Hybrid Neurologists Care for Crossover Neurodegenerative Diseases

In honor of today, Rare Disease Day, we’re looking at a few of the rare neurodegenerative diseases. Do you know someone with a rare disease? What have been your greatest challenges?

Amyotrophic  Lateral Sclerosis (ALS) is linked to Frontotemporal Lobar Degeneration (FTLD). Parkinson’s disease (PD) is linked to Alzheimer’s disease (AD). Progressive Supranuclear Palsy (PSP) is caused by an abnormal accumulation of tau, a protein that plays a role in AD.

It may sound like an alphabet soup of diseases, but these intertwined diseases are changing the way we look at, and hope to treat, progressive neurodegenerative diseases attacking the brain.

For diseases historically considered in the domain of distinct neurological sub-specialties – movement disorders, neuromuscular conditions, and dementia – the steady increase in our understanding of their overlapping causes and symptoms, as well as their co-existence in the same individual, has led to a shift in how care is delivered. Physicians, nurse practitioners, therapists, and other care-team members are cross-training and collaborating more than ever.

Rachel Gross, MD, now an assistant professor of Neurology in the Perelman School of Medicine at the University of Pennsylvania, noticed the overlap as she completed advanced training in Neurology. She decided to get dual-trained in both Cognitive Neurology and Movement Disorders while completing her fellowship at Penn. “I realized that patients who suffer from diseases characterized by a movement disorder and dementia would appreciate seeing one physician who can address both their motor and cognitive issues.”

At the recent FTLD Caregiver Conference, hosted by the Penn FTLD Center, we saw how neurological sub-specialties are blending, overlapping, and informing each other. For instance, researchers are now considering FTLD and ALS to be on the same spectrum of disease. Some people may start with the physical stiffness seen in Lou Gehrig’s disease, while someone else – with the same underlying protein markers – starts with FTLD-symptoms like behavioral outbursts or language mix-ups.

Some of the less common neurodegenerative diseases are also informing the more common diseases. Last year, we reported genetic clues on risk factors and biological causes of a rare neurodegenerative disease called progressive supranuclear palsy (PSP). Biologically, PSP is primarily caused by an abnormal accumulation of tau protein, which is well-known for its secondary role in Alzheimer’s disease.

Pieces of research – genetics, biomarker tests, imaging, pathology, and clinical symptoms – are starting to come together to give us a better picture of neurodegenerative disease. The team of researchers and clinicians, at Penn and beyond, are in a race to find a cure. The more experts battling neurodegenerative diseases, the better!

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Persistence, Dedication on Display as Penn Medicine Aging Researchers Visit Ugandan Colleagues

A trio of Penn Medicine researchers recently traveled to Uganda for a conference on brain diseases, bringing together local and international experts to talk about the latest research impacting brain health in Sub-Saharan Africa.

I talked with John Trojanowski, MD, PhD, director of Penn’s Institute on Aging and co-director of the Center for Neurodegenerative Disease Research, about the trip and discussed the impact this journey had on the continuing quest to find diagnostic tools and treatments for neurodegenerative diseases such as Alzheimer’s disease.

Photo by Stan Jacobson

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Hope on Horizon for FTLD

For families touched by Frontotemporal Lobar Degeneration (FTLD), or any disease that has no cure, a cure can’t be found soon enough.

At our recent FTLD Caregiver Conference, we caught up with Murray Grossman, MD, EdD, director of the Penn FTLD Center, to ask him about the progress in FTLD research. His comments during the conference were very promising, saying that “we’re on the cusp of disease-modifying drugs,” for frontotemporal lobar degeneration, with a goal of having a treatment for FTLD in 10 years.

In this video, Dr. Grossman explains the latest biological understanding of the disease, improvements in diagnostic tools, and efforts towards finding treatments that modify or stop the progression of FTLD and related diseases.

If you’re interested in clinical trial information for FTLD, visit the Penn FTLD Center website, or check back here for updates.

Caring for Caregivers: Conference Highlights Challenges for FTLD Caregivers

If you’ve never heard of Frontotemporal Lobar Degeneration, consider yourself lucky. If you are familiar with FTLD, you know that it strikes people in their prime – the average age of onset is 58, but ranges from 30 – 82. Over months or years, the neurodegenerative disease progressively causes people to lose control of their speech and language, as well as personality and social functioning. The disease is fatal, and currently has no cure.

Last weekend, Penn’s FTLD Center hosted its annual meeting for the caregivers of people with FTLD, as well as some of the related diseases – Amyotrophic lateral sclerosis (ALS or Lou Gehrig’s disease), Progressive supranuclear palsy (PSP), and corticobasal degeneration (CBD). The caregivers listened to the latest research and understanding of the diseases, and learned from caregiving experts about available resources and important caregiving skills.

Over the next few days, we’ll be posting short interviews from experts and looking into some of the topics covered at the conference – including the overlapping pathology behind these diseases, promising drug candidates from Penn’s drug discovery program, and the struggles caregivers face on a daily basis.

To start, we’ve compiled a transcript of live-tweets from the FTLD Caregiver Conference.

If you attended the conference, what information did you find most fascinating? For me, it was learning that they’re hoping to have an effective treatment for FTLD in 10 years. With an amazing team and the full-spectrum of research underway here at Penn, I don’t doubt it.

Cardiac Health, Education Linked to Brain Health, says Institute on Aging Visiting Scholar

“The percentage of people with cognitive impairment may actually be going down in the U.S.,” explained Kenneth Langa, MD, PhD, a professor of Medicine at the University of Michigan, as he gave the second Institute on Aging Visiting Scholars lecture of the year last week, here at Penn.

Dr. Langa, a primary investigator on the powerful Health and Retirement Study, was describing data comparing rates of cognitive impairment between 1993 and 2002. As wealth and years of education rose between the 1993 and 2002 cohorts, there was a decrease seen in dementia. In that same time frame, cardiovascular disease, diabetes, and obesity rates went up.


The team also compared brain health in America with a similarly-designed British analysis, finding that Americans’ cognitive health, on the whole, was significantly better than the Brits. Dr. Langa suggested that the earlier push to treat hypertension in America and the greater number of people being treated for hypertension in the U.S. may be associated with better cognitive function in the states, despite our higher rates of cardiovascular disease, obesity and diabetes. Future studies will look at whether this pattern, and the gap in brain health levels, have changed as the level of cardiac care equals out.

When comparing the economic costs of caregiving among different groups, the research showed that caregiving costs for dementia are highest (29%), more than depression (15%), stroke (10%), diabetes (10%), and cancer (2%).

The Health and Retirement Study, started in 1992, has collected health and social science data from 24,000 Americans over the age of 50. From a research perspective, the carefully collected publicly-available data has been a powerful tool for scientists, producing nearly 2000 publications by 1000 authors. The group, led out of the University of Michigan, is now looking at how major medical events, such as severe sepsis, hip fracture, or cardiac bypass, can increase cognitive impairment.

Here’s a transcript of our live-tweets during the event. We’ll post the full podcast here when it’s available.

Welcome!

Thanks for stopping by the new Institute on Aging blog, Science of Aging! We’ll be working hard to share the latest research on age-related diseases, updates from IOA lectures and events, and news featuring Penn’s Institute on Aging experts.

We decided to launch our social media efforts to open the doors and invite you in for a look behind the scenes at Penn’s Institute on Aging, where we’re constantly seeking ways to improve age-related conditions, from Alzheimer’s to osteoporosis. Continue reading