posted yet to the CurePSP website (psp.org).

It seems from the slides that it was a very good webinar.  I’ve copied below the text from some of the slides shown during the webinar.

Here are some highlights from my perspective:

  • Assistive Device Golden Rule:  If you find yourself reaching out to touch walls, furniture, friends or care partners (or they are reaching to you) while walking–you are in need of an assistive device.
  • If falls continue no matter what we do, then safety gear may be the answer:  helmets; HipSaver®; chair or bed alarm; wheelchair; geriatric recliner chair.
  • How can PT and OT help?  Assess & treat functional mobility and ADL abilities.  Perform home safety assessments.  Develop new and safer ways to perform activities.  Train care-partners to safely assist.  Recommend appropriate adaptive equipment for the home.  Recommend appropriate mobility devices.

 

Robin

—————————————————————————-

Gait & Balance Questions Answered
by Heather Cianci, PT, MS, GCS, Penn Neurological Institute
5-28-09 CurePSP Webinar

Causes of Gait Changes & Falls
..Rigid/Stiff Muscles and Joints
..Shuffling steps
..Narrow base of support (feet too close)
..Slowed or absent balance reactions
..Slowness of Movement
..Visual Changes
–Difficulty with scanning your surroundings
–Double vision
..Loss of Coordination
..Freezing
..Blurred vision
..Orthostatic hypotension –sudden blood pressure drop
..“Alien limb”
..Myoclonus–quick, jerky movements
..Sensation changes
..Impaired safety judgment

Common Places Falls Occur
..Walking
..Turning
..On Stairs or Curbs
..While Reaching
..Transferring:
–In & out of bed
–In & out of the shower/bath tub
–In & out of the car
–Up & down from a chair or the toilet

Tips for Walking Safely
..Slow down & concentrate
–You must now “tell”your body what to do
–If you are unable to do this, it is important to have a care partner remind you
..To avoid shuffling
–Focus on landing with the heel hitting down first, not the toes
..Do one thing at a time
–Do not reach for the refrigerator door while walking toward it
..Keep your hands free
..Be aware of changes in the floor surface
–Tilt your head down to look at the floor
–Stop before the surface change and step over the threshold

Assistive Device Golden Rule
If you find yourself reaching out to touch walls, furniture, friends or care partners (or they are reaching to you) while walking–you are in need of an assistive device.

Getting Out of Bed
..Bend up knees
..Roll completely on your side
..Grab rail
..Allow your legs to drop off of the side of the bed…
..While you push yourself up sideways into sitting
..If help is needed, care partner can lift under shoulder & push down at outer thigh

Getting In & Out of the Car
To Safely Transfer
–Slide seat back
–Back into car & then slide legs in, reverse to get out
–Plastic bag on seat can make moving on fabric seats easier
–Place cushion on low seats to help with standing

Handy Bar (for gettign in and out of the car)
Allows for a safe place to hold
www.handybar.com, 888-738-0611

Sit to Stand Transfer
..Scoot to front of chair
..Open legs wide
..Pull feet back under the knees
..Lean forward –“Nose over Toes”
..Push forward until butt lifts off of chair
..Then push up to stand

Protection Products/Devices
If falls continue no matter what we do, then safety gear may be the answer…
Helmets
•http://www.cpsc.gov/CPSCPUB/PUBS/349.pdf(US Consumer Safety Product Commission brochure)
•www.headsaver.com.au
•Local sporting goods store
HipSaver®(shorts with hip and tailbone pads)
•www.hipsaver.com
•www.alimed.com or 800-225-2610
Chair or Bed Alarm
•Posey Sitter II®Alarm Unit
•www.posey.comor 800-447-6739
•Features different alarms or voice recording when person moves from chair or bed
Wheelchair or Geriatric Recliner Chair
•Contact local PT/OT or Rehabilitation Center to find a “Seating Clinic”or Wheelchair specialist

Rehabilitation Strategies
Current Research is Supporting
..Balance exercises
..Balance exercises along with eye movement training
..Treadmill and weight-supported treadmill training
..Group exercise programs
Exercise needs to be ongoing throughout the course of the disease. Delays in the need for wheelchairs, less falls, improvements in gait & balance, and reduced gait changes are possible.

