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- W2035449971 abstract "IN 1950, Alvord et al<sup>1</sup>first suggested that the principal neuropathological feature of phenylketonuria (PKU) was a delay in the development of myelin. Subsequently, other investigators described areas of pallor in sections of central white matter stained for myelin and retarded myelination of the long tracts.<sup>2,3</sup>Similar abnormalities were found in maple syrup urine disease (MSUD).<sup>4-6</sup>Biochemical investigations of the cerebral white matter of patients with these diseases have also indicated that myelin was accumulating at a slower rate than that found in normal brains. In both PKU and MSUD, the water content of cerebral white matter was increased and the lipid content reduced.<sup>7-10</sup>The reduction of cerebrosides exceeded that of other lipid classes,<sup>9-11</sup>which is to be expected, since the accumulation of this lipid most closely parallels the formation of normal myelin.<sup>12</sup> While biochemical studies of this type confirmed prior histological observations," @default.
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- W2035449971 date "1968-12-01" @default.
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- W2035449971 title "Development of Myelin in Inherited Disorders of Amino Acid Metabolism" @default.
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- W2035449971 doi "https://doi.org/10.1001/archneur.1968.00480060022002" @default.
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