Prefrontal cortex cognitive deficits in children treated early and continuously for PKU

Monographs of the Society for Research in Child Development

Volumn 62, Issue 4, 1997, Pages

  Diamond, Adele ^a   Prevor, Meredith B ^a   Callender, Glenda ^a   Druin, Donald P ^a  

^a NONE

Author keywords

[No Author keywords available]

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EID: 0346937769     PISSN: 0037976X     EISSN: None     Source Type: Journal    
DOI: 10.2307/1166208     Document Type: Article

References (234)

1. Anderson, V. E., Siegel, F. S., Fisch, R. O., & Wirt, R. D. (1969). Responses of phenylketonuric children on a continuous performance test. Journal of Abnormal Psychology, 74, 358-362.
2. Bachevalier, J. (1990). Ontogenetic development of habit and memory formation in primates. In A. Diamond (Ed.), The development and neural bases of higher cognitive functions. Annals of the New York Academy of Sciences, 608, 457-484.
3. Bachevalier, J., Brickson, M., & Hagger, C. (1993). Limbic-dependent recognition memory in monkeys develops early in infancy. Neuroreport, 4, 77-80.
4. Bachevalier, J., & Mishkin, M. (1984). An early and a late developing system for learning and retention in infant monkeys. Behavioral Neuroscience, 98, 770-778.
5. Bachevalier, J., & Mishkin, M. (1986). Visual recognition impairment follows ventromedial but not dorsolateral prefrontal lesions in monkeys. Behavioral Brain Research, 20, 249-261.
6. Balamore, U., & Wozniak, R. H. (1984). Speech-action coordination in young children. Developmental Psychology, 20, 850-858.
7. Bannon, M. J., Bunney, E. B., & Roth, R. H. (1981). Mesocortical dopamine neurons: Rapid transmitter turnover compared to other brain catecholamine systems. Brain Research, 218, 376-382.
8. Barbizet, J. (1970). Prolonged organic amnesias. In J. Barbizet (Ed.), Human memory and its pathology (D. K. Jardine, Trans.). San Francisco: W. H. Freeman.
9. Bättig, K., Rosvold, H. E., & Mishkin, M. (1960). Comparison of the effects of frontal and caudate lesions on delayed response and alternation in monkeys. Journal of Comparative Physiology, 53, 400-404.
10. Bauer, R. H., & Fuster, A. H. (1976). Delayed-matching and delayed-response deficit from cooling dorsolateral prefrontal cortex in monkeys. Journal of Comparative and Physiological Psychology, 90, 293-302.
11. Bench, C. J., Frith, C. D., Grasby, P. M., Friston, K. J., Paulesu, E., Frackowiak, R. S., & Dolan, R. J. (1993). Investigations of the functional anatomy of attention using the Stroop test. Neuropsychologia, 31, 907-922.
12. Benton, A. L. (1969). Disorders of spatial orientation. In P. Vincken & G. Bruyn (Eds.), Handbook of clinical neurology (Vol. 3). Amsterdam: North-Holland.
13. Benton, A. L., & Hamsher, K. deS. (1976). Multilingual aphasia examination manual (revised). Iowa City: University of Iowa Press.
14. Berry, H. K., O'Grady, D. J., Perlmutter, L. J., & Bofinger, M. K. (1979). Intellectual development and achievement of children treated early for phenylketonuria. Developmental Medicine and Child Neurology, 21, 311-320.
15. Bessman, S. P. (1979). The justification theory: The essential nature of the non-essential amino acids. Nutrition. Reviews. 37, 209-220.
16. Bickel, H., Beckers, R. G., Waraberg, E., Schmid-Rüter, E., Feingold, J., Cahalane, S. F., Bottine, E., Jonxis, J. H. P., Colombo, J. P., & Carson, N. (1973). Collective results of mass screening for inborn errors in eight European countries. Acta Paediatrica, 62, 413-416.
