UAB Huntington’s Disease Clinic

In January 2011, the UAB Department of Neurology opened a new multidisciplinary Huntington’s Disease Clinic. Our clinic has been named by the Huntington’s Disease Society of America (HDSA) as one of only 22 Huntington’s Disease Centers of Excellence in the United States. Below are highlights of this initiative, including a brief bio of our faculty and staff. Your care is important to us, and we hope that you will find this enhancement of our Huntington’s Disease clinic encouraging.

    sung    Victor W. Sung, MD
Victor W. Sung, MD: Dr. Sung is the Director of the UAB Huntington’s Disease Clinic and graduated from Vanderbilt University with a BS in Neuroscience in 2001. He received an MD from UT-Southwestern in Dallas in 2005. He completed residency training in Neurology at UAB in 2009, during which time he was elected to membership of the Alpha Omega Alpha Honor Society and received the Argus Award for Best Neurology Resident. After residency, he completed a VA National Quality Scholar Fellowship and a Movement Disorders Fellowship at UAB. In addition to directing the Huntington’s Disease Clinic, he is the Stroke Center Director at the Birmingham VA Medical Center and Associate Director of the UAB Medical Student Neurology Clerkship.

david standaert   David G. Standaert, MD, PhD

David G. Standaert, MD, PhD:Dr. Standaert is the Chair of the UAB Department of Neurology, the John N. Whitaker Chair of Neurology, and was recruited from Harvard University in 2006. He received an MD and PhD from Washington University in St. Louis in 1988 and completed residency training at the University of Pennsylvania. At Harvard, Dr. Standaert was involved in research and clinical care for Huntington’s. In addition to being Associate Director of the Huntington’s Disease Clinic, he also serves as Director of the Center for Neurodegeneration and Experimental Therapeutics (CNET) and Director of the Division of Movement Disorders at UAB. 
 jenna  Jenna Smith, RN, BSN
Jenna Smith, RN, BSN: Jenna completed her Bachelor of Science in Nursing at the University of Alabama at Birmingham in 2006. Jenna was a member of the Alabama and National Student Nurses Associations, and was also inducted into the Sigma Theta Tau International Honor Society of Nursing. Upon graduation, Jenna worked in the Neurosciences Intensive Care Unit where she provided care for Neurology, Neurosurgical, and Trauma patients. In 2010, Jenna accepted a position in the Department of Neurology as the Clinical Coordinator for the Huntington’s Disease Clinic and Research Nurse Coordinator for the Division of Movement Disorders. Jenna is available in clinic and will also take all HD-related phone calls and manage general patient issues.

HD Clinic 2016 001
Your UAB Huntington’s Disease Clinic team

Front row (Left to Right): Jenna Smith (Registered Nurse); Dr. Taylor Preston (Psychiatrist); Dr. Victor Sung (Neurologist); Leslie Harper (Speech Therapist);  

Back row (Left to Right): Cindy Sergeant (Occupational Therapist); Fallon Brewer (Genetics Counselor); Gayle Benson (Physical Therapist); Amy Holmes (Social Worker)
work closely together to provide multi-disciplinary care to address all your needs at each visit.

Laura Ellis, MS, LPT is the Physical Therapist for the clinic and will provide PT evaluation and individualized treatment plans for balance and walking during each visit.

Cindy Sargent,OTR/L is the Occupational Therapist for the clinic and will provide OT evaluation and individualized treatment plans for coordination and tasks involving daily basic function during each visit.

Leslie Harper, MS, CCC-SLP is the Speech Therapist for the clinic and will provide speech and language evaluation and individualized treatment plans for speech and swallowing during each visit.

Amy Holmes, LGSW is the Social Worker for the clinic and is familiar with several resources available and can help with social and psychosocial needs.

Fallon Brewer, MS, CGC is the Certified Genetic Counselor and is very involved in the clinic to address any questions related to genetic testing.

We know Huntington’s disease not only affects you as an individual but also your family.   Each member of our team is specialty trained to provide the unique care required for Huntington’s Disease patients. Our goal is to provide the best comprehensive care available to patients while also providing support for your family and loved ones. We look forward to serving you, and please feel free to contact us if there is anything we can do for you.

Jenna Smith, RN, BSN

triebel webKristen Triebel

A University of Alabama at Birmingham researcher has received an American Cancer Society Mentored Research Scholars grant to study long-term decision-making abilities in patients with advanced cancer.Kristen Triebel, Psy.D., assistant professor of neurology and director of the Neuropsychology Fellowship Training Program in the UAB Department of Neurology, has been awarded a five-year, $728,000 grant for her work titled “Decisional Capacity Evaluation in Metastatic Brain Cancer.”

Metastasis, or the spread of a cancer from one organ or disease site to another,to the brain occurs in 25 percent of all individuals with cancer. Due to cognitive impairment, emotional distress and other changes occurring as a result of their severe illness, individuals with brain metastases frequently have reduced capacity to make well-informed decisions about their medical treatment. These decisions may include deciding among focused or whole-brain radiation, surgery, chemotherapy or palliative care, or whether to pursue experimental treatment. Surprisingly, decisional capacity is not routinely assessed in this patient population prior to patients’ consenting to treatment.

