17. Phenylketonuria (PKU)

    1 - Toxic metabolites from an aberrant liver enzyme
    2 - Maternal PKU
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Autism in individuals with phenylketonuria PKU) has long been recognized.  Visible
impairment of myelination is the major neuropathological finding in PKU.  But Williams
et al. (1980) included a case of PKU in an investigation of neuropathology in autism
and found the only barely visible sign to be reduced dendritic spines on pyramidal
neurons.  Mental retardation has occurred in children of mothers with PKU whose
phenylalanine intake was not well controlled during pregnancy.  Prenatal exposure to
high levels of phenylalanine and its toxic metabolites appears to have an effect more
like that of prenatal exposure to alcohol.
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Preface

III -
EVIDENCE OF NEURO-
LOGICAL  IMPAIRMENTS
9.   The nature of autism
10. Language and hearing
11. The auditory system
12. Brainstem nuclei
13. Perinatal vulnerability
14. Brainstem damage
15. Neuropathology
16. Fetal alcohol syndrome
17. Phenylketonuria
18. Regressive autism

Toxic metabolites from
an aberrant liver enzyme
Maternal PKU