| | Type | Denomination | Main clinical features | GAGS* | Enzyme deficiency |
| | MPS IH | Hurler syndrome | Severe phenotype, macrocephaly, macroglossia, facial coarseness, hydrocephalus, mental retardation, joint stiffness, thoracic and lumbar kyphosis, possible odontoid hypoplasia. | DS, HS | α-L-Iduronidase |
| | MPS IS | Scheie syndrome | Joint stiffness, corneal turbidity, aortic valve disease, normal intellectual capabilities, macroglossia, normal stature, living until adulthood. | DS, HS | α-L-Iduronidase |
| | MPS I H/S | Hurler-Scheie syndrome | Intermediate phenotype, macrocephaly, macroglossia, joint limitations. | DS, HS | α-L-Iduronidase |
| | MPS II | Hunter Syndrome | Severe course MPS I H-like, moderate course-clinical intermediate phenotype, late manifestations, living until adulthood with or without mental retardation. | DS, HS | Iduronate-2-sulfatase |
| | MPS III A | SanfilippoA syndrome | Behavior disorders, aggressiveness. | HS | Heparan-N-sulfatase |
| | MPS III B | SanfilippoB syndrome | Progressive dementia, living until the second and third decade. | HS | α-N-acetyl-glucosaminidase |
| | MPS III C | SanfilippoC syndrome | Remarkable interfamilial variability, mild dimorphism. | HS | AcetylCoA:a-glucosaminide
acetyltransferase |
| | MPS III D | SanfilippoD syndrome | Rough hair, limpid cornea, usually normal height. | HS | N-acetilglucosamine-6-solfatase |
| | MPS IV A | MorquioA syndrome | Short trunk dwarfism, thin corneal opacities, skeletal dysplasia and spondylo epiphyseal, final height under 125 cm. | KS | N-acetilglucosamine-6-solfatase |
| | MPS IV B | MorquioB syndrome | Idem MPS IV A, adulthood height under 120 cm. | KS | β-galactosidase |
| | MPS VI | Maroteaux-Lamy syndrome | Hurler phenotype with pronounced corneal opacities and normal intellectual capabilities. | DS | N-acetylgalactosamine 4-sulfatase |
| | MPS VII | Sly syndrome | High phenotypic variability, dense granulocyte inclusions, macrocephaly, possible odontoid hypoplasia, shortness. | CS, DS, HS | β-glucuronidase |
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