IOA Director presents at 2016 Söderberg Prize Seminar, which recognized the first Alzheimer’s disease researcher to receive the award

Earlier this month, IOA Director John Q. Trojanowski, MD, PhD, among others, presented a lecture on “Experimental Transmission of Tau Pathology: Implications for Diagnosis and Therapy” at the 2016 Söderberg Prize Seminar at the Swedish Society of Medicine in Stockholm, Sweden.

Screen Shot 2016-04-28 at 2.06.05 PMThis Seminar recognized Kaj Blennow, MD, PhD, professor at the University of Goteborg, Sahigrenska University Hospital, and the first Alzheimer’s disease researcher to receive this award. Queen Silvia of Sweden, an avid Alzheimer’s disease research advocate was in attendance to present the award to Dr. Blennow (pictured).

 

In 2013, the Queen Sylvia Nursing Award was launched in Her Majesty’s (H,M.) name as a way to encourage nursing students to become more interested and involved in research on dementia. “The aim is to support young, driven people with creative ideas that can question convention in how we care for the elderly and dementia patients,” explained H.M. Queen Silvia in an interview with AARP’s The Journal.

To learn more about the Queen Silvia Nursing Award, click here.

To view the full Söderberg Prize Seminar 2016 Program, click here.

Photos courtesy of the Swedish Society of Medicine and the Prize Jury.

Penn kicks off REACT! Study: The Rhythm Experience and Africana Culture Trial

Last week, the University of Pennsylvania officially kicked off their first round of The Rhythm Experience and Africana Culture Trial, also known as the REACT! Study, led by Kathy Jedrziewski, PhD, Deputy Director of the Institute on Aging. This Alzheimer’s Association-funded study is being conducted in collaboration with the University of Pittsburgh’s Brain Aging & Cognitive Health (BACH) Lab, led by the study’s Principal Investigator Kirk I. Erickson, PhD, Associate Professor, Department of Psychology at the University of Pittsburgh.

This research study will compare two activities, African Dance and an Education/Discussion group, which Drs. Jedrziewski and Erikson believe could be beneficial for older African Americans (ages 65-75). The study will examine whether brain health, fitness levels or quality of life improve as a result of participating in these activities three times per week for six months.

However, after the first week of classes, participants are already praising the benefits. With just a few short days under their belts, several members from both the education and dance groups said they have already learned so much and look forward to seeing what is to come. This study not only gives them the opportunity to enhance their socialization and perhaps their physical and cognitive health, but it also allows them to help others in the aging African American community through this research by providing evidence about potentially effective ways to improve and maintain quality of life as we age.

 

While the study has already started, coordinators will continue to recruit on a rolling basis. If you or anyone you know is interested in participating in the REACT! Study, contact:

Philadelphia – Penn Campus
Shardae Williams, Project Coordinator
215-573-8153

Pittsburgh – The BACH Lab at the University of Pittsburgh
412-302-6679

For more information, visit: www.med.upenn.edu/aging/react.html

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Joseph A. Pignolo Award in Aging Research 2016: “REST and Stress Resistance in Aging and Alzheimer’s Research”

On Tuesday, March 1, 2016, the Institute on Aging presented their annual Joseph A. Pignolo Award in Aging Research. This year’s awardee was Bruce A. Yankner, MD, PhD, professor of Genetics and Neurology and Co-director of the Glenn Center for the Biology of Aging at Harvard Medical School, for his 2014 publication in Nature on “REST and Stress Resistance in Aging and Alzheimer’s Research.

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John Q. Trojanowski, MD, PhD (IOA Director), Bruce A. Yankner, MD, PhD (2016 Pignolo Awardee), and Robert J. Pignolo, MD, PhD (founder of the Pignolo Award in Aging Research).

