“How to have a better death” and “A better way to care for the dying” (Economist)

There are two Interesting articles in today’s Economist magazine (economist.com) that report on the “huge gap between what people want from end-of-life care and what they are likely.”  This gap was found in a survey done by The Economist in partnership with the Kaiser Family Foundation.  For the survey, people in the US, Brazil, Italy and Japan were asked a set of questions about dying and end-of-life care.

Here’s a link to the first article, which is actually a short editorial by the magazine:


End-of-life care
How to have a better death
Death is inevitable. A bad death is not
Economist, Print edition
Apr 29th 2017

It cites two statistics:

* Nearly a third of Americans who die after 65 will have spent time in an intensive-care unit in their final three months of life.

* Almost a fifth undergo surgery in their last month.

Here’s a link to the second article:


End-of-life care
A better way to care for the dying
How the medical profession is starting to move beyond fighting death to easing it
Economist International Edition
Apr 29th 2017

The second article, titled “A better way to care for the dying,” addresses what Atul Gawande, MD, calls “the experiment of making mortality a medical experience.”  It cites a few statistics:

* People in rich countries can spend eight to ten years seriously ill at the end of life.

* Many deaths are preceded by a surge of treatment, often pointless.  Nearly a third of elderly Americans undergo surgery during their final year; 8% do so in their last week.

* By 2020, 40% of Americans are expected to die alone in nursing homes.

* One international review of prognoses of patients who die within two months suggests that seriously ill people live on average little more than half as long as their doctors suggested they would. Another study found that, for patients who died within four weeks of receiving a prognosis, doctors had predicted the date to within a week in just a quarter of cases. Mostly, they had erred on the side of optimism.

* Remarkably, in three trials the patients receiving palliative care lived longer, even though the quantity of conventional treatment they opted to receive was lower.

* In one study just 43% of people who had written living wills wanted the same treatment course two years later.

Both articles are worth reading.

A protein called PERK may be a target for PSP, CBD, and other tauopathies

Brain Support Network will very likely be hosting and organizing a PSP/CBD conference in San Francisco in October.  (Stay tuned….) One of the international researchers we’ll be inviting to speak is Gunter Hoglinger from Munich.  He’s been involved in PSP and CBD genetics research for at least a decade.  Very impressive guy.

I was looking up a bit about Dr. Hoglinger online and came across this Science Daily article based on a press release from early February 2017 about research published by him and the German Center for Neurodegenerative Diseases (DZNE).  This is basic research using donated brain tissue, cell cultures, and mice.  This basic research can be the basis of good clinical trials down the road.

Here’s an excerpt from the Science Daily article:

“In previous studies, Höglinger and his colleagues had found that the risk for PSP is associated with variants at the PERK [protein kinase RNA-like endoplasmic reticulum kinase] gene, and that loss of PERK function induces tau pathology in humans. For the current study, they examined the functioning of this protein more closely, to see how its effects could be positively influenced. To this end, they investigated samples of brain tissue from deceased patients, cell cultures and mice with a genetic disposition for PSP.  ‘We found that the disease sequelae decrease when PERK is activated with pharmaceuticals,’ [Hoglinger said.]  ‘Therefore, the protein could be a starting point for the development of new drugs.'”

The short article is copied below.




Science News
A protein called PERK may be a target for treating progressive supranuclear palsy
Acting upon the maintenance system of neurons alleviates disease sequelae in laboratory experiments

Date:  February 6, 2017
Source:  DZNE – German Center for Neurodegenerative Diseases

The brain disease ‘progressive supranuclear palsy’ (PSP) is currently incurable and its symptoms can only be eased to a very limited degree. PSP impairs eye movements, locomotion, balance control, and speech. Scientists have now discovered a molecular mechanism that may help in the search for effective treatments.

The brain disease “progressive supranuclear palsy” (PSP) is currently incurable and its symptoms can only be eased to a very limited degree. PSP impairs eye movements, locomotion, balance control, and speech. Scientists at the German Center for Neurodegenerative Diseases (DZNE) and the Technical University of Munich (TUM) have now discovered a molecular mechanism that may help in the search for effective treatments. Their study focusses on a protein called PERK (protein kinase RNA-like endoplasmic reticulum kinase). A team of researchers led by Prof. Günter Höglinger reports on this in the journal EMBO Molecular Medicine.

