Hugo Moser, M.D., Director of the Neurogenetics Research Center at the Kennedy Krieger Institute and University Professor of Neurology and Pediatrics at Johns Hopkins University, died on 20 January 2007 at the age of 82. His medical and scientific career spanned over 50 years with over 400 publications. He made significant contributions to numerous disorders including Farber’s disease, metachromatic leukodystrophies and Rett syndrome, but his principal focus was on X-linked adrenoleukodystrophy (X-ALD) and the other peroxisomal disorders.
Development of the diagnostic assay for very long chain fatty acids, characterization of the phenotypic variation, and pursuit of treatment were just some of Hugo’s contributions to the adrenoleukodystrophy research field. The treatment of X-ALD remained one of his principal concerns. The therapy that would be pursued most vigorously was the use of the agent Lorenzo’s oil, a combination of glycerol trierucate and glycerol trioleate. This therapy has had to sustain the waves of enthusiasm and pessimism as further understanding of the disease has been achieved. While still not absolutely secured, its role in therapy has been advanced through Hugo’s steadfast pursuit.
Active right up until his recent illness, he was pursuing the development of methodology for newborn screening for X-ALD and other peroxisomal disorders. This was due to the belief that the greatest difference in this disease could be made when individuals were in the presymptomatic phase.
Hugo was dedicated to his patients and their families. He was generous with his time, actively participating in family support groups, and responding personally to requests for assistance.
Throughout his career, Hugo had several productive collaborations, but the most important one was with his beloved wife Ann. Their scientific and personal partnership was amazing to witness and both were dedicated to the work performed.