This paper analyzes the gene expression changes that occur in the aging brain and shows the coherent pattern of changes in genes that turn on or off in the neurons of the brain as it ages. The greatest impact was seen in the REST (RE1 neuron-silencing transcription factor) gene. It was previously thought that this gene only functioned in fetal brain development—keeping neural genes at bay until the brain had a chance to build its underlying architecture—however, Dr. Yankner and his team found that it is also expressed in the adult human brain and is dramatically up-regulated in neurons as the brain ages. The significance of this in neurodegenerative research is that they discovered that in the brains of individuals with Alzheimer’s disease, the protein is actually much less up-regulated, or completely absent.

Using both mouse models and culture dishes in a laboratory, they found that regular stressors encountered by an aging brain such as oxidative stress—a disturbance in the balance between the production of reactive oxygen species and antioxidant defenses—and amyloid stress associated with AD had a significant impact on sustaining the REST gene.

“This was a galvanizing observation for us,” explained Dr. Yankner. “It suggested that some people can resist the onslaught of Alzheimer’s because they’re able to up-regulate this intrinsic defense mechanism [REST]. So, a very important question is why some people can do it and some people can’t…”

Dr. Yankner assumes there is a potential genetic underpinning, but also believes that environmental factors contribute as well.

In terms of future research, and based on their current findings, Dr. Yankner and his lab are interested in understanding exactly how REST accomplishes its different functions and manages to maintain neurons in a functional state for so many years. To do this, they are characterizing all of the genes and protein partners that interact with REST and are looking at them as potential therapeutic targets that may be used to delay the onset of Alzheimer’s disease.

View more photos from the 2016 Joseph A. Pignolo Award in Aging Research.

*This study was funded by the National Institute on Aging, the National Institutes of Health Common Fund (NCF), and the Paul F. Glenn Foundation for Medical Research.

 The main focuses of Dr. Yankner’s lab are to understand 1) the molecular biology of the aging brain and how that intersects with pathological aging in diseases such as Alzheimer’s disease (AD), Parkinson’s disease (PD) and Frontotemporal degeneration (FTD) and 2) using humans as a model system by understanding how they age in the brain, from changes in genes, DNA, and proteins, and modeling this in cells in culture and genetically engineered model systems including C. elegans (Caenorhabditis elegans) nematode worms.

The Joseph A. Pignolo, Sr. Award in Aging Research is given out as part of the Institute on Aging (IOA) Visiting Scholars series to annually recognize an outstanding contribution to the field of biogerontology. It was created by geriatrician and gerontologist Robert J. Pignolo, M.D., Ph.D. in honor of his father.

Recognizing MSA: Multiple System Atrophy

What is Multiple System Atrophy (MSA)?

According to the National Institute of Neurological Disorders and Stroke (NINDS), MSA is a “progressive neurodegenerative disorder characterized by a combination of symptoms that affect both the autonomic nervous system (the part of the nervous system that controls involuntary action such as blood pressure or digestion) and movement. The symptoms reflect the progressive loss of function and death of different types of nerve cells in the brain and spinal cord.”

Symptoms may include:

  • Fainting spells
  • Problems with heart rate
  • Bladder control
  • Tremors
  • Loss of muscle coordination
  • Speech problems
  • Gait impairment
  • Rigidity

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Like many other neurodegenerative diseases, some symptoms of MSA can be treated with medications, but there is currently no cure or means of halting progression.

MSA vs. Parkinson’s Disease

While many symptoms can mimic those of Parkinson’s disease (PD), often making it difficult to distinguish a diagnosis in the early stages, MSA typically progresses much more rapidly than PD. Within a few years of initial symptoms, majority of MSA patients will require a cane or walker to aid in getting around.

Moving Towards a Cure

Researchers at Penn, and outside of Penn, continue to work vigorously for answers that will help benefit and improve the lives of those suffering from MSA.

“I would say that, although research in any rare disease is challenging, there have been advances in MSA, from the generation of animal models that are essential to understand the disease and develop new therapies, to several clinical trials that have already been completed in patients with this disorder,” explained Pedro Gonzales-Alegre, MD, PhD, associate professor of Neurology, Division of Movement Disorders at the University of Pennsylvania. “There are also efforts to develop better imaging biomarkers that will help us better diagnose MSA and monitor disease progression of the disease in clinical trials.”