How Can I Get More Help with Finding Assistive Devices?
RESNA (Rehabilitation Engineering & Assistive Technology Society of North America)
..www.resna.org
..“Projects”
..“Statewide AT Program”
..“State AT Program”or “Device Loan”
NATTAP (National Assistive Technology Technical Assistance Partnership)
..Locate programs available in each state
..703-524-6686

How Can I Find Someone to do Home Modifications?
Certified Aging-In-Place Specialist (CAPS)
..Certified specialist through The National Association of Home Builders
..Remodelers, Contractors, Interior Designers, Therapists
..800-368-5242
..www.nahb.org
–Resources –Online Directories –Find a CAPS

How Can PT and OT Help?
..Assess & treat functional mobility & ADL abilities
..Perform home safety assessments
..Develop new & safer ways to perform activities
..Train care-partners to safely assist
..Recommend appropriate adaptive equipment for the home
..Recommend appropriate mobility devices

Locating a PT or OT
..American PT Association
–1-800-999-2782
–www.apta.org
–“Find a Certified Specialist (Neurologic or Geriatric)”
..American OT Association
–301-652-2682
–www.aota.org
..LSVT®BIG
–888-438-5788
–www.lsvtglobal.com
–“Find a Clinician”
..Call Local Outpatient Rehab Centers
–Generally those associated with Hospitals (Regular & Rehabilitation), & non-sports oriented centers
–Ask if they have therapists who have worked with PSP or those who deal with neurological & balance problems
..We Move
–www.wemove.org
–“Find a Doctor”–Physical Medicine & Rehab
..National Parkinson Foundation
–800-327-4545
–www.parkinson.org
–Allied Team Training (ATTP)
–List of Graduates

Davunetide (aka, NAP, aka AL-108) trial now to focus on PSP

In November 2008, CurePSP, through the Pollin CBD Research Fund, awarded a research grant to UCSF and Dr. Boxer to study NAP (AL-108) in CBD. See:
http://forum.psp.org/viewtopic.php?t=7669

Within the last month or so I heard from a CBD family who plans to participate in the trial that UCSF would begin enrollment some time in the fall of 2009. There were to be at least two additional sites participating — Mayo Rochester and UPenn.

I heard a few days ago from Sharon, a local support group member, that the NAP trial would be open to PSPers. I received confirmation from UCSF today:

“After feedback from the European equivalent of the FDA, we were forced to focus on PSP, although we will still enroll a smaller number of CBD and FTD patients. The study will also be much larger than originally planned because of additional $ raised by the company that manufactures the drug. We are still the lead site and there will likely be 25 North American sites. More details about sites will be forthcoming in the next few months.”

UCSF emphasized that they have no further details to provide at this time. They did point me to a press release from Allon Pharmaceuticals, NAP’s manufacturer, that came out yesterday. I’ve copied that below.

One item I will clarify later is whether participating patients must have dementia or cognitive impairment.

Robin

http://allontherapeutics.com/ir_news_25Jun_2009.html

Allon Therapeutics forms Steering Committee for Phase II human trial

VANCOUVER — June 25, 2009 – Allon Therapeutics Inc. (TSX:NPC) reported today that leading neurologists and psychiatrists have joined a special Steering Committee to help the Company design and conduct a Phase II human clinical trial that will evaluate whether Allon’s lead neuroprotective drug candidate, davunetide intranasal (AL-108), has the potential to become the first effective treatment for a number of brain disorders broadly categorized as frontotemporal dementia (FTD). The study will evaluate the effect of davunetide for the treatment of Progressive Supranuclear Palsy (PSP), one type of FTD. A smaller study in other related forms of FTD will be carried out in parallel.

PSP is a degenerative brain disease that is often characterized by progressive difficulty with balance and walking, eye movement abnormalities, and cognitive and personality changes. PSP affects approximately 20,000 people in the United States, a patient population size consistent with an orphan drug designation. The company expects that efficacy in PSP would define the opportunity to use davunetide (AL-108) in other FTD subtypes that are tauopathies.

Gordon McCauley, President and CEO of Allon, said the Company plans to begin the Phase II trial in FTD during the second half of 2009.

“Physicians and researchers who specialize in FTD are enthusiastic about evaluating davunetide intranasal (AL-108) in FTD patients, primarily because about 50% of FTD and related disorders are tauopathies, or tau-related diseases — and Allon’s technology is recognized as the most clinically advanced tau-related therapy,” McCauley said.

