Neurologist Moser Dies at 82

The Associated Press
Tuesday, January 23, 2007; 1:25 AM

BALTIMORE -- Dr. Hugo Wolfgang Moser, a neurologist whose work with a rare disorder was depicted in the 1992 movie "Lorenzo's Oil," has died. He was 82 and had been undergoing treatment for pancreatic cancer.

Moser, who died Saturday at Johns Hopkins Hospital, researched childhood mental retardation and advocated testing all newborns for the condition known as adrenoleukodystrophy, or ALD.

His professional posts included director of the Neurogenetics Research Center at the Kennedy Krieger Institute and professor of neurology and pediatrics at the Johns Hopkins University School of Medicine.

"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."

By the 1980s, 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 damages the myelin, the material that coats nerve fibers in the brain. The myelin damage is irreversible and causes the neurological system to break down.

The most common form of ALD 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 Lorenzo as "a bright, exuberant 5-year-old who went into a sudden, shocking decline" as damage to myelin left him unable to walk, see and hear. Lorenzo, who turned 28 last year, can hear, his father said.

The parents were often at odds with doctors unconvinced 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.

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," Odone recalled Monday from his home in Fairfax, Va.

Moser published a study in 2005, based on research with 84 boys treated at Kennedy Krieger, showing that Lorenzo's Oil can prevent onset of the disease's symptoms for most boys who receive a diagnosis of ALD. The disease is passed from mothers to sons.

Moser was portrayed in the movie by Peter Ustinov.

© 2007 The Associated Press