Rochester Brain Expert Recognized For Lifetime Contributions

June 11, 2007

An internationally known expert on what is perhaps the most amazing structure in the universe – the human brain – has been recognized by his peers for his contributions to our knowledge about the brain and the diseases that affect it.

James Powers, M.D., professor of Pathology and Laboratory Medicine and of Neurology at the University of Rochester School of Medicine & Dentistry, received the Lifetime Achievement Award for Meritorious Contributions to Neuropathology at the annual meeting of the American Association of Neuropathologists recently in Washington. The group is the largest and most prestigious neuropathology association, with members around the world.

Powers has focused on neuropathology – the intense study of the brain and conditions that affect it – since becoming a doctor in 1969. He has performed tens of thousands of procedures called “brain autopsies,” taking an incredibly detailed look at the brain of a person who has died, to learn more about that person’s life and the conditions that affected him or her. Powers is able to translate the slightest nuances of shape, color or texture that for most people signify little, and weave together a person’s health history – perhaps affected by stroke, Alzheimer’s or Parkinson’s diseases, or multiple sclerosis.

Powers is an expert on diseases known as peroxisomal disorders, where the cellular structures that break down toxic substances in the brain and other organs are defective or absent because of a genetic defect. Oftentimes these substances end up damaging the myelin, the fatty coating that insulates nerves, and so Powers is widely known for his work on myelin diseases as well.

Much of Powers’s work is on inherited diseases that take an exceptionally harsh course in children. In diseases known as pediatric leukodystrophies, where the myelin in a child is damaged, most children are born healthy, but at some point when they are young, often around the age of four to six, the defect in myelin begins taking a toll. Depending on the disease, the child might suffer from seizures, blindness, difficulty walking and breathing, and mental retardation. The diseases are almost always fatal before the child reaches adulthood.

For more than three decades Powers has studied adreno-leukodystrophy or ALD, a rare disease that affects mainly boys that was portrayed in the movie Lorenzo’s Oil. Boys with ALD are completely healthy until around the age of five or six, when they begin having problems such as difficulty in school, aggressiveness, or memory problems. Typically they die within two years of diagnosis.

Powers’s investigations into such diseases have brought him into the lives of hundreds of families where the diseases are inherited. Nearly every year he attends the annual meeting of the United Leukodystrophy Foundation, a group that supports research into the diseases and helps families cope with the fallout of having a loved one with a disease like ALD. He spends several days with the families, filling them in on new findings, what to expect, and what can currently be done for the children.

“It’s an uplifting experience, to do what you can for these families,” said Powers. “When their loved one has been afflicted by such an awful disease, it’s something we can give to them – some understanding of what is happening, what to expect, and reasons to be hopeful.”

Powers is currently on the trail of an extremely rare brain disease that has not been identified previously. The findings on the new disease, discussed at the recent neuropathology meeting, come from slides of three patients that Powers collected over a span of 14 years. Such cases are sent to him regularly from pathologists seeking solutions to tough-to-crack puzzles about the brain.

Not all of Powers’s work focuses on people who have passed away. When a surgeon needs a piece of brain tissue analyzed, for instance, when investigating how far a brain tumor may have spread in a patient fighting brain cancer, they turn to doctors like Powers to help them analyze the results.

Beyond his work with patients and families and his research in the laboratory, Powers has been a very effective teacher. Since coming to Rochester in 1992, he has been commended for his outstanding teaching four times, and this year he was selected by the graduating medical students to give the faculty address to graduating students.

Powers received his bachelor’s degree in biology from Manhattan College and his medical degree from the Medical University of South Carolina. Before coming to Rochester, Powers worked at the Medical University of South Carolina and at Columbia University. He has also served as every officer of the American Association of Neuropathologists, including president and secretary-treasurer.

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