In order to continue in this upward direction, raising awareness, knowledge, and advocacy becomes increasingly important. One of the key players in MSA awareness is CurePSP, a close partner of Penn Medicine. CurePSP is a nonprofit advocacy organization whose mission is to increase the public’s knowledge of prime of life diseases such as progressive supranuclear palsy (PSP), Corticobasal degeneration (CBD), and MSA. They define a prime of life disease as a condition that often strikes during a person’s most productive and rewarding years.

The video below, courtesy of CurePSP, shares the touching story of James Wark, MD, a child neurologist, who is now living with Multiple System Atrophy, and the struggles that he and his family have been faced with since his diagnosis.

 

Multiple System Atrophy Awareness Month

March is Multiple System Atrophy Awareness Month. Show your support on Facebook and Twitter with the banner and message below:

March is #MSAAwarenessMonth! Help spread the word and learn more about #MSA here: http://bit.ly/1LqYaTM

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Corticobasal Degeneration: When it looks, sounds, and acts like Parkinson’s… but it’s not.

What is Corticobasal Degeneration?

Corticobasal degeneration (CBD) is often misdiagnosed as Parkinson’s disease. As defined by the
National Institute of Neurological Disorders and Stroke (NINDS), CBD is a neurological disorder that is characterized by cerebralcortex_basalganglianerve cell loss and shrinkage of multiple areas of the brain including the cerebral cortex and basal ganglia. Much like other neurodegenerative diseases, CBD is progressive, gradually worsening over time.

CBD classically presents itself as a movement disorder, typically affecting one side of the body before the other, but can also impact cognition. Since some symptoms can mirror those of Parkinson’s, one of the most useful signs of Corticobasal degeneration is apraxia. Apraxia refers to an individual’s inability to perform certain movements not because of physical muscle damage, but due to neurological damage.

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Other symptoms include difficulty controlling the muscles of the face and mouth, stiffness, shakiness, and slowness in either the upper or lower extremities, difficulty with speech and articulation, and memory and/or behavioral problems. These symptoms usually occur around the age of 60, although, symptom onset can be as early as 40.

“CBS (corticobasal syndrome) and Parkinson’s disease can clinically be difficult to distinguish but have very different underlying pathologies,” explained David Irwin, MD, Instructor of Neurology, University of Pennsylvania. “Current research efforts are focused on differentiating the underlying diseases (differentiating inclusions formed from alpha-synuclein, tau, amyloid and TDP-43) to help direct promising therapies targeting these proteins.”

While occupational, physical, and speech therapy can help manage CBD, there is currently no cure or treatment to slow the progression of the disease, and most symptoms are generally resistant to possible therapeutic strategies.

You can find more information on CBD at:

National Institute of Neurological Disorders and Stroke

Penn’s Frontotemporal Degeneration Center

CurePSP

The Association for Frontotemporal Degeneration

Frontotemporal Degeneration: It’s more than just dementia

What is Frontotemporal Degeneration (FTD)?

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FTD is an aging-related neurodegenerative disease affecting the frontal and temporal lobes of the brain, disrupting a variety of functions such as behavior, personality, language, movement, and in some cases, cognition.

According to the Association for Frontotemporal Degeneration (AFTD), the most telltale sign of FTD is a gradual and progressive decline in behavior and/or language. As the disease progresses, planning and organizing activities, behaving appropriately in public settings, and socializing generally become increasingly difficult for individuals with FTD.

The disease often occurs in a person’s 50s or 60s, but has been seen as early as 21 and as late as 80. It is the most common form of dementia in people under 60 years old.

Is FTD inherited?