The Company identified some of the members of its FTD Steering Committee as:

• Adam L. Boxer, M.D., Ph.D., Director of the University of California, San Francisco (UCSF) Alzheimer’s Disease and Frontotemporal Dementia Clinical Trials Program and Assistant Professor of Neurology at the UCSF Memory and Aging Center, who specializes in Alzheimer’s Disease, Frontotemporal Dementia and related disorders such as Corticobasal Degeneration and Progressive Supranuclear Palsy.

• Rachelle S. Doody, M.D., Ph.D., Professor of Neurology and Effie Marie Cain Chair in Alzheimer’s Disease Research at Baylor College of Medicine, Houston, who specializes in Alzheimer’s disease and memory disorders.

• Murray Grossman, M.D., Professor of Neurology and Psychiatry at the University of Pennsylvania School of Medicine, who specializes in the diagnosis and treatment of non-Alzheimer’s forms of dementia such as frontotemporal dementia, corticobasal degeneration, amyotrophic lateral sclerosis, and Lewy body disease.

• Anthony E. Lang, M.D., Director of the Division of Neurology and Jack Clark Chair for Parkinson’s Disease Research at the University of Toronto, and Director of the Morton and Gloria Shulman Movement Disorders Centre, Toronto Western Hospital.

• Bruce L. Miller, M.D., Professor of Neurology at the University of California, San Francisco, A.W. & Mary Margaret Clausen Distinguished Chair, and Clinical Director of the UCSF Memory and Aging Center, which treats patients with diseases that cause dementia, including Alzheimer’s disease, corticobasal degeneration, Creutzfeldt-Jakob disease and frontotemporal dementia.

• Lon S. Schneider, M.D., Professor of Psychiatry, Neurology and Gerontology at the Keck School of Medicine of the University of Southern California, whose specialization includes Alzheimer’s disease and dementia.

FTD encompasses several cognitive disorders, including Behavioral Variant-Frontotemporal Dementia, Semantic Dementia and Progressive Nonfluent Aphasia, and the movement disorders, Corticobasal Degeneration and Progressive Supranuclear Palsy. No effective treatment is currently available for FTD, and several FTD syndromes are fatal within three to five years. FTD is often incorrectly diagnosed as Alzheimer’s disease.

In 2008, Allon reported efficacy results from a Phase IIa clinical trial that demonstrated that davunetide intranasal (AL-108) improved short-term and working memory performance in patients with amnestic mild cognitive impairment, a precursor to Alzheimer’s disease.

The pathology of Alzheimer’s disease and many forms of FTD has some similarities, including the presence of altered forms of the brain protein tau. In Alzheimer’s, altered tau forms tangles, part of the well-established plaques and tangles hallmarks of Alzheimer’s pathology. PSP is associated with “pure” tau pathology, unlike Alzheimer’s disease in which both amyloid and tau pathology is identified. This provides additional rationale for the use of AL-108 which targets tau in PSP.

Allon has shown that decreasing the levels of altered forms of tau with davunetide intranasal (AL-108) preserved the memory of mice bred to replicate Alzheimer’s or FTD pathology. Allon’s preclinical studies have also shown that davunetide intranasal (AL-108) preserved the memory and learning function of mice bred to replicate the altered tau pathology associated with FTD.

This trial at UCSF is now recruiting. Trial info was posted to clinicaltrials.gov about two weeks ago. See:
http://clinicaltrials.gov/ct2/show/NCT01056965

This is a 12-week study of davunetide (NAP, AL-108) or placebo is those with PSP, CBD, or two frontotemporal lobar degeneration disorders associated with tau pathology (FTDP-17 and PNFA). The drug and the placebo are both administered via nasal spray.

I noticed these two inclusion criteria may disqualify some:
* Subject resides outside a skilled nursing facility or dementia care facility. Residence in an assisted living facility is allowed.
* Able to ambulate with or without assistance.

And this exclusion criteria may disqualify some:
* Early, symptomatic autonomic dysfunction
* Within 4 weeks of screening or during the course of the study, concurrent treatment with memantine, acetylcholinesterase inhibitors, antipsychotic agents or mood stabilizers (valproate, lithium, etc.) or benzodiazepines (other than temazepam or zolpidem).
* Treatment with lithium, methylene blue, tramiprosate, ketone bodies, Dimebon or any putative disease-modifying agent directed at tau within 90 days of screening.
* Subject not willing to attempt LP [Robin’s note: LP probably means lumbar puncture.]