PSP belongs to a group of neurological diseases referred to as “tauopathies.” In these diseases, a molecule called “tau” forms clumps rather than stabilizing the cytoskeleton as it normally does. Affected neurons can degenerate or even perish. To prevent such events, pathological molecules are normally repaired or disposed of by the organism. The protein PERK is part of such a maintenance system. However, in PSP, this mechanism appears to be defective. In previous studies, Höglinger and his colleagues had found that the risk for PSP is associated with variants at the PERK gene, and that loss of PERK function induces tau pathology in humans. For the current study, they examined the functioning of this protein more closely, to see how its effects could be positively influenced. To this end, they investigated samples of brain tissue from deceased patients, cell cultures and mice with a genetic disposition for PSP.

“We found that the disease sequelae decrease when PERK is activated with pharmaceuticals. That is to say: when its effect is enhanced,” says Höglinger, who leads a research group at the DZNE’s Munich site. “These results are still basic research and far from being ready for use in patients. However, our investigations show that PERK is an important part of the disease mechanism. Therefore, the protein could be a starting point for the development of new drugs.”

Höglinger also sees potential for tackling diseases other than PSP. This is because PERK helps eliminate abnormal tau molecules, and these also occur in other brain diseases. “These results could have a broad relevance. Because defective tau molecules play an important role especially in Alzheimer’s disease,” the researcher says.

Journal Reference:
Julius Bruch, Hong Xu, Thomas W Rösler, Anderson De Andrade, Peer‐Hendrik Kuhn, Stefan F Lichtenthaler, Thomas Arzberger, Konstanze F Winklhofer, Ulrich Müller, Günter U Höglinger. PERK activation mitigates tau pathology in vitro and in vivo. EMBO Molecular Medicine, 2017; e201606664 DOI: 10.15252/emmm.201606664

“Next-Generation Tau PET Tracers Strut Their Stuff” – differentiating PSP from AD

This is a report by Alzforum from the Alzheimer’s/Parkinson’s 2017 conference in Vienna at the end of March.  The focus of the report is on next-generation tau PET tracers.  Tau is the protein involved in Alzheimer’s Disease (AD), progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), Pick’s disease, and chronic traumatic encephalopathy.

There are five new PET tracers under development.  The report says:

“[The] new tracers…appear at first glance to be able to overcome the limitations of the earlier compounds. In general, the newcomers boast higher brain uptake and more specific binding, yielding cleaner-looking scans with sharper distinction between positive and negative findings. While the older tracers work only in AD, some of the new ones appear to light up other tauopathies, as well. Researchers at Piramal Imaging wowed the crowd with scans showing a distinct, specific pattern of binding of their tracer in progressive supranuclear palsy (PSP) compared to AD.”  (Check out the online version of the article for AD vs. PSP images.)

The first-generation tau PET tracers described in the report are:  Lilly/Avid’s AV-1451 (flortaucipir) and THK5351, discovered at Tohoku University in Sendai, Japan, and licensed by GE Healthcare for commercial distribution.  The report indicates that both tracers have lots of problems.

As a result, many researchers are “now eyeing Merck’s and Piramal’s [tracers]. … Merck reported on their tau PET tracer, MK-6240, at the Human Amyloid Imaging (HAI) meeting held January.”  Other companies working on tau ligands include Genentech, Roche, and Janssen.

“Piramal started a Phase 1 trial on four people with AD, three with PSP, and two healthy controls. … Notably, AD and PSP scans revealed distinct patterns. In PSP, only a few discrete regions, mainly the pallidum and substantia nigra, lit up. In contrast, AD patients took up tracer in broader areas known to accumulate tau tangles, such as the lateral temporal lobe, hippocampus, entorhinal cortex, and precuneus.  Curiously, one of the AD patients had a negative tau scan. Stephens noted this patient had mild AD, with an MMSE of 26, and may not have accumulated much pathological tau yet. Incidentally, other PET experts, too, noted that as more research groups image both amyloid and tau pathology in the same cognitively impaired people, they are finding a few whose scans are amyloid-positive but tau-negative.”

Here’s a link to the full report:


Next-Generation Tau PET Tracers Strut Their Stuff
Series – AD/PD 2107 Draws Record Number of Scientists To Vienna
14 Apr 2017
by Alzforum



PSP mentioned on “Chicago Med” TV show

I received this email on Sunday from Storme, one of the founding members of our local progressive supranuclear palsy (PSP) support group:

You’ve probably already heard this, but I was watching last week’s “Chicago Med,” and one of the patients had PSP!  Her diagnosis was sort of secondary to her personal story; she was afraid her boyfriend would vamoose upon learning she would “lose swallowing, speech and brain function.”  So nothing especially helpful, but I was pleased to hear it mentioned on a popularly-watched TV show nonetheless.