17. Bickel, H., Gerrard, J., & Hickmans, E. M. (1954). The influence of phenylalanine intake on the chemistry and behavior of a phenylketonuric child. Acta Paediatrica, 43, 64-77.
18. Bodis-Wollner, I. (1988). Altered spatio-temporal contrast vision in Parkinson's disease and MPTP-treated monkeys: The role of dopamine. In I. Bodis-Wollner & M. Piccolino (Eds.), Dopaminergic mechanisms in vision. New York: Alan Liss.
19. Bodis-Wollner, I. (1990). Visual deficits related to dopamine deficiency in experimental animals and Parkinson's disease patients. Trends in Neural Science, 13, 296-302.
20. Bodis-Wollner, I., Marx, M. S., Mitra, S., Bobak, P., Mylin, L., & Yahr, M. (1987). Visual dysfunction in Parkinson's disease: Loss in spatiotemporal contrast sensitivity. Brain, 110, 1675-1698.
21. Borkowski, J. G., Benton, A. L., & Spreen, O. (1967). Word fluency and brain damage. Neuropsychologia, 5, 135-140.
22. Bradberry, C. W., Karasic, D. H., Deutch, A. Y., & Roth, R. H. (1989). Regionally-specific alterations in mesotelencephalic dopamine synthesis in diabetic rats: Association with precursor tyrosine. Journal of Neural Transmission, 78, 221-229.
23. Brass, C. A., & Greengard, O. (1982). Modulation of cerebral catecholamine concentrations during hyperphenylalaninaemia. Biochemical Journal, 208, 765-771.
24. Brody, B. A., Ungerleider, L. G., & Pribram, K. H. (1977). The effects of instability of the visual display on pattern discrimination learning by monkeys: Dissociation produced after resections of frontal and inferotemporal cortex. Neuropsychologia, 15, 439-448.
25. Brozoski, T. J., Brown, R. M., Rosvold, H. E., & Goldman, P. S. (1979). Cognitive deficit caused by regional depletion of dopamine in prefrontal cortex of rhesus monkey. Science, 205, 929-932.
26. Brunner, R. L., Berch, D. B., & Berry, H. (1987). Phenylketonuria and complex spatial visualization: An analysis of information processing. Developmental Medicine and Child Neurology, 29, 460-468.
27. Brunner, R. L., Jordan, M. K., & Berry, H. K. (1983). Early-treated phenylketonuria: Neuropsychologic consequences. Journal of Pediatrics, 102, 831-835.
28. Brush, E. S., Mishkin, M., & Rosvold, H. E. (1961). Effects of object preferences and aversions on discrimination learning in monkeys with frontal lesions. Journal of Comparative and Physiological Psychology, 54, 319-325.
29. Bubser, M., & Schmidt, W. J. (1990). 6-hydroxydopamine lesion of the rat prefrontal cortex increases locomotor activity, impairs acquisition of delayed alternation tasks, but does not affect uninterrupted tasks in the radial maze. Behavioral Brain Research, 37, 157-168.
30. Bugbee, N. M., & Goldman-Rakic, P. S. (1981). Functional 2-deoxyglucose mapping in association cortex: Prefrontal activation in monkeys performing a cognitive task. Society for Neuroscience Abstracts, 7, 239.
31. Burgess, P. W., & Shallice, T. (1996). Response suppression, initiation and strategy use following frontal lobe lesions. Neuropsychologia, 34, 263-273.
32. Butters, N., Pandya, D., Sanders, K., & Dye, P. (1969). Behavioral deficits in monkeys after selective lesions within the middle third of sulcus principalis. Journal of Comparative and Physiological Psychology, 76, 8-14.
33. Casey, B. J., Cohen, J. D., Noll, D. C., Forman, S., & Rapoport, J. L. (1993). Activation of the anterior cingulate during the Stroop conflict paradigm using functional MRI. Society for Neuroscience Abstracts, 19, 1285.