The UCSF trial contacts are:
Mary Koestler, RN, PhD, phone 415 476 0661, mkoestler AT memory.ucsf.edu
Arielle Lasky, phone 415 476 8333, alasky AT memory.ucsf.edu [Robin changed this from Nicholson to Lasky, as clinicaltrials.gov got updated]

Dr. Adam Boxer at UCSF’s MAC is the principal investigator.

I’ve heard from a few people (including one member here) that Mayo Jax intends to participate in this trial. Presumably this will happen later in 2010.

Robin

Will speech deteriorate over time?

I received this email earlier today:

“Mother seems to have all the major symptoms described with PSP except her speech. I can still understand everything she says easily (although her voice is weaker). Will this change over time too?”

Here’s my answer:

Probably though no one can be certain. People with the PSP-parkinsonism type of PSP don’t have dysarthria as a primary symptom. But these people often develop speech problems later on.

In a woman in our local support group who has PSP-P, her speech was understandable by me for the last couple of years. Now her voice has gotten so quiet that I can’t hear her, and no one else can either. She hardly speaks any more now.

Seizures in CBD: a case report (UCSF)

CBD folks –
Here’s a case report out of UCSF of someone with a clinical diagnosis of CBD who had a seizure. The abstract says: “To our knowledge, however, there have been no reports of seizures associated with corticobasal degeneration (CBD).” Perhaps nothing has been published in the medical literature about this but there are plenty of case reports available on the CBD-related Yahoo!Group about seizures! I note that this patient had a brain biopsy where the results were “suggestive of CBD.” Of course the only conclusive diagnosis of CBD is available in a brain autopsy (upon death). On a living patient, a pathologist or surgeon can’t get to the right areas of the brain to confirm CBD. The risks of a brain biopsy are not insignificant; my impression is that a biopsy is done (on living patients) only in extreme cases.

Robin


Neurocase. 2009 Jun 18:1-5. [Epub ahead of print]

Seizures in corticobasal degeneration: A case report.

Douglas VC, Dearmond SJ, Aminoff MJ, Miller BL, Rabinovici GD.
Neurology Clinic, University of California, San Francisco, CA, USA.

Seizures are relatively common in Alzheimer disease (AD) and other neurodegenerative disorders. To our knowledge, however, there have been no reports of seizures associated with corticobasal degeneration (CBD). We describe a patient with brain biopsy features suggestive of CBD whose course was complicated by complex partial seizures with secondary generalization. Thus, the occurrence of seizures in a patient with dementia should not exclude the diagnosis of CBD.

PubMed ID#: 19544144 (see pubmed.gov for this free abstract only)

No LRRK2 mutations in 88 PSP patients (Germany)

This recently-published abstract is on some German research into whether the LRRK2 genetic mutation occurs in PSP. This may be of most interest to those who participated in the 23andMe.com genetics testing.

I learned two things from the abstract:

* LRRK2 mutations “have been shown to be the most common genetic cause of both familial and sporadic Parkinson’s disease. Patients harboring LRRK2 mutations develop late onset PD that in most cases cannot be clinically distinguished from idiopathic PD.”

I thought LRRK2 was only associated with familial PD, and I thought familial PD was typically early-onset PD.

* “LRRK2 mutations have been reported to result in a broad spectrum of neuropathological alterations including progressive supranuclear palsy (PSP)-like Tau pathology.”

The weakness of this research is that the 88 PSP patients involved have a clinical diagnosis of PSP. The authors concluded that “there is no evidence that mutations in exon 31 of LRRK2 are a major risk factor for PSP. Our study, however, cannot rule out that other genetic variations in LRRK2 may be associated with PSP.”

Robin

European Journal of Neurology. 2009 Jun 15. [Epub ahead of print]

Screening for LRRK2 R1441 mutations in a cohort of PSP patients from Germany.

Madžar D, Schulte C, Gasser T.
Department of Neurodegenerative diseases, Hertie Institute for clinical brain research, University of Tuebingen, Tuebingen, Germany.

Background and purpose: Mutations in the leucine-rich repeat kinase gene (LRRK2) have been shown to be the most common genetic cause of both familial and sporadic Parkinson’s disease. Patients harboring LRRK2 mutations develop late onset PD that in most cases cannot be clinically distinguished from idiopathic PD.

Furthermore, LRRK2 mutations have been reported to result in a broad spectrum of neuropathological alterations including progressive supranuclear palsy (PSP)-like Tau pathology.

