The present invention relates to intrathecal administration of lysosomal enzymes for effective treatment of lysosomal storage diseases. In some embodiments, the invention provides stable formulations for IT delivery of Alpha N-acetylglucosaminidase.本發明係關於經腦脊髓膜內投與溶酶體酵素來有效治療溶酶體貯積症。在一些實施例中,本發明提供用於IT遞送αN-乙醯基葡萄糖胺酶之穩定調配物。