Bruce Miller, MD, professor of neurology and psychiatry at the UCSF, where he holds the A.W. & Mary Margaret Clausen Distinguished Chair, and Lennart Mucke, MD, Joseph B. Martin Distinguished Professor of Neuroscience at UCSF and director of the Gladstone Institute of Neurological Disease, have been selected to receive the Potamkin Prize for major contributions to the understanding of the causes, prevention, treatment, and cure for Pick’s, Alzheimer’s, and related diseases.
The Potamkin Prize will be awarded on Thursday, April 15 by the American Academy of Neurology at its annual meeting. The award was established in honor of Luba Potamkin, a beloved TV spokesperson for her family’s East Coast auto dealerships, who died of Alzheimer’s disease in 1994.
Commenting on the Potamkin Prize, Mucke said it was a “wonderful reinforcement of the synergism” between himself and Miller. “Working hand-in-hand, we have developed innovative translational programs for the investigation and treatment of dementia and related disorders.”
Miller concurs and describes the Potamkin Prize as the highlight of his academic career. “At UCSF, I have been able to bring a new generation of clinical and basic scientists into the study of frontotemporal lobar degeneration (FTLD)—investigators who have the chance to treat and cure this condition.”
“I feel a great sense of responsibility to the Potamkin family, and the families whom they champion, with this award,” Miller says. “I am inspired to bring a greater focus upon FTLD, to help find ways of treating and preventing this disorder.”
Miller, the clinical director of UCSF’s Memory and Aging Center, has a special interest in the behavioral effects of dementia, notably FTLD, formerly and commonly known as Pick’s disease. Along with Alzheimer’s disease, FTLD is the leading cause of dementia in patients under 65 years.
FTLD is characterized by emotional deficits, impulsivity and lack of empathy in the behavioral variant of the disease. A second subset of the disease, known as semantic dementia, is marked by a gradual loss of the meaning associated with words. Progressive non-fluent aphasia is the third disorder in the FTLD spectrum, impacting patients’ production of language.
Remarkably, patients with FTLD frequently exhibit unexpected artistic and musical talents that may initially intensify as the disease progresses – a phenomenon that Miller has meticulously documented, in an attempt, he says, to “humanize this tragic condition.” This extraordinary trait is attributed to the strengthening and remodeling of parts of the brain linked to creativity, compensating for damaged areas associated with language.
Miller, author of the book “The Human Frontal Lobes” and the medical director of the John Douglas French Foundation for Alzheimer’s Disease, has directed numerous pharmaceutical trials for patients with dementias. He has partnered with UCSF’s Nobel laureate Stanley Prusiner, MD, in a study on Creutzfeldt-Jakob disease (CJD), a rare, rapidly progressive dementia, accompanied by personality changes and visual disturbances. A 1991 recipient of the Potamkin Prize, Prusiner won the Nobel Prize in Physiology or Medicine in 1997 for his discovery of prions, a class of proteins that causes CJD in humans and “mad cow” disease in cattle. The discovery has informed research into the role of misprocessed proteins in more common brain diseases, including Alzheimer’s and Parkinson’s disease.
The Potamkin Prize comes at a timely phase of Miller’s career. When he started studying FTLD more than 25 years ago, the mantra in neurology was “Don’t pick Pick’s disease,” he says. At the time, the disease was viewed as a “rare, biologically obscure illness that could not be diagnosed at the bedside.”
Today, thanks in part to the pioneering work of Miller and his colleagues, the condition is gaining significantly wider prominence and neurologists are able to distinguish it from Alzheimer’s in its earlier stage. Promising advances have been made in the genetics and molecular pathology of the disorder and recent developments include testing therapies that target the genetic cause for FTLD, a milestone that Miller describes as “the most exciting work” he has ever undertaken.
Jointly leading collaborative efforts to develop better therapies for Alzheimer’s disease, FTLD and related disorders, is Mucke, co-recipient of the Potamkin Prize and director of the Gladstone Institute of Neurological Disease.
Mucke is best known for instigatin, a turnaround in the direction of research into Alzheimer’s from an almost exclusive focus on morphological abnormalities in the brain to a focus on the molecular processes that cause the dysfunction of neural networks in the brain.
Ten years ago, many scientists believed the condition was caused by the accumulation of amyloid plaques in the brain. Mucke and colleagues showed that a minuscule constituent of these plaques, known as amyloid-beta peptide, can disrupt the function of brain cells independent of plaques, thus narrowing the whereabouts of the disease culprit from the “haystack” to the “needle.”
Mucke has also generated genetically engineered mice to pinpoint the key molecules and proteins that contribute to the progression of Alzheimer’s thereby setting the stage for new therapies to combat the disease. Using mouse models of Alzheimer’s disease, Mucke and colleagues have successfully prevented the disease progression and even reversed cognitive impairments.
The announcement of the Potamkin Prize winners follows the good news that a new neurosciences building will be constructed on UCSF’s thriving Mission Bay campus. The five-story building, which has been described by Prusiner as the culmination of a 10-year dream, will house the Memory and Aging Center and the Department of Neurology, the W. M. Keck Foundation Center for Integrative Neuroscience, which studies brain function, and the Institute for Neurodegenerative Diseases.
Neurosciences Building to Take Scientific Research to the Next Level
UCSF Today, January 21, 2010