Methods: We screened a cohort of 88 clinically confirmed PSP patients for mutations in exon 31.

Results: We did not find any of the known mutations or any new variants.

Conclusions: Thus, there is no evidence that mutations in exon 31 of LRRK2 are a major risk factor for PSP. Our study, however, cannot rule out that other genetic variations in LRRK2 may be associated with PSP.

PubMed ID#: 19538213 (see pubmed.gov for this abstract only)

Pathology of corticobasal syndrome predictable in life?

Here’s an interesting abstract of some research out of the UK. “Twelve patients with CBS [corticobasal syndrome] were followed prospectively; six had AD and six had classic CBD neuropathology.” Clinical records were reviewed for presenting symptoms. “Initial episodic memory complaints and poor performance on the combined orientation-memory subtest of the Addenbrooke’s Cognitive Examination (ACE) reliably predicted AD pathology while varying combinations of early frontal-lobe type behavioral symptoms, nonfluent language disturbance, orobuccal apraxia, and utilization behavior predicted CBD pathology ante-mortem.”
Robin

Movement Disorders. 2009 Jun 16. [Epub ahead of print]

Is the pathology of corticobasal syndrome predictable in life?

Shelley BP, Hodges JR, Kipps CM, Xuereb JH, Bak TH.
Department of Clinical Neurosciences, University of Cambridge, Addenbrooke’s Hospital, Cambridge, UK.

Corticobasal syndrome (CBS) has been associated with a heterogeneous spectrum of pathologies with an increasing number of reports of Alzheimer’s type pathology. There is, however, no means of predicting pathology of CBS in vivo at present. We compared the clinical features of patients presenting with CBS who have either pathologic changes of classic corticobasal degeneration (CBD) or Alzheimer’s disease (AD) at post-mortem to identify predictors of the specific pathological processes in life. Twelve patients with CBS were followed prospectively; six had AD and six had classic CBD neuropathology. After review of the presenting clinical features, we identified nine potential predictor variables, compared their frequency in the two groups, and performed a discriminant function analysis. Initial episodic memory complaints and poor performance on the combined orientation-memory subtest of the Addenbrooke’s Cognitive Examination (ACE) reliably predicted AD pathology while varying combinations of early frontal-lobe type behavioral symptoms, nonfluent language disturbance, orobuccal apraxia, and utilization behavior predicted CBD pathology ante-mortem. CBS is frequently associated with Alzheimer’s disease pathology. Early episodic memory impairment versus early behavioral symptomatology appears to best predict AD or CBD pathology in life. (c) 2009 Movement Disorder Society.

PubMed ID#: 19533751 (see pubmed.gov for this free abstract only)

orobuccal – “oro” refers to mouth and “buccal” refers to cheek

Severe memory impairment in CBD patient

This article in a Korean-based journal notes that CBD patients typically have “relatively mild motor symptoms. … Our observations indicate that CBD can in rare cases present with severe episodic memory impairment associated with frontal executive dysfunctions in the early stage of illness.” This is a case report on one patient where the diagnosis of CBD has not been confirmed.
Robin

Journal of Clinical Neurology. 2008 Jun;4(2):94-8. Epub 2008 Jun 20.

Severe episodic memory impairment in a patient with clinical features compatible with corticobasal degeneration.

Kim SK, Park KW, Kang DY, Cha JK, Kim SH, Kim JW.
Department of Neurology, Dong-A University College of Medicine, Busan, Korea.

Corticobasal degeneration (CBD) is a progressive neurodegenerative disorder characterized by asymmetric parkinsonism associated with apraxia, cortical sensory loss, and alien-limb phenomenon. Neuropsychological testing in patients with CBD typically shows deficits in executive functions, praxis, language, and visuospatial functioning, but not in memory. We report a CBD patient with severely impaired memory function but relatively mild motor symptoms. Detailed neuropsychological assessment showed significant verbal and visual memory deficits accompanied by frontal executive dysfunctions. Our observations indicate that CBD can in rare cases present with severe episodic memory impairment associated with frontal executive dysfunctions in the early stage of illness.

