 This is a single center, open-label study of patients with late infantile MLD. All patients were previous treated 26 weeks in the phase I trial EudraCT number: 2006-005341-11, NCT00418561. All patients will be offered continuing treatment in this study and will in this protocol receive 13 infusions, whereby the patients total have had 27 infusions of Metazym. One infusion will be given every other week. After a total of 52 weeks of treatment the subjects will continue treatment in a compassionate use protocol. Safety AESAE will be monitored at every visit. 