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Hugo Moser, MD

(1924-2007)

Written by: Michael V. Johnston, MD and Gary W. Goldstein, MD

Dr. Hugo Moser

Dr. Hugo Moser, one of the world’s most eminent and gracious neurologists, who was famous for his pioneering research on adrenoleukodystrophy (ALD) and related peroxisomal disorders, died at Johns Hopkins Hospital in Baltimore on January 20, 2007 at age 82. He was University Professor of Neurology and Pediatrics at Johns Hopkins University School of Medicine and Director of the Neurogenetics Department at the Kennedy Krieger Institute. It was our good fortune and pleasure to work closely with him for more than 18 years. When we arrived at the Institute, he had already served as its President for more than a decade and had reached an age when many people are considering retirement. However, we soon learned that he was only at mid- career in terms of his major contributions to pediatric neurology research.

Dr. Moser was born in Bern, Switzerland in 1924 and spent his early years in Berlin where his father was an art dealer and his mother was an actress. The family fled Nazi Germany in 1933 for Holland, and in 1940 they moved across Europe and then to Cuba before they obtained visas to reach New York. After attending Harvard College and Columbia Medical School, he became an Assistant in Medicine at Peter Bent Brigham Hospital in 1950 followed by a residency in Neurology at Massachusetts General Hospital. His training was interrupted by service in the US Army during the Korean War. In 1976 he moved from his position as Professor of Neurology at Harvard Medical School and Massachusetts General Hospital to Baltimore to lead the John F. Kennedy Institute, which later became the Kennedy Krieger Institute. With his wife, Ann, he established a world renowned research program at the Institute on the causes of mental retardation, initially focusing on new evidence that blood levels of very long chain fatty acids are elevated in ALD. Research on the childhood cerebral form of this disorder along with related disorders of the peroxisome and attempts to treat and prevent these disorders became his passion for the next 30 years.

By every ordinary criteria of academe, he was a fantastic success, with hundreds of papers in excellent journals and decades-long, multi-million dollar funding from NIH.

The laboratory methods that he and Ann developed for diagnosis of peroxisomal disorders became the international standard, and mailing instructions for sending samples to their laboratory at Kennedy Krieger can be found in hospitals throughout the world. His work put the institute on the map as a leading center for translational neuroscience research. However, one additional accomplishment set him apart from virtually every other pediatric neurologist and the vast majority of physicians: his portrayal in a major motion picture. Peter Ustinov starred as Professor Nikolais, a character fashioned after Dr. Moser, in the 1992 movie “Lorenzo’s Oil” about a 5 year old boy suddenly stricken by ALD. The boy’s parents, Augusto and Michaela Odone, were played by Nick Nolte and Susan Sarandon. The movie portrayed the parents’ struggle to have the medical profession take seriously a non-standard therapy based on olive and rapeseed oils. Although Dr. Moser was not fond of the movie because he felt it portrayed him as a somewhat detached academic who demanded a controlled trial, he eventually did publish evidence that Lorenzo’s oil benefits patients in the early stages of ALD and the Odone’s credited him with keeping an open mind. Recently, Dr. Moser assisted Mr. Odone with his own recovery from a major illness while he was hospitalized at Hopkins, and both men were later featured in a front page story with a color picture together in the Wall Street Journal.

Dr. Moser was a charismatic lecturer and teacher and an enthusiastic member of the Child Neurology Society and the International Child Neurology Association, and other major neurological organizations. He loved to travel the globe spreading the word about his research, although in recent years he was hampered by myasthenia gravis making it necessary for him to use a walker. This did not seem to slow him down, although his wife Ann began to go along to look after him. After he returned from one of his trips, there would be phone calls, letters and emails from young people who had been inspired by his lectures and who wanted to come to Baltimore to work with his group. Occasionally we were surprised when someone would call from the airport to tell us that they had arrived to work with him. Many of these young people did join his laboratory over the years, and became co-authors on his publications as well as leading investigators in their own right. Many also needed a place to stay and lived for weeks to years with him and Ann in their big house in the Roland Park section of Baltimore.

What we liked most about him was his warm, self-effacing manner, his enthusiasm and the opportunity to learn from him about his latest discovery or idea for new research. He was always generous and accessible to students, residents and faculty, and enjoyed hearing about new research ideas. He thanked you for sharing ideas with him, and made you feel that he valued your opinion. When he was working on a new idea or project, he would sometimes appear at your office door, his voice literally shaking with excitement. He was a self-confessed workaholic who remained vigorous working
12 hour days until the last few months of his life. We often saw his car in the parking lot when we arrived in the morning and it was still there when we went home. Just prior to major surgery for cancer last fall, we found him in his hospital room in the Halsted building at Hopkins working feverishly to meet an NIH grant deadline. He and Ann developed a new method that would allow all newborn infants to be screened for ALD, and he wanted to be sure that the project would be considered for funding even if his surgery did not go well. He had a prolonged recovery from his surgery but by early January he was back home listening to the opera and reading the New York Times, vividly recalling his adventures with modern hospital care. As usual, he had bounced back from adversity, and he seemed indestructible. We were genuinely surprised and greatly saddened when he succumbed two weeks later.

Several years ago his portrait was painted and has been hanging in the front of the lobby at the Institute so that it is the first thing we see when coming to work each morning. It is a good likeness of him, and shows him with a cheerful face and rosy cheeks. He is smiling and looks excited and quite pleased, perhaps as he was when he had solved a problem, got a good score on a grant, or thought he had discovered a new way to help patients and families with ALD. In addition to all that he has given us as a legacy, this is how we will remember Hugo.

A memorial service for the family was held in early February in Massachusetts and another one is being planned here in Baltimore. Cards and letters can be sent to Mrs. Ann Moser care of the Kennedy Krieger Institute, 707 North Broadway, Suite 500, Baltimore, MD 21205.