PubMed ID#: 19513310 (see pubmed.gov for this abstract only; the abstract is available there for free)

Clinical heterogeneity in PSP (7 Japanese autopsy cases)

These Japanese researchers investigated the clinical symptoms of seven autopsy-confirmed PSP cases. Amazingly, “only three patients (42.9%) matched the clinical diagnostic criteria of PSP” at the time of death. “In addition, only one patient (14.3%) matched these criteria at the time of the initial symptoms. Such underdiagnosis of PSP was mainly caused by heterogeneity, variety of the timing, and presence of symptoms in exclusion criteria. The present study also demonstrated that the clinical features of PSP may change dramatically according to the disease stage. Target symptoms should be selected based on time and stage to optimize patient quality of life.”
Robin

Neuropathology. 2009 Jun 7. [Epub ahead of print]

Clinical heterogeneity in progressive supranuclear palsy: Problems of clinical diagnostic criteria of NINDS-SPSP in a retrospective study of seven Japanese autopsy cases.

Sakamoto R, Tsuchiya K, Mimura M.
Department of Neuropsychiatry, Showa University School of Medicine, Setagaya-ku, Tokyo, Japan.

Progressive supranuclear palsy (PSP) is known to display variable atypical clinical features. In the absence of clinical markers to diagnose PSP, neuropathological examination is the “gold standard” for diagnosis.

We retrospectively investigated clinical features in seven autopsy-confirmed cases of PSP. Only three patients (42.9%) matched the clinical diagnostic criteria of PSP proposed by the National Institute of Neurological Disorders and Stroke and the Society for PSP (NINDS-SPSP) at the time of death. In addition, only one patient (14.3%) matched these criteria at the time of the initial symptoms. Such underdiagnosis of PSP was mainly caused by heterogeneity, variety of the timing, and presence of symptoms in exclusion criteria. The present study also demonstrated that the clinical features of PSP may change dramatically according to the disease stage. Target symptoms should be selected based on time and stage to optimize patient quality of life.

PubMed ID#: 19508347 (see pubmed.gov for this abstract only; the abstract is available for free there)

Combo CoQ10 + creatine – neuroprotective in PD mouse model

This announcement about an animal study in PD that showed that a combo of CoQ10 and creatine produced “neuroprotective effects” was posted today on the Parkinson Forum.

http://forum.parkinson.org/forum/viewtopic.php?t=7557

Dear Friends, the following is interesting, but bear in mind that it is an animal study, and it’s too early to say whether it will apply to humans. Nonetheless, it may be of interest to some.

Best,
Kathrynne Holden, MS, RD
http://www.nutritionucanlivewith.com/

========================================================

Coenzyme Q10 and Creatine Show Potential in the Treatment of Neurodegenerative Diseases

Reference: “Combination therapy with coenzyme Q10 and creatine produces additive neuroprotective effects in models of Parkinson’s and Huntington’s diseases,” Yang L, Calingasan NY, et al, J Neurochem, 2009; 109(5): 1427-39. (Address: M. Flint Beal, MD, Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York Presbyterian Hospital, 525 East 68th Street, F610, New York, NY 10021, USA. E-mail: [email protected] ).

Summary: In a study involving a mouse model of Parkinson’s disease, a rat model of Huntington’s disease and a transgenic mouse model of Huntington’s disease, administration of coenzyme Q10 and creatine in combination was found to produce additive neuroprotective effects. Specifically, administration of the 2 agents was found to protect against dopamine depletion in the striatum and loss of tyrosine hydroxylase neurons in the substantia nigra pars compacta. In addition, significant reductions in lipid peroxidation and pathologic alpha-synuclein accumulation in the SNpc neurons was found, as well as reductions in striatal lesion volumes. The treatment was found to significantly block 3-NP-induced impairment of glutathione homeostasis and reduce lipid peroxidation and DNA oxidative damage in the striatum. Moreover, it was found to improve motor performance and extend survival in the mouse model of Huntington’s disease. These results suggest that the combination of co enzyme Q10 and creatine hold potential as therapeutic agents in the treatment of neurodegenerative conditions such as Parkinson’s disease and Huntington’s disease.

“Revealed: how Alzheimer’s infects the brain”(tau)

Here’s an excerpt from a newspaper article on a breakthrough discovery in Alzheimer’s Disease: “It is the first time that scientists have detected infectious properties in the so-called tau protein which causes aggregates of particles known as ‘neurofibrillary tangles’ to build up inside the brain cells of Alzheimer’s patients. The tangles lead to the disease’s symptoms. [The] researchers emphasised that the discovery does not mean that the disease itself is infectious, only that the tau protein seen in Alzheimer’s disease is able to convert otherwise healthy brain proteins into the defective form associated with the disease.”

