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Donald H. Silberberg, MD

Arch Neurol. 1967;17(5):524-529.

	Since this article does not have an abstract, we have provided the first 150 words of the full text PDF and any section headings.

IT has been demonstrated that a low phenylalanine diet will permit normal development of the child born with phenylketonuria (PKU). If the child is loaded orally with phenylalanine, transient behavioral disturbances occur coincidental with a transient elevation of serum phenylalanine and elevation of the various metabolites which have been identified in these patients. However, attempts by many investigators to correlate excretions of specific metabolites with the degree of retardation in untreated patients has not demonstrated which chemical, or group of metabolites, is most responsible for the neurologic damage.²

Evidence has accumulated that, in at least some patients with PKU, histologic changes occur in the brain which are visible using conventional neuropathologic techniques. Alvord et al,³ in 1950, first described instances of deficient myelination in cerebrum and cerebellum of patients with PKU. Others have since confirmed this finding.^4-6 Recently, Malamud⁶ described distinct structural alterations in . . . [Full Text PDF of this Article]

Author Affiliations
Philadelphia

From the Department of Neurology, University of Pennsylvania School of Medicine, Philadelphia.

Footnotes
Submitted for publication June 2, 1967; accepted June 22.

Read in part before the meeting of the American Neurological Association, Washington, DC, June 14, 1966.1

Reprint requests to Department of Neurology, Hospital of University of Pennsylvania, Philadelphia 19104 (Dr. Silberberg).

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