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Hugo Moser, Neurologist and ALD Researcher, Dies at 82

Stump, Elizabeth

doi: 10.1097/01.NT.0000264221.81954.90
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Hugo Wolfgang Moser, MD, PhD, a neurologist renowned for his research on complex genetic disorders and particularly adrenoleukodystrophy (ALD), died on Jan. 20 in Baltimore, of pancreatic cancer, at the age of the 82.

Dr. Moser was a professor of neurology and pediatrics at Johns Hopkins University School of Medicine, prior to which he had served as a medical officer during the Korean War and studied at Harvard and Massachusetts General Hospital. He was president of the Kennedy Krieger Institute from 1976 to 1988 and later became director of the Neurogenetics Research Center at the Institute.

Early on in his career, Dr. Moser began researching brain lipids and leukodystrophies. He specialized in genetic disorders that involve dysfunction of the peroxisomes — organelles within a cell that contain enzymes responsible for critical cellular processes, including oxidation of fatty acids, biosynthesis of membrane phospholipids (plasmalogens), cholesterol, and bile acids. He helped to identify the characteristic biochemical abnormalities and the gene mutations responsible for each of the 15 peroxisomal disorders. Dr. Moser came to be recognized for his work in the most common of these disorders, ALD.

In 1976, Dr. Moser moved to Baltimore and to the Kennedy Krieger Institute to work specifically on ALD, where he and his wife and research partner, Ann Moser, developed the first diagnostic test for ALD, detecting through serum the very long chain of fatty acids. Dr. Moser established methods of early diagnosis and programs around the world to evaluate methods of therapy. His groundbreaking study published in 2005 proved that Lorenzo's oil — a combination of fats extracted from olive and rapeseed oils — prevents the onset of disease symptoms for the vast majority of children with ALD (Archives of Neurology 2005;62:1073–1080).

Dr. Moser's commitment to fighting the disease was profiled in the 1992 film Lorenzo's Oil.

“Without Hugo's tireless commitment to studying the efficacy of Lorenzo's oil, we would still be lacking the scientific validation that has proven the oil prevents the onset of ALD symptoms,” said Augusto Odone, the developer of Lorenzo's oil and the father of the son whose name has been lent to the therapy. “Through this journey, I found not only a brilliant partner in ALD research, but a dear friend whose support has been invaluable to our family all these years,” he said in a statement from the Kennedy Krieger Institute.

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In July 2006, Dr. Moser outlined a technique for an ALD screening to be “piggybacked” onto states' existing newborn screening programs, and further testing of the technique is currently underway at the Kennedy Krieger Institute under Ann Moser's leadership.

Dr. Moser remained extremely active throughout the ALD community even as his health began to fail, and served on the Board of Directors of the International Child Neurology Society, The Myelin Project, the Montel Williams Foundation, and the United Leukodystrophy Foundation. He was internationally known as an advocate for individuals with mental retardation and developmental disabilities. Dr. Moser was elected in October 2006 to the rank of Fellow of the American Association for the Advancement of Science for his revolutionary work in the field of developmental neurogenetics.

“Hugo Moser's passing is a profound loss for the field of neurogenetics, the international adrenoleukodystrophy community, and all who had the privilege of working with this great man,” said Dr. Gary Goldstein, president of the Kennedy Krieger Institute, in a statement from the Institute. “As anyone who knew him could attest, he was far more than a dedicated faculty member at Kennedy Krieger; he was a friend and mentor to us all.”

©2007 American Academy of Neurology