FTD Disorders Registry is now live

The FTD Disorders Registry is an online database to collect information from those affected by all types of Frontotemporal Degeneration: behavioral variant FTD (bvFTD), any one of the primary progressive aphasias (PPA), progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), or FTD with motor neuron disease (also called FTD-ALS). Persons diagnosed, caregivers (current/former), family, and friends can join and tell your story.

Brain Support Network encourages all those affected by PSP and CBD to join the registry.


Even if your family member has passed away, you can still join the registry and tell your story.


Excerpts on PSP and CBD in “The Dementias” (NIH online-only booklet)

This email may be of interest to those dealing with the dementia forms of PSP and CBD.  (Not everyone with these diseases has dementia.  To read about the types of PSP and CBD, look under the “PSP Education” and “CBD Education” pages of the Brain Support Network website.)

The National Institutes of Health (nih.gov) has several publications on neurological diseases.  I recently came across their booklet on “The Dementias,” which includes a section on tauopathies as types of dementia.  Tauopathies are caused by the abnormal accumulation of the protein tau.  Both corticobasal degeneration (CBD) and progressive supranuclear palsy (PSP) are covered.  Other tauopathies addressed include frontotemporal disorders (such as Pick’s) and argyrophilic grain disease (AGD).  (In brain donations we’ve helped with, AGD co-occurs in about 20% of all PSP cases.)

“The Dementias” booklet links to the NIH pages on CBD and PSP.  I think the PSP detail page is quite good (and it’s listed as one of our “Top Resources for PSP”).  I don’t think the CBD detail page is nearly as good.

Here are excerpts from the tauopathies section.  Look at the booklet online for other chapters — risk factors, diagnosis and treatment, etc. — and other types of dementia.




Excerpts from

The Dementias
NIH Online Booklet
Published September 2013 (Last Updated July 2016)

Types of Dementia

Various disorders and factors contribute to the development of dementia. Neurodegenerative disorders such as AD, frontotemporal disorders, and Lewy body dementia result in a progressive and irreversible loss of neurons and brain functions. Currently, there are no cures for these progressive neurodegenerative disorders.

Some types of dementia disorders are described below.


In some dementias, a protein called tau clumps together inside nerve cells in the brain, causing the cells to stop functioning properly and die. Disorders that are associated with an accumulation of tau are called tauopathies.

In AD, the tau protein becomes twisted and aggregates to form bundles, called neurofibrillary tangles, inside the neurons. Abnormal clumps (plaques) of another protein, called amyloid, are prominent in spaces between brain cells and are a hallmark of the disease. Both plaques and tangles are thought to contribute to reduced function and nerve-cell death in AD, but scientists do not fully understand this relationship. It is not clear, for example, if the plaques and tangles cause the disorder, or if their presence flags some other process that leads to neuronal death in AD.

Other types of tauopathies include the following disorders:

Corticobasal degeneration (CBD) is a progressive neurological disorder characterized by nerve-cell loss and atrophy (shrinkage) of specific areas of the brain, including the cerebral cortex and the basal ganglia. The disorder tends to progress gradually, with the onset of early symptoms around age 60. At first, one side of the body is affected more than the other side, but as the disease progresses both sides become impaired. An individual may have difficulty using one hand, or one’s hand may develop an abnormal position.

Other signs and symptoms may include memory loss; trouble making familiar, focused movements (apraxia) such as brushing one’s teeth; involuntary muscular jerks (myoclonus) and involuntary muscle contractions (dystonia); alien limb, in which the person feels as though a limb is being controlled by a force other than oneself; muscle rigidity (resistance to imposed movement); postural instability; and difficulty swallowing (dysphagia). People with CBD also may have visual-spatial problems that make it difficult to interpret visual information, such as the distance between objects.

There is no cure for CBD. Supportive therapies are available to reduce the burden of certain symptoms. For example, botulinum toxin can help control muscle contractions. Speech therapy and physical therapy may help one learn how to cope with daily activities.

Frontotemporal disorders (FTD) are caused by a family of brain diseases that primarily affect the frontal and temporal lobes of the brain; they account for up to 10 percent of all dementia cases. Some, but not all, forms of FTD are considered tauopathies. In some cases, FTD is associated with mutations in the gene for tau (MAPT), and tau aggregates are present. However, other forms of FTD are associated with aggregates of the protein TDP-43, a mutated protein found among people with a type of ALS that is inherited. Mutations in a protein called progranulin may also play a role in some TDP43-opathies.

