14800-DvRappard

108 Chapter 7 Table 1. Characteristics of MLD patients MLD code MLD type Age / y a Baseline scan b Follow-up scans b Eligible for HCT Treated with HCT Outcome 37 Juvenile 6.4 No 5 Yes Yes Moderate 16 Juvenile 6.5 Yes 1 Yes Yes Good 39 Juvenile 7.0 Yes 0 No No Poor 29 Juvenile 7.1 Yes 0 No No Poor 53 Juvenile 7.1 Yes 1 No Yes Poor 5 Juvenile 7.2 Yes 0 No No Poor 4 Juvenile 8.1 No 1 Yes Yes Moderate 33 Juvenile 8.1 No 1 No No Poor 6 Juvenile 8.6 Yes 0 No No Poor 54 Juvenile 12.5 Yes 0 No No Poor 14 Juvenile 14.1 Yes 3 Yes Yes Good 22 Juvenile 15.1 Yes 0 No No Poor 21 Adult 17.8 Yes 1 Yes Yes Good 30 Adult 20.1 No 5 Yes Yes Good 51 Adult 22.5 Yes 0 No Yes Poor 41 Adult 25.4 Yes 2 Yes Yes Good 32 Adult 26.9 Yes 1 Yes No Moderate 2 Adult 28.4 Yes 3 Yes Yes Good 46 Adult 28.8 No 1 Yes Yes Good 23 Adult 32.5 Yes 0 No No Moderate 15 Adult 35.2 Yes 2 Yes Yes Moderate a) Age at first examination at 1.5T Siemens Sonata. b) At 1.5T Siemens Sonata. Eleven patients were eligible for HCT (four juvenile, seven adult); twelve received HCT: ten eligible (one eligible adult patient declined HCT), and two initially classified as non- eligible, after careful consideration and discussion with parents. HCT was performed as previously described. 7 For 16 patients baseline data at the 1.5T Siemens Sonata (Erlangen, Germany) were available, and follow-up data for eight of these. The dedian age at baseline scan was 14.6 y (range 6.5-35.3 y). At latest clinical follow-up, five (all eligible and treated) had good outcome, three (two eligible, one treated) moderate and eight poor outcome (all non-eligible, two treated, three deceased).