34. Chirigos, M., Greengard, P., & Udenfriend, S. (1960). Uptake of tyrosine by rat brain in vivo. Journal of Biological Chemistry, 235, 2075-2079.
35. Churchland, A., & Diamond, A. (1996). Solving the riddle of the late appearance of success on the delayed nonmatching to sample task. Society for Neuroscience Abstracts, 22, 281.
36. Cohen, J. D., Forman, S., Braver, T. S., Casey, B. J., Servan-Schreiber, D., & Noll, D. C. (1994). Activation of prefrontal cortex in a non-spatial working memory task with functional MRI. Human Brain Mapping, 1, 293-304.
37. Cohen, L. B., & Gelber, E. R. (1975). Infant visual memory. In L. B. Cohen & P. Salapatek (Eds.), Infant perception: From sensation to cognition (Vol. 1). New York: Academic.
38. Corsi, P. M. (1972). Human memory and the medial temporal regions of the brain. Unpublished doctoral dissertation, McGill University.
39. Costello, P. M., Beasley, M. G., Tillotson, S. L., & Smith, I. (1994). Intelligence in mild atypical phenylketonuria. European Journal of Pediatrics, 153, 260-263.
40. Cowie, V. A. (1971). Neurological and psychiatric aspects of phenylketonuria. In H. Bickel, F. Hudson, & L. Woolf (Eds.), Phenylketonuria and some other inborn errors of amino acid metabolism. Stuttgart: Georg Thiese.
41. Craft, S., Gourovitch, M. L., Dowton, S. B., Swanson, J. M., & Bonforte, S. (1992). Lateralized deficits in visual attention in males with developmental dopamine depletion. Neuropsychologia, 30, 341-351.
42. Deiber, M.-P., Passingham, R. E., Colebatch, J. G., Friston, K. J., Nixon, P. D., & Frackowiak, R. S. J. (1991). Cortical areas and the selection of movement: A study with positron emission tomography. Experimental Brain Research, 84, 393-402.
43. de Sonneville, L. N. J., Schmidt, E., Michel, U., & Batzler, U. (1990). Preliminary neuropsychological test results. European Journal of Pediatrics, 149(Suppl. 1), S39-S44.
44. Diamond, A. (1981). Retrieval of an object from an open box: The development of visualtactile control of reaching in the first year of life. Society for Research in Child Development Abstracts, 3, 78.
45. Diamond, A. (1985). Development of the ability to use recall to guide action, as indicated by infants' performance on AB̄. Child Development, 56, 868-883.
46. Diamond, A. (1990a). Developmental time course in human infants and infant monkeys, and the neural bases, of inhibitory control in reaching. In A. Diamond (Ed.), The development and neural bases of higher cognitive functions. Annals of the New York Academy of Sciences, 608, 637-676.
47. Diamond, A. (1990b). The development and neural bases of memory functions as indexed by the AB̄ and delayed response tasks in human infants and infant monkeys. In A. Diamond (Ed.), The development and neural bases of higher cognitive functions. Annals of the New York Academy of Sciences, 608, 267-317.
48. Diamond, A. (1990c). Rate of maturation of the hippocampus and the developmental progression of children's performance on the delayed non-matching to sample and visual paired comparison tasks. In A. Diamond (Ed.), The development and neural bases of higher cognitive functions. Annals of the New York Academy of Sciences, 608, 394-426.
49. Diamond, A. (1991a). Frontal lobe involvement in cognitive changes during the first year of life. In K. R. Gibson & A. C. Petersen (Eds.), Brain maturation and cognitive development: Comparative and cross-cultural perspectives. New York: Aldine de Gruyter.
50. Diamond, A. (1991b). Neuropsychological insights into the meaning of object concept development. In S. Carey & R. Gelman (Eds.), The epigenesis of mind: Essays on biology and knowledge. Hillsdale, NJ: Erlbaum.
51. Diamond, A. (1995). Evidence of robust recognition memory early in life even when assessed by reaching behavior. Journal of Experimental Child Psychology, 59, 419-456.
