The value of CT in diagnosis and prognosis of different inborn neurodegenerative disorders in childhood

Inborn errors of metabolism in 40 children have been investigated by computed tomography to obtain data on the degree of cerebral involvement in neurodegenerative and storage disorders: 20 children had various mucopolysaccharidoses, 8 sphingolipidoses, 3 mucolipidoses, 2 oligosaccharidoses, 3 ceroidlipofuscinoses and 4 had various leucodystrophies. Diagnosis in all patients except Alexander's disease was established by biochemical or histological means.