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Mucopolysaccharidosis type 1 (MPS I) is an inherited lysosomal storage disease that is caused by deficiency of the enzyme α-l-iduronidase, leading to accumulation of the glycosaminoglycans heparan and dermatan sulfate throughout the body. Central nervous system (CNS) symptoms of MPS I include cognitive decline, spinal cord compression, hydrocephalus, hearing loss, and vision impairment. The most severe form of MPS I is known as Hurler syndrome, and patients with this type are almost uniformly determined to be intellectually impaired by early childhood [1]. The cause of cognitive decline in MPS I is primarily the direct effect of the disease on the brain. Other effects of the disease such as the development of hydrocephalus and vision and hearing impairment also contribute. Poor physical health, including respiratory insufficiency and organomegaly, also play a role in cognitive impairment. In the attenuated Hurler–Scheie and Scheie types of MPS I, classical cognitive decline is not expected, although spinal cord compression, visual loss, and hydrocephalus can still occur.