In FTD, changes to nerve cells in the brain’s frontal lobes affect the ability to reason and make decisions, prioritize and multitask, act appropriately, and control movement. Some people decline rapidly over 2 to 3 years, while others show only minimal changes for many years. People can live with frontotemporal disorders for 2 to 10 years, sometimes longer, but it is difficult to predict the time course for an affected individual. In some cases, FTD is associated with progressive neuromuscular weakness otherwise known as amyotrophic lateral sclerosis (ALS, or Lou Gehrig’s disease). The signs and symptoms may vary greatly among individuals as different parts of the brain are affected. No treatment that can cure or reverse FTD is currently available.

Clinically, FTD is classified into two main types of syndromes:

* Behavioral variant frontotemporal dementia causes a person to undergo behavior and personality changes. People with this disorder may do impulsive things that are out of character, such as steal or be rude to others. They may engage in repetitive behavior (such as singing, clapping, or echoing another person’s speech). They may overeat compulsively; lose inhibitions, causing them to say or do inappropriate things (sometimes sexual in nature); or become apathetic and experience excessive sleepiness. While they may be cognitively impaired, their memory may stay relatively intact.

* Primary progressive aphasia (PPA) causes a person to have trouble with expressive and receptive speaking—finding and/or expressing thoughts and/or words. Sometimes a person with PPA cannot name common objects. Problems with memory, reasoning, and judgment are not apparent at first but can develop and progress over time. PPA is a language disorder not to be confused with the aphasia that can result from a stroke. Many people with PPA, though not all, develop symptoms of dementia. In one form of PPA, called semantic PPA or semantic dementia, a person slowly loses the ability to understand single words and sometimes to recognize the faces of familiar people and common objects.

Other types of FTDs include:

* Frontotemporal dementia with parkinsonism linked to chromosome 17 (FTDP-17), a rare form of dementia that is believed to be inherited from one parent and is linked to a defect in the gene that makes the tau protein. The three core features are behavioral and personality changes, cognitive impairment, and motor symptoms. People with this type of FTD often have delusions, hallucinations, and slowness of movement and tremor as seen in Parkinson’s disease. Typical behavioral/personality characteristics include apathy, defective judgment, and compulsive and abusive behavior. Diagnosis of the disorder requires the confirmed presence of clinical features and genetic analysis. Palliative and symptomatic treatments such as physical therapy are the mainstays of management.

* Pick’s disease, a tauopathy subtype of FTD characterized by hallmark Pick bodies—masses comprised of tau protein that accumulate inside nerve cells, causing them to appear enlarged or balloon-like. Some of the symptoms of this rare neurodegenerative disorder are similar to those of AD, including loss of speech, inappropriate behavior, and trouble with thinking. However, while inappropriate behavior characterizes the early stages of Pick’s disease, memory loss is often the first symptom of AD. Antidepressants and antipsychotics can control some of the behavioral symptoms of Pick’s disease, but no treatment is available to stop the disease from progressing.

Progressive supranuclear palsy (PSP) is a rare brain disorder that damages the upper brain stem, including the substantia nigra (a movement control center in the midbrain). This region also is affected in Parkinson’s disease, which may explain an overlap in motor symptoms shared by these disorders. Eye movements are especially affected, causing slow and then limited mobility of the eye. The most common early signs and symptoms include loss of balance, unexplained falls, general body stiffness, apathy, and depression. A person with this type of dementia may suddenly laugh or cry very easily (known as pseudobulbar affect). As the disorder progresses, people develop blurred vision and a characteristic vacant stare that involves loss of facial expression. Speech usually becomes slurred, and swallowing solid foods or liquids becomes difficult. PSP gets progressively worse, but people can live a decade or more after the onset of symptoms. Dextromethorphan, a common ingredient in cough medicine, has been approved for the treatment of pseudobulbar affect.

Argyrophilic grain disease is a common, late-onset degenerative disease characterized by tau deposits called argyrophilic grains in brain regions involved in memory and emotion. The disease’s signs and symptoms are indistinguishable from late-onset AD. Confirmation of the diagnosis can be made only at autopsy.

Blog by Debra Ford, whose husband Dave has PSP

In skimming a Facebook page on PSP, I came across Debra Ford’s blog.  See:


Her blog is titled “The Reality of the Battle.”  The tag line is:

“When warriors go down on their kneeds, the battle is not over.  It has just begun.”