The ACS grant allows Triebel to critically study patients with advanced cancer, including lung and breast cancer that has metastasized to the brain, and pancreatic cancer. She also will examine issues in these patients’ treatments, cognitive function and quality of life.

“Patients with advanced cancer are seriously ill and have to make a lot of important medical decisions,” said Triebel, an associate scientist in the Cancer Control and Population Sciences program at the UAB Comprehensive Cancer Center.“We are looking to see whether our initial findings from metastatic cancer patients can generalize to other cancer populations who have comparable levels of illness — even those who don’t get tumors that spread to the brain.”

According to Triebel, the goal is to be able to improve decision-making capacity. “We hope this research leads to better understanding of patients at risk, so we can advise on best practices for assessment and interventions to support patients in this capacity, ultimately improving their quality of life.”

Triebel, who received a Center for Clinical and Translational Sciencegrant in 2012, leveraged that research into this five-year grant from ACS. “This award provides opportunities for me to grow a research program by fostering collaboration with other cancer investigators. And it also allows me to pursue other career-development opportunities.”

At a time when funding opportunities are difficult to attain, Triebel is an example of what researchers need to do to secure additional funding that has the potential to change the future of cancer treatment and care. She emphasizes the protected time and money to conduct the research that the CCTS grant allowed, as well as “access to successful researchers who taught me how to conduct research and write grants,” she said. “That grant was great, providing two years of support that allowed me to do the research, which in turn helped me secure this larger ACS grant.”

Ultimately this work comes down to the patients. “Certainly, this study will address the knowledge gap by investigating medical decision-making capacity in patients; but the great potential is to improve clinical practice and decision-making for these populations,” said Triebel.

By: Beena Thannickal

Three University of Alabama at Birmingham Honors College students won poster awards at the 49th annual National Collegiate Honors Council meeting in November.

The majority of the NCHC’s 900 member institutions participated in the conference. Students from honors programs and colleges nationwide submitted posters in nine categories.

The winner in the business, engineering and computer science category was Lily Deng, a junior University Honors Program and biomedical engineering major mentored by Ho-Wook Jun, Ph.D., associate professor in the Department of Biomedical Engineering, whose presentation was titled “Crosstalk Between Human Mesenchymal Stem Cells and Human Umbilical Vein Endothelial Cells on a Peptide Amphiphile Scaffold.”

The co-winners in the natural sciences and mathematics category were Ranjani Ponnazhagan, a junior University Honors Program neuroscience major mentored by David Standaert, M.D., chairman of the Department of Neurology, whose presentation was titled “Metabotropic Glutamate Receptor 4 Positive Allosteric Modulators Attenuate LPS-Induced Inflammation in Microglia Cells”; and John Decker, a junior University Honors Program neuroscience major mentored by Paul Gamlin, Ph.D., professor in the Department of Ophthalmology, whose presentation was titled “Voluntary Eye-Movements to Cyclopean, Monocularly Invisible Targets.”

Deng and Ponnazhagan are also part of the Early Medical School Acceptance Program.

NCHC 20143 sPhoto credit: Tianjiao ZhangEach poster presentation was judged by a faculty member, typically an honors director or dean from honors colleges and programs throughout the United States and abroad.

“It is quite an achievement for an honors program or college to have a student winner in any of the nine categories and an extremely rare event for a program or school to have multiple winners,” said Michael Sloane, Ph.D., director of UHP. The UAB contingent included 30 honors students as well as faculty and staff from the Honors College and its specialized programs.

“The conference was a wonderful opportunity for our honors students to present their research in a national forum and interact with other honors students from across the country,” said Honors College Dean Shannon Blanton, Ph.D. “We are extremely proud of their achievements and delighted that such accomplished students represent UAB.”

By: Meghan Davis
Seven weeks after weight-loss surgery, a group of women have seen significant changes in their body shapes and sizes. They’re each down 20 to 30 pounds, but that’s not the only change their bodies are going through.

The women’s weight loss is caused by a change in appetite, which results from changes in brain function, explains UAB neuroradiologist Robert Kessler, M.D. (pictured above in UAB's Advanced Imaging Facility). On positron emission tomography (PET) scans, Kessler can see an obvious transformation in the women’s brains, particularly in dopamine neurotransmission.

Using a specialized brain PET scan that he has developed, Kessler can visualize levels of dopamine receptors — molecules that help transmit the brain’s messages of motivation and reward. Before surgery, the women had increased levels of the receptors, which appear on the PET scans as glowing white patches throughout the brain. But after their surgeries, these changes have faded; the women’s brains exhibit a more balanced map of dopamine receptors. In real-world terms, Kessler thinks, these tempered receptor levels reflect a shift to a more normal reward perception, helping the women control their appetites after surgery.

Obesity — and the drive to overeat — isn’t the only pathology that Kessler can see when he peers into people’s brains with a PET scan. During the past 30 years, he has helped illuminate changes to the brain that might underlie schizophrenia, drug addiction, depression and dementia, among other disorders. By looking at a person’s brain PET scan and carefully measuring the levels of neurotransmitter function, Kessler can tell whether someone is more prone to taking risks than average, whether they’re more of a “slacker” or a “go-getter,” and whether or not they have “the ability to experience rewarding stimuli in a normal manner or if they have lost that ability,” he said.