52. Diamond, A., Ciaramitaro, V., Donner, E., Djali, S., & Robinson, M. (1994). An animal model of early-treated PKU. Journal of Neuroscience, 14, 3072-3082.
53. Diamond, A., Ciaramitaro, V., Donner, E., Hurwitz, W., Lee, E., Grover, W., & Minarcik, C. (1992). Prefrontal cortex cognitive deficits in early treated PKU: Results of a longitudinal study in children and of an animal model. Society of Neuroscience Abstracts, 18, 1063.
54. Diamond, A., & Doar, B. (1989). The performance of human infants on a measure of frontal cortex function, the delayed response task. Developmental Psychobiology, 22, 271-294.
55. Diamond, A., & Goldman-Rakic, P. S. (1985). Evidence for involvement of prefrontal cortex in cognitive changes during the first year of life: Comparison of performance of human infant and rhesus monkeys on a detour task with transparent barrier. Society for Neuroscience Abstracts, 11, 832.
56. Diamond, A., & Goldman-Rakic, P. S. (1986). Comparative development in human infants and infant rhesus monkeys of cognitive functions that depend on prefrontal cortex. Society for Neuroscience Abstracts, 12, 742.
57. Diamond, A., & Goldman-Rakic, P. S. (1989). Comparison of human infants and rhesus monkeys on Piaget's AB task: Evidence for dependence on dorsolateral prefrontal cortex. Experimental Brain Research, 74, 24-40.
58. Diamond, A., & Herzberg, C. (1996). Impaired sensitivity to visual contrast in children treated early and continuously for PKU. Brain, 119, 523-538.
59. Diamond, A., & Lee, E. (in preparation). Early success on the delayed nonmatching to sample task when the act of reaching for the stimulus immediately produces the reward.
60. Diamond, A., & Taylor, C. (1996). Development of an aspect of executive control: Development of the abilities to remember what I said and to "Do as I say, not as I do." Developmental Psychobiology, 29, 315-334.
61. Diamond, A., Towle, C., & Boyer, K. (1994). Young children's performance on a task sensitive to the memory functions of the medial temporal lobe in adults, the delayed nonmatching to sample task, reveals problems that are due to non-memory related task demands. Behavioral Neuroscience, 108, 1-22.
62. Diamond, A., Zola-Morgan, S., & Squire, L. (1989). Successful performance by monkeys with lesions of the hippocampal formation on AB and object retrieval, two tasks that mark developmental changes in human infants. Behavioral Neuroscience, 103, 526-537.
63. DiLella, A. G., Marvit, J., Lidsky, A. S., Güttler, F., & Woo, S. L. C. (1986). Tight linkage between a splicing mutation and a specific DNA haplotype in phenylketonuria. Nature, 322, 799-803.
64. Dobson, J. C., Kushida, E., Williamson, M. L., & Friedman, E. G. (1976). Intellectual performance of 36 phenylketonuric patients and their non-affected siblings. Pediatrics, 58, 53-58.
65. Dobson, J. C., Williamson, M. L., Azen, C., & Koch, R. (1977). Intellectual assessment of 111 four-year-old children with phenylketonuria. Pediatrics, 60, 822-827.
66. Drewe, E. A. (1975). An experimental investigation of Luria's theory on the effects of frontal lobe lesions in man. Neuropsychologia, 13, 421-429.
67. Dyer, C. A., Kendler, A., Philibotte, T., Gardiner, P., Cruz, J., & Levy, H. L. (1996). Evidence for central nervous system glial cell plasticity in phenylketonuria. Journal of Neutrapathology and Experimental Neurology, 55, 795-814.
68. Eslinger, P. J., & Grattan, L. M. (1993). Frontal lobe and frontal-striatal substrates for different forms of human cognitive flexibility. Neuropsychologia, 31, 17.