Debra’s husband Dave was diagnosed in 2006 with Alzheimer’s, and in 2009 the diagnosis changed to progressive supranuclear palsy (PSP).  In late August 2016, Debra started a blog.  She posts a few times each month.   We aren’t told where they live but they are definitely in the US as she mentions the VA and YMCA in one post.

Here’s a recent post (February 4, 2017) I liked a lot:

4 neurologists
3 family doctors
4 physical therapists
1 personal trainer
2 speech therapists
3 wheelchairs
1 scooter
2 gyms
2 walkers
1 wheelchair carriers
2 sliding boards
1 toilet lift
1 portable toilet
2 personal lifts
1 traposize
3 gaitbelts
10 years
unlimited family love and support”

Her first post is copied below.



Posted by: Debra Ford
Monday, August 29, 2016

The battle started ten years ago when Dave was misdiagnosed with Alzheimer disease. It would be three years before we received the correct diagnosis of PSP (Progressive Supernuclear Palsey). The disease attacks the part of the brain that controls movement. It has many of the Parkinson Disease symptoms. For disease details go to http://www.ninds.nih.gov/disorders/psp/detail_psp.htm.  For those who know and love Dave, it is heartbreaking to watch. For Dave progressive means him losing his ability to walk, swallow, see, speak, slowly and plateauing over the past ten years.  The plateaus give us a small period of relief and brief hope that he may not get worse. The hope is false hope, the only true hope is in grace of God, that he will provide the strength and knowledge we need until Dave goes home with the Lord. This battle has taught me how to cope with what is happening to Dave, how to be a prayer warrior, and I pray make me a better person. Thank you for following our story. I pray by sharing our story, his west coast family and those here who love him will understand how brave Dave is in his fight to stay mobile and participate in our everyday life.

Johns Hopkins Overview of Progressive Supranuclear Palsy

Johns Hopkins University has an online health library with info about various conditions.  I read about their page on progressive supranuclear palsy (PSP) from a Facebook (FB) page.  Here’s a link to the Johns Hopkins overview of PSP:


There were mostly negative comments on the FB page; many readers felt that the negative aspects of PSP were under-emphasized.

My opinion:  This seems like a reasonable overview of PSP, especially for a family in the early stages.



What is progressive supranuclear palsy?
Johns Hopkins University

Progressive supranuclear palsy (PSP) is a complex condition that affects the brain.

* Progressive means that the condition’s symptoms will keep worsening over time.
* Supranuclear refers to the region of the brain affected by the disorder — the section above 2 small areas called nuclei.
* Palsy is a disorder that results in weakness of certain muscles.

PSP affects your ability to walk normally by impairing your balance. It also affects the muscles controlling your eyes, making it difficult to focus and see things clearly.

Progressive supranuclear palsy is rare. It may be easily mistaken for Parkinson disease, which is much more common and has similar symptoms. But with PSP, speech and difficulty swallowing are usually affected more significantly than with Parkinson disease. Problems moving the eyes, especially problems looking downward, are also more common in PSP. And unlike people with Parkinson disease, people with PSP are more likely to lean backward (and fall backward) rather than forward.

PSP is more common in men than women. Most of the time, it affects people in late middle age or older.

Although PSP isn’t fatal, symptoms do continue to worsen and it can’t be cured. Complications that result from worsening symptoms, such as pneumonia (from breathing in food particles while choking during eating), can be life threatening.

What causes progressive supranuclear palsy?

Although experts basically understand how PSP happens, they don’t understand why it happens. PSP occurs when brain cells in an area of the brain stem become damaged, but how and why these cells are damaged isn’t clear.

What are the symptoms of progressive supranuclear palsy?

Symptoms of PSP tend to start out subtly. Then over time they become more noticeable and severe. Often, the first sign is a problem with balance while walking. You may fall a lot or find that you feel a bit rigid or uncomfortable when you walk.

These are also early signs of PSP:

* Becoming more forgetful and cranky
*Having unusual emotional outbursts, like crying or laughing at unexpected times
* Becoming angry for no real reason
* Tremors in the hands
* Trouble controlling eye movements
* Blurred vision
* Slurred speech
* Trouble swallowing
* Dementia
* Depression
* Trouble directing your eyes where you want them to go
* Inability to control the eyelids, such as unwanted blinking or being unable to open your eyes
* Trouble holding someone’s gaze

How is progressive supranuclear palsy diagnosed?

