It describes postdoctoral models and kits for the treatment and / or prevention of soluble storage disorders such as Gaucher's disease. It also introduces post-processing models and toolkits for the treatment and / or prevention of degenerative disorders of the n system.Central patrons, such as Warren's Parkinson's or Levy's body dementia. Requirement 1: a method for the prevention and / or treatment of anencephaly in patients at risk of development or diagnosed with anencephalywhere the method comprises: administering to the patient an effective amount of (3R, 4R, 5S) -5- (difluoromethyl) piperidin-3,4-diol or a salt thereof during a first period of enzymatic potentiation； Do not administer (3R, 4R, 5S) -5- (difluoromethyl) piperidin-3,4-diol or any of its salts during a substrate replacement period； and then administer to the patient an effective amount of (3R, 4R, 5S) -5- (difluoromethyl) piperidin-3,4-diol or one of its salts during a second period of enzymatic potentiation,donde la cantidad eficaz se encuentra en el intervalo de aproximadamente 50 a aproximadamente 300 mg de equivalente de base libre (FBE) al da Reivindicaci n 9 El m todo de la reivindicaci n 1 donde la sal es el fumarato de (3R 4R 5S) - Do not administer (3R, 4R, 5S) - 5 - (difluoromethyl) pyridine-3, 4-diol or any of its salts during matrix replacement； then effectively administer patient (3R, 4R, 5S) - 5 - (difluoromethyl) pyridine-3, 4-diol or one of its salts during the second enzyme fortification period in an effective amount of This range is about 50-300 mg of free radical equivalent (FBE) per day.Se describen pautas posológicas y kits para el tratamiento y/o la prevención de trastornos de almacenamiento lisosomal tales como la enfermedad de Gaucher. También se describen pautas posológicas y kits para el tratamiento y/o la prevención de trastornos degenerativos del sistema nervioso central tales como las sinucleinopatías enfermedad de Parkinson o demencia por cuerpos de Lewy. Reivindicac
