STUDIES ON PHENYLKETONURIA

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Abstract
Of 23 albino rats, 17 were given phenyl-lactic acid and 6phenylpyruvicacid. Typical myelopathy was observed in the spinal cord of 14 animals; gross degeneration was found in the spinal and cortical nerve-cells of all animals, in the brain stem of 9. Symmetrical spongy foci were observed in the subcortical white matter of the frontal lobes in 1 case, in the white substance of Ammon''s horn in 2 cases, and in the brain stem in 1 case. This kind of medullary destruction shows a certain difference from human histopathological findings, which cannot be regarded as essential, since spongy cerebral foci have been observed also in humans. The results of the reported study fully confirm the hypothesis of Crome et al. who have suggested that pathological changes of the central nervous system may be ascribed to the toxic effect of phenylalanine.