69. Fagan, J. F., III. (1970). Memory in the infant. Journal of Experimental Child Psychology, 9, 217-266.
70. Fagan, J. F., III. (1973). Infants' delayed recognition memory and forgetting. Journal of Experimental Child Psychology, 16, 424-450.
71. Fantz, R. L. (1964). Visual experience in infants: Decreased attention to familiar patterns relative to novel ones. Science, 146, 668-670.
72. Faust, D., Libon, D., & Pueschel, S. (1986). Neuropsychological functioning in treated phenylketonuria. International Journal of Psychiatry in Medicine, 16, 169-177.
73. Fernstrom, J. D., & Fernstrom, M. H. (1988). Tyrosine availability and dopamine synthesis in the retina. In I. Bodis-Wollner & M. Piccolino (Eds.), Dopaminergic mechanisms in vision. New York: Alan Liss.
74. Fernstrom, M. H., Baker, R. L., & Fernstrom, J. D. (1989). In vivo tyrosine hydroxylation rate in retina: Effects of phenylalanine and tyrosine administration in rats pretreated with p-chlorophenylalanine. Brain Research, 499, 291-298.
75. Fernstrom, M. H., Volk, E. A., & Fernstrom, J. D. (1986). In vivo inhibition of tyrosine uptake into rat retina by large neutral, but not acidic, amino acids. American Journal of Physiology, 251, E393-E399.
76. Fishier, K. (1993). Intellectual development in galactosemia. In G. N. Donnell (Ed.), Galactosemia: New frontiers in research. Washington, DC: National Institutes of Health.
77. Følling, A. (1934). Über Ausscheidung von Phenylbrenztraubensäure in der harn als Stoffwechselanomalie in verbindung mit imbezillität. Hoppe-Seyler's Ztschr. Physiol. Chem., 227, 169.
78. Fuster, J. M. (1973). Unit activity in prefrontal cortex during delayed-response performance: Neuronal correlates of transient memory. Journal of Neurophysiology, 36, 61-78.
79. Fuster, J. M., & Alexander, G. E. (1970). Delayed response deficit by cryogenic depression of frontal cortex. Brain Research, 20, 85-90.
80. Fuster, J. M., & Alexander, G. E. (1971). Neuron activity related to short-term memory. Science. 173, 652-654.
81. Gaffan, D., Gaffan, E. A., & Harrison, S. (1984). Effects of fornix transection on spontaneous and trained non-matching by monkeys. Quarterly Journal of Experimental Psychology, 36, 285-303.
82. Gerstadt, C., Hong, Y., & Diamond, A. (1994). The relationship between cognition and action: Performance of 31/2-7 year old children on a Stroop-like day-night test. Cognition, 53, 129-153.
83. Glick, S. D., Jerussi, T. P., & Zimmerberg, B. (1977). Behavioral and neuropharmacological correlates of nigrostriatal asymmetry in rats. In S. Haenad et al. (Eds.), Lateralisation of the nervous system. New York: Academic.
84. Goldman, P. S., & Rosvold, H. E. (1970). Localization of function within the dorsolateral prefrontal cortex of the rhesus monkey. Experimental Neurology, 27, 291-304.
85. Goldman, P. S., & Rosvold, H. E. (1972). The effects of selective caudate lesions in infant and juvenile rhesus monkeys. Brain Research, 43, 53-66.
86. Goldman-Rakic, P. S. (1987). Circuitry of primate prefrontal cortex and regulation of behavior by representational memory. Handbook of Physiology, 5, 373-417.
87. Goldstein, K. (1944). The mental changes due to frontal lobe damage. Journal of Psychology, 17, 187-208.
88. Greengard, O., & Brass, C. A. (1984). Developmental changes of cerebral phenylalanine uptake from severely elevated blood levels. Neurochemical Research, 9, 837-847.
89. Greengard, O., Yoss, M. S., & DelValle, J. A. (1976). α-methylphenylalanine, a new inducer of chronic hyperphenylalaninemia in suckling rats. Science, 192, 1007-1008.