A careful evaluation of symptoms can diagnose PSP. But it is often hard to diagnose in its early stages as it may mimic Parkinson disease or an inner ear infection. This is because balance is so affected by PSP. Diagnosis usually includes ruling out other conditions.

Balance problems and changes in gait are the clearest symptoms that can identify PSP, particularly when combined with an inability to control or move the eyes.

How is progressive supranuclear palsy treated?

While there is no medicine or procedure available to cure PSP or completely control its symptoms, there are strategies and methods that can help manage many of the symptoms.

To improve balance and improve flexibility of the muscles, medicines used to treat Parkinson disease may be effective. These include the medicine levodopa, which may be used along with other medicines. Some of the older types of antidepressants, such as amitriptyline, fluoxetine, and imipramine, can also help relieve symptoms.

If you have PSP, you may be able to use certain aids to make life easier. For example:

* Special glasses with prisms may improve your vision.
* A weighted tool that helps you walk more easily can prevent you from falling backward.
* Physical therapy and exercise may slightly improve flexibility in some people.

When symptoms are advanced and swallowing becomes too hard, you may need a feeding tube. This tube goes from an opening made in the skin of your abdomen into the stomach and provides you with needed nutrition.

What are the complications of progressive supranuclear palsy?

PSP can cause serious complications when symptoms affect your ability to swallow. You could easily choke on food or breathe food into your lungs. And being more likely to fall increases the risk of suffering a serious injury to the head or breaking a bone.

Living with supranuclear palsy

Although there is no known cure, medicines and devices can help you live with the symptoms. Work with your healthcare provider to find ways to make walking safer and improve your vision. PSP is not fatal but it is important that you do not breathe in food particles (aspirate) while you are eating because it could be life threatening.

When should I call my healthcare provider?

Although it’s easy to try to brush off initial symptoms as being a little clumsy or maybe having an ear infection, it’s a good idea to see a doctor at the earliest sign of symptoms, especially if you have problems with your eyes or vision.

Always seek advice from your healthcare provider if you or your caregiver notice sudden or significant changes in your symptoms.

Key points about supranuclear palsy

* Experts are still working to understand more about progressive supranuclear palsy and find more effective ways to treat it.
* Although the disease itself isn’t life threatening, its complications can be.
* Be aware of suspicious symptoms and talk with your healthcare provider if you notice any problems with your eyes, vision, or balance.

Next steps

Tips to help you get the most from a visit to your healthcare provider:

* Know the reason for your visit and what you want to happen.
* Before your visit, write down questions you want answered.
* Bring someone with you to help you ask questions and remember what your provider tells you.
* At the visit, write down the name of a new diagnosis, and any new medicines, treatments, or tests. Also write down any new instructions your provider gives you.
* Know why a new medicine or treatment is prescribed, and how it will help you. Also know what the side effects are.
* Ask if your condition can be treated in other ways.
* Know why a test or procedure is recommended and what the results could mean.
* Know what to expect if you do not take the medicine or have the test or procedure.
* If you have a follow-up appointment, write down the date, time, and purpose for that visit.
* Know how you can contact your provider if you have questions.

Multimodal Imaging Ties Tau to Neurodegeneration, and Symptoms

This is an Alzforum (alzforum.org) article about important researcher into tauopathies by researchers at Mass General.  The article was posted last week to Alzforum; the research study was published online in JAMA Neurology a couple of weeks ago.

What the researchers confirmed is that there is a “tight correlation between tau neurofibrillary tangles and neurodegeneration in individual patients in early clinical stages of various forms of Alzheimer’s disease.”  Three patients with typical Alzheimer’s Disease (AD) were studies, and three patients with atypical AD were studied.  One of the “atypical AD” cases was a person with corticobasal syndrome (CBS)

In this study, all patients were given a tau PET scan, an amyloid PET scan, and an MRI.  Researchers found that “tau predicts atrophy [which] predicts symptoms.”  It is not the protein amyloid in the brain that predicts atrophy or predicts symptoms.

In fact, we have known this from brain donation for a long time but now researchers have confirmed this in living patients.

Perhaps one reason that a CBS patient was studied rather than a PSP (progressive supranuclear palsy) patient is that the tau load in CBD is greater than in PSP.

Here’s a link to the article:


Multimodal Imaging Ties Tau to Neurodegeneration, and Symptoms
07 Mar 2017

It is challenging reading.  Check it out online for cool images of the patient with corticobasal syndrome.