Abstract #2125

A Seven Years Quantitative MRI and MRS Follow-Up Study on Successful Bone Marrow Transplantation for Presymptomatic Juvenile Metachromatic Leukodystrophy

Xiao-Qi Ding¹, Annette Bley², Alfried Kohlschtter², Jens Fiehler³, Heinrich Lanfermann¹

¹Institute of Diagnostic and Interventional Neuroradiology , Hannover Medical School, Hannover, Germany; ²Department of Paediatrics, University Medical Centre Hamburg-Eppendorf, Hamburg, Germany; ³Department of Neuroradiology, University Medical Centre Hamburg-Eppend, Hamburg, Germany

Bone marrow transplantation (BMT) has been advocated as treatment of juvenile metachromatic leukodystrophy (MLD). The effectiveness of this high-risk treatment is still questionable due to the rarity of follow-up reports. We carried out a 7 years MRI follow-up on a boy with juvenile MLD who had received BMT treatment in the presymptomatic phase and remained free of MLD symptoms during the observation. Conventional morphological MRI showed minor stable white matter lesions while quantitative T2-mapping and MR spectroscopy evidenced a stagnancy of the demyelination process and an ongoing maturation of the brain.