90. Guitton, D., Buchtel, H. A., & Douglas, R. M. (1985). Frontal lobe lesions in man cause difficulties in suppressing reflexive glances and generating goal-directed saccades. Experimental Brain Research, 58, 455-472.
91. Guthrie, R. (1996). The introduction of newborn screening for phenylketonuria: A personal history. European Journal of Pediatrics, 155, S4-S5.
92. Guthrie, R. E., & Susi, A. (1963). A simple phenylalanine method for detecting phenylketonuria in large populations of newborn infants. Pediatrics, 32, 338-343.
93. Güttler, F. (1988). Epidemiology and natural history of phenylketonuria and other hyperphenylalaninemias. In R. J. Wurtman & E. Ritter-Walker (Eds.), Dietary phenylalanine and brain function. Boston: Birkhäuser.
94. Güttler, F., & Lou, H. C. (1986). Dietary problems of phenylketonuria: Effect on CNS transmitters and their possible role in behaviour and neuropsychological function. Journal of Inherited Metabolic Disease, 9, 169-177.
95. Harlow, H. F. (1950). Analysis of discrimination learning by monkeys. Journal of Experimental Psychology, 40, 26-39.
96. Holtzman, N. A., Kronmal, R. A., van Doorninck, W., Azen, C., & Koch, R. (1986). Effect of age at loss of dietary control on intellectual performance and behavior of children with phenylketonuria. New England Journal of Medicine, 314, 593-598.
97. Hommes, F. A. (1990). Demyelination in hyperphenylalaninemia. Annals of the New York Academy of Sciences, 605, 449-452.
98. Hommes, F. A., & Moss, L. (1992). Myelin turnover in hyperphenylalaninemia: A re-evaluation with the HPH-5 mouse. Journal of Inherited Metabolic Disease, 15, 243-251.
99. Hsia, D. Y.-Y. (1970). Phenylketonuria and its variants. Progress in Medical Genetics, 7, 29-68.
100. Hudson, F. P., Mordaunt, V. L., & Leahy, I. (1970). Evaluation of treatment begun in the first three months of life in 184 cases of phenylketonuria. Archives of Disease in Childhood, 45, 5-12.
101. Huether, G., Kaus, R., & Neuhoff, V. (1982). Brain development in experimental hyperphenylalaninemia: Myelination. Neuropediatrics, 13, 177-182.
102. Ikeda, M., Levitt, M., & Udenfriend, S. (1967). Phenylalanine as substrate and inhibitor of tyrosine hydroxylase. Archives of Biochemistry and Biophysics, 120, 42-47.
103. Irie, K., & Wurtman, R. J. (1987). Release of norepinephrine from rat hypothalamic slices: Effects of desipramine and tyrosine. Brain Research, 432, 391-394.
104. Iuvone, P. M., Galli, C. L., Garrison-Gund, C. K., & Neff, N. H. (1978). Light stimulates tyrosine hydroxylase activity and dopamine synthesis in retinal amacrine neurons. Science, 202, 901-902.
105. Iuvone, P. M., Tigges, M., Fernandes, A., & Tigges, J. (1989). Dopamine synthesis and metabolism in rhesus monkey retina: Development, aging, and the effects of monocular visual deprivation. Visual Neuroscience, 2, 465-471.
106. Jacobsen, C. F. (1936). Studies of cerebral function in primates: 1. The functions of the functional association areas in monkeys. Comparative Psychology Monographs, 13, 3-60.
107. Jacobsen, C. F., & Nissen, H. W. (1937). Studies of cerebral function in primates: The effects of frontal lobe lesions on the delayed alternation habit in monkeys. Journal of Comparative Physiological Psychology, 23, 101-112.
108. Jenkins, I. H., Brooks, D. J., Nixon, P. D., Frackowiak, R. S. J., & Passingham, R. E. (1994). Motor sequence learning: A study with positron emission tomography. Journal of Neuroscience