PSP and CBD, like AD, are tauopathies – disorders of tau protein.

http://www.independent.co.uk/news/scien … 99214.html

Revealed: how Alzheimer’s infects the brain
Breakthrough may lead to new treatments for senile dementia
By Steve Connor, Science Editor
The Independent (UK newspaper)
Monday, 8 June 2009

A scientific breakthrough in the understanding of how Alzheimer’s disease may spread across the brain of elderly patients might lead to novel ways of treating senile dementia, scientists have announced.

A study has discovered that a key brain protein linked with Alzheimer’s disease has infectious properties that allow defects in the protein to be transmitted through the brain and so leads to debilitating neuro-degeneration.

It is the first time that scientists have detected infectious properties in the so-called tau protein which causes aggregates of particles known as “neurofibrillary tangles” to build up inside the brain cells of Alzheimer’s patients. The tangles lead to the disease’s symptoms.

But the researchers emphasised that the discovery does not mean that the disease itself is infectious, only that the tau protein seen in Alzheimer’s disease is able to convert otherwise healthy brain proteins into the defective form associated with the disease.

Alzheimer’s disease is one of the fastest growing and most costly medical conditions. About 700,000 people in the UK have some form of dementia and this will grow to about 940,000 by 2015, rising to more than 1.7 million by 2051 as a result of the demographic time bomb of an ageing population.

Medical researchers believe that the latest findings – which have so far been shown in laboratory mice rather than human patients – could open up new ways of treating Alzheimer’s disease by developing drugs that block the tau’s infectious properties.

The results of the study also show that Alzheimer’s disease has underlying similarities to Creutzfeldt-Jakob disease (CJD), a brain disease that is believed to be caused by other kinds of infectious proteins called “prions” which can, under certain circumstances, be transmitted from one person to another.

Scientists have known for many years that the brain cells of Alzheimer’s patients experience a build up of tau protein in the form of complicated tangles and that these tangles spread in a characteristic fashion through the brain.

The latest study, published in the journal Nature Cell Biology, offers the first proper explanation for how these tangles spread and suggests a possible target for drug companies hoping to develop ways of slowing down the progression of Alzheimer’s disease.

“This opens new avenues in dementia research that will aim to understand how abnormal tau can spread. We can also investigate how diseases caused by tau aggregates and prions are similar,” said Michel Goedert of the Medical Research Council’s Laboratory of Molecular Biology in Cambridge.

“We have looked at whether tau tangles can spread in mice. The injection of brain extract from tangle-bearing mice into animals without tangles caused their tau to tangle and spread from the sites of injection to neighbouring brain regions,” said Dr Goedert, who took part in the study.

The scientists used a strain of genetically modified mouse with a gene for the human form of the defective tau protein. When the tau of this mouse was injected into the brain of ordinary laboratory mice, it caused a build-up of the same sort of tangles seen in Alzheimer’s patients.

“This research in mice does not show that tau pathology is contagious or that it can spread easily from mouse to mouse. What it has revealed is how tau tangles spread within brain tissues of individual mice,” Dr Goedert said.

“It suggests that tangles of proteins that build up in the brain to cause symptoms could have some contagious properties, within brain tissue but not between mice that haven’t been injected with tissue from another mouse and certainly not between people,” he said.

There is no epidemiological evidence that brain diseases like Alzheimer’s or Parkinson’s can spread from one person to another and the latest study does not suggest that such disorders are caused by an infection, said David Allsop, professor of neuroscience at Lancaster University.

“This is interesting because it could explain how tangles spread from one region of the brain to another during the course of Alzheimer’s disease and some other ‘tangle’ diseases,” Professor Allsop said.

Susanne Sorensen, the head of research at the Alzheimer’s Society, said that the study could result in a greater understanding of how tau tangles form and spread through the brain.

“There is still so much we do not understand about the changes in tau that lead to tangle formation in humans and, eventually, widespread cell death,” Dr Sorensen said. “Each new piece of knowledge helps to build a better picture and takes us closer to the point where we can stop loss of brain tissue and dementia for good.”

Rebecca Wood, the chief executive of the Alzheimer’s Research Trust, said: “This greater understanding of how tangles spread in Alzheimer’s may lead to new ways of stopping them and defeating the disease.”