When asked during a past Q&A with the Boston Globe, Murray Grossman, MD, director of Penn’s FTD Center, explained that FTD can be inherited, but that it is not typically the case. “Twenty-five percent of the patients we see with FTD have a high-risk family history,” he said, but the remaining 85 percent are considered “sporadic FTD.” In sporadic FTD, there is no known cause—genetic or environmental—and researchers are still searching for any signs of genetic changes that may be responsible for these random cases.

Is FTD curable?

There is currently no cure or treatment for FTD.

Some medications such as antidepressants, neuroleptics (antipsychotics), and dopaminergic agents used in Parkinson’s disease, can be prescribed to help manage symptoms, but should be treated on a case-by-case basis as there is always a risk for worsening symptoms, adverse reactions, and other unfavorable side effects.

A Family’s FTD Story

The video below, produced by CurePSP, tells the story of artist David Wetzl and his journey with Frontotemporal degeneration and gives a first-hand look at exactly what this disease can strip from you in your prime of life.

To learn more about FTD, visit:
The Penn FTD Center
The Association for Frontotemporal Degeneration


RELATED EVENT:

Penn FTD Center Caregiver Conference

 On May 20, 2016, the University of Pennsylvania’s Frontotemporal Degeneration Center will host their Annual FTD Caregiver Conference. This day-long event is for caregivers and anyone else interested in learning more about FTD and related conditions such as Amyotrophic lateral sclerosis (ALS or Lou Gehrig’s disease) and Corticobasal Degeneration (CBD).

This event is free and open to the public. Registration is required and opens on April 8, 2016.

Event Details:
Friday, May 20, 2016
8:00am – 4:30pm
Smilow Center for Translational Research
3400 Civic Center Boulevard
Philadelphia, PA 19104

For more information on the event, including the agenda, participating advocacy groups, and giving opportunities, please contact:
Christine Ray at rayc@mail.med.upenn.edu or (215) 349-5873

Out of town? If you plan on traveling to Philadelphia for the conference from another city or state, please visit the University of Pennsylvania’s visitor page for information on local accommodations.

To see coverage from last year’s 2015 FTD Caregiver Conference, click here.

Progressive Supranuclear Palsy: A “prime of life disease”

Progressive supranuclear palsy (PSP) is a rare brain disorder that affects the nerve cells in the brain that control walking, balance, mobility, vision, speech, and swallowing and gradually worsens over time. It affects an estimated three to six in every 100,000 people worldwide—or approximately 20,000 Americans—according to the National Institute of Neurological Disorders and Stroke (NINDS).

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While one of the most classic identifying symptoms for PSP is the inability to aim and move the eyes properly, the most frequent first symptom to arise is the loss of balance while walking. This can appear in the form of unexplained falls, stiffness, or awkwardness in gait.

Some symptoms are very similar to those in Parkinson’s disease, including stiffness, movement problems, and clumsiness for example, but PSP, true to its name, progresses much quicker. Other distinctions between the two disorders are seen symptomatically through things like postural differences—people with PSP tend to stand exceptionally straight or with their head tilted backwards whereas people with PD are typically bent forward—while others have more to do with the pathology of the disease. In PSP, the most prominent protein accumulation seen in the brain is tau, but the most prominent protein found in a Parkinson’s brain is alpha-synuclein.

Living with Progressive Supranuclear Palsy

In the CurePSP produced video below, hear one family’s touching story of how a PSP diagnosis has changed their lives.

A close partner of the neurodegenerative disease-related centers here at Penn, CurePSP identifies as one of the leading nonprofit advocacy organization focused on prime of life diseases with the goal of understanding their causes and discovering potential treatments, and has funded more than 160 research studies. 

* CurePSP recognizes the following as prime of life diseases: Progressive Supranuclear Palsy (PSP), Corticobasal Degeneration (CBD), Primary Progressive Aphasia (PPA), Motor Neuron Disease (MND), Lewy Body Dementia (LBD), Frontotemporal Degeneration (FTD), Amyotrophic Lateral Sclerosis (ALS), Parkinson’s Disease (PD), Alzheimer’s Disease (AD), and  Multiple System Atrophy (MSA).