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Effect of Dietary Phenylalanine Restriction On Visual Attention Span In Mentally Retarded Subjects With Phenylketonuria

Published online by Cambridge University Press:  18 September 2015

F.D. Giffin ,
J.T.R. Clarke  and
D.M. d'Entremont
F.D. Giffin
Affiliation:
Department of Pediatrics, The Atlantic Research Centre for Mental Retardation, Dalhousie University, and Department of Psychology, lzaak Walton Killam Hospital for Children, Halifax, Nova Scotia
J.T.R. Clarke*
Affiliation:
Department of Pediatrics, The Atlantic Research Centre for Mental Retardation, Dalhousie University, and Department of Psychology, lzaak Walton Killam Hospital for Children, Halifax, Nova Scotia
D.M. d'Entremont
Affiliation:
Department of Pediatrics, The Atlantic Research Centre for Mental Retardation, Dalhousie University, and Department of Psychology, lzaak Walton Killam Hospital for Children, Halifax, Nova Scotia
*
Department of Pediatrics, Clinical Research Centre, Room C-R1, Dalhousie University, Halifax, Nova Scotia B3H 4H7, Canada
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An ABA within-subject study design was used to assess the effect of dietary phenylalanine restriction on the visual attention span of three mentally retarded males (9, 15, and 21 years old)with phenylketonuria. Visual attention span was measured by recording the amount of time the subjects visually fixated pictures projected on a screen according to a standardized test protocol. After 4-6 weeks of baseline testing (A-phase), each subject was placed on a phenylalaninerestricted diet, designed to maintain plasma phenylalanine levels at 0.3 - 0.9 mM, for 8-14 weeks (B-phase). A return to baseline phenylalanine intake (A-phase) was achieved by surreptitiously adding sufficient L-phenylalanine to the therapeutic diets to increase plasma concentrations of the amino acid to pretreatment levels. Diet treatment (B-phase) was associated with highly significant improvementsin visual attention span in two subjects (multivariate analysis of variance, P <<0.001); the third subject, the most retarded, showed no effect. No objectivelydemonstrable change in behavior was documented in any subject. However, the results support the view that phenylalanine toxicity extends beyond early childhood and that some component of the toxicity is reversible, even in severely retarded patients with phenylketonuria.

Type
Research Article
Information
Canadian Journal of Neurological Sciences , Volume 7 , Issue 2 , May 1980 , pp. 127 - 131
Copyright
Copyright © Canadian Neurological Sciences Federation 1980

References

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