Find out how a cyclotron works, and what makes UAB’s new cyclotron unique among U.S. academic medical centers, in the video above and in this feature from UAB Magazine.
Kessler, who joined the UAB faculty in 2013 as director of neurochemical brain imaging and PET neurotracer development in the Department of Radiology, says these specialized PET scans are paving the way toward a new level of understanding of brain diseases. “At a very basic scientific level, there’s no other technology that can look at the human brain and inform you about specific molecules and receptors,” Kessler said. At UAB, he’s taking advantage of the university’s TR24 cyclotron — the largest at any U.S. academic medical center — to develop new PET scans. And he has launched collaborations with UAB researchers across the psychiatric and neurological sciences to help them apply his techniques to even more questions.

Focusing on Receptors

As a medical student, resident and fellow in the 1970s, Kessler first became interested in the human brain at a time that clinicians had few methods to visualize the organ. Surgeons could physically see the outer layers of the brain when they opened the skull for an operation, or pathologists could dissect an autopsied brain; but viewing the activity — in a living human — of the molecules that make up the brain’s electrical pathways wasn’t possible.

In 1977, Kessler joined a lab at the National Institutes of Health just as this was changing. One of his mentors there became the first to use a PET scan to visualize the activity of the brain. The earliest scans, rather than pinpointing specific receptors as Kessler does now, were designed to simply show which cells in the brain were undergoing metabolism — a sign of activity — at any given moment. But the basic idea has been the same for more than three decades now: A patient gets an injection of a radioactive tracer into their bloodstream. Depending on the design of the tracer, it accumulates in particular organs or cells of the body. Then, a PET machine is used to measure the location of the accumulated radioactivity.

“It quickly became clear to me that PET was going to become an important tool for understanding the brain,” Kessler said. “And we began to use it to look at everything from brain tumors and schizophrenia to aging and dementia.”

As Kessler immersed himself in the new technology, first at NIH and then at Vanderbilt University, he helped develop new tracers that would pave the way for the rest of his career: 18F-Fallypride, and later 18F-FPEB. Rather than building up in all metabolizing brain cells, these radioactive molecules bind specifically to dopamine and glutamate receptors.

Among the first questions Kessler asked with 18F-Fallypride was ‘What are the effects of antipsychotic drugs on the brains of patients with schizophrenia?’ A new class of antipsychotic drug had recently been developed; the drugs had fewer side-effects than older versions, but researchers didn’t know why. Kessler and his collaborators discovered that the new drugs targeted different areas of the brain than the old drugs, offering not only an explanation for the differences, but a way to test future drugs for their efficacy.

Dopamine had also already become known as a chemical that mediates reward-seeking behavior and pleasure. So Kessler’s lab began to look at how levels of the dopamine receptor and the effects of dopamine release on dopamine receptors might relate to drug abuse, impulse control, addiction and the ability to feel pleasure.

“People who are depressed lose their ability to enjoy rewards and experience the pleasures of life; people who are addicted have very distorted reward functions where they crave just one reward,” Kessler said. “We showed that dopamine plays a key role in all of these.”

mix kessler FDG illustrationThese are 18F-Fallypride PET images of dopamine D2 type receptors, averaged across several normal subjects. There are high levels of these receptors (red color) in deep brain structures and lower levels in the cortex. These include the basal ganglia and thalamus (A), amygdala and temporal cortex (B), and substantia nigra (C). These regions are concerned with movement, emotion and cognition.

The Future of Brain PET

PET scans offer the most direct way to observe what happens at a molecular level in the brain when someone develops, or recovers from, a psychiatric disorder or addiction, Kessler says. Drug developers and pharmaceutical companies now use PET scans to fine-tune prospective new treatments, he notes. If they know they need to lower the number of dopamine receptors in one area of the brain, for instance, they can use PET scans to determine which drugs, and drug dosages, effectively achieve this.

One of the ongoing challenges in the field, Kessler says, is developing new tracers. With UAB’s new cyclotron, Kessler says he has the tools at his fingertips to continue developing and perfecting tracers that bind to different receptors in the brain. He’s already begun work with the neurotransmitter glutamate; like dopamine, glutamate can be studied through PET tracers that bind to glutamate receptors. And glutamate is thought to have roles in autism, Huntington’s disease, Parkinson’s and anxiety disorders, among other things.

This fall, Kessler launched a study examining glutamate receptors in the brains of addicts as they voluntarily withdraw from methamphetamine. The findings — if they show key differences from normal brains — could lead to new drugs to help meth addicts quit their addiction. Studies on Parkinson’s, depression and Alzheimer’s disease are also in the works with UAB collaborators.

Although “this is a tough area to work in for many reasons,” Kessler said — from the vagaries of chemical half-lives to the sheer complexity of the brain itself — he wouldn’t have it any other way. “You just can’t get this kind of information anywhere else.”

By: Sarah C.P. Williams