BONE-MARROW RELAXATION-TIMES IN GAUCHER-DISEASE BEFORE AND AFTER ENZYME REPLACEMENT THERAPY

The aim of this work was to monitor the effectiveness of enzyme replac ement therapy on the basis of the changes in T1 relaxation times in Ga ucher patients. A total of 26 patients underwent MR before enzyme repl acement therapy; of them, 18 have been followed-up. A total of 22 age- matched controls underwent the same MR study. Scans were focused on th e femoral neck, and T1 relaxation times were measured by means of a mi xed spin-echo inversion recovery sequence. The T1 relaxation times in Gaucher patients were significantly longer than normal (p < 0.05). Aft er enzyme replacement therapy, T1 relaxation times gradually became cl oser to those of control subjects, and there was also a significant de crease (p < 0.01) with respect to values before therapy, probably due to an increase in the fat/water ratio. Evaluation of T1 relaxation tim e map supply a useful indication of Gaucher disease regression after e nzyme replacement therapy particularly in those cases in which a norma l skeletal appearance corresponds to prolonged T1 relaxation times.