Hurler Syndrome: A report of three cases.

ABSTRACT

The mucopolysaccharidoses (MPS) are a group of inherited metabolic disorders that result in widespread skeletal, visceral, and mental abnormalities. A defect in metabolic degradation leads to the storage of mucopolysaccharide macromolecules in the nervous system and other body tissues. The MPS are classified into various types, and there are additional diseases, such as the mucolipidoses and a gangliosidosis, that demonstrate similar clinical and radiologic findings. We hereby present 3 cases of Hurler Syndrome, TypeI mucopolysaccharidoses, and enumerate there radiological characteristic radiological findings.