Hopkins doctor studied rare disorder ALD

Dr. Hugo Wolfgang Moser 1924-2007

January 23, 2007|By Jacques Kelly and Dennis O'Brien | Jacques Kelly and Dennis O'Brien,Sun reporters

CLARIFICATION

A headline for the obituary of Dr. Hugo Moser in Wednesday's editions of The Sun described him as a "Hopkins doctor." While Dr. Moser was on the faculty of the Johns Hopkins School of Medicine, his principal work was with the Kennedy Krieger Institute, which is affiliated with Hopkins but independent of it.

Dr. Hugo Wolfgang Moser, a renowned Baltimore neurologist whose work with a rare genetic disorder was depicted in the 1992 film Lorenzo's Oil, died Saturday at Johns Hopkins Hospital of surgical complications after treatment for pancreatic cancer. He was 82.

The Swiss-born Dr. Moser, portrayed in the movie by actor Peter Ustinov, spent his life researching the scientific basis of childhood mental retardation and advocated testing all newborns for the condition known as adrenoleukodystrophy, or ALD.

He was director of the Neurogenetics Research Center at the Kennedy Krieger Institute, a professor of neurology and pediatrics at the Johns Hopkins University School of Medicine, and was conducting research into ALD treatments for children and adults

"Most people would retire at 65," said Dr. Gary Goldstein, president of Kennedy Krieger. "But the most productive part of his life happened in the past 20 years. He signed a grant application on the way to the operating room [for his surgery] last fall. He wanted to get this disease cured before he died."

"Hugo was really one of the giants of neurology," said Dr. Douglas Kerr, a professor of neurology at the Hopkins medical school. "He's trained countless neurologists, and I'd consider him a model of how we all want to do neurology."

Dr. Moser's wife and longtime research partner, Ann Boody Moser, described him as a workaholic. Goldstein agreed, adding, "He was a tireless worker. His car was the first one in the parking lot in the morning, and it was the last one out at night. He gave his life to his research."

But the Roland Park resident made time to attend Baltimore Symphony Orchestra and Shriver Hall concerts, and Baltimore Opera Co. productions.

Born in Bern, Dr. Moser spent his early childhood in Berlin, where his father, Hugo L. Moser, was an art dealer and his mother, Maria, was an actress. He considered becoming a concert pianist, but early on switched to science.

His family fled Nazi Germany in 1933 and lived in Heemstede, Holland. In 1940, they crossed France, Italy, and Spain and finally embarked to Cuba before obtaining visas to reach New York. He attended Harvard College from 1942 to 1943, and left to enter military service.

He received his medical degree from Columbia University in 1948.

In 1950, he moved to Boston to become an assistant in medicine at Peter Bent Brigham Hospital. He served in Korea as an Army physician before returning to Harvard to earn a master's degree. He was a resident in and later professor of neurology at Massachusetts General Hospital until 1976.

He came to Baltimore that year as a professor of neurology and pediatrics at Johns Hopkins and president of the John F. Kennedy Institute, now the Kennedy Krieger Institute, which is internationally known in the field of pediatric developmental disabilities. He remained as its president until 1988.

As a neurologist, Dr. Moser studied childhood genetic disorders and when he came to Kennedy Krieger, he had already developed an interest in ALD, a crippling and ultimately deadly childhood disorder.

By the 1980s, Dr. Moser and his wife were working to develop a screening technique that would enable detection of ALD at birth.

The rare disease is caused by a genetic mutation that leads to the accumulation of substances called very long chain fatty acids in cells. That, in turn, damages the myelin, the material that coats nerve fibers in the brain, much like the insulating material that protects telephone wire. The myelin damage - to date irreversible - is what causes the neurological system to break down.

While there are various forms of ALD, the most prevalent is the childhood cerebral form. In Lorenzo's Oil, actors Susan Sarandon and Nick Nolte portrayed the relentless efforts of Augusto and Michaela Odone to find treatments for their son, Lorenzo.

A Hopkins publication described the child as "a bright, exuberant 5-year-old who went into a sudden, shocking decline" as damage to myelin brought cognitive loss and left him unable to walk, see and hear. Lorenzo, who turned 28 in May, cannot see or walk but can hear, his father said.

The parents were often at odds with a medical community skeptical about the effectiveness of a treatment made from olive and rapeseed oils. The compound was patented by Augusto Odone and is considered experimental by the Food and Drug Administration.

Mr. Odone, 74, credited Dr. Moser with being more receptive than most physicians.

"Unlike other doctors, who did not believe in Lorenzo's Oil, Dr. Moser kept an open mind," Mr. Odone recalled yesterday from his home in Fairfax, Va.

He said that when Mrs. Odone died in 2000, Dr. Moser wrote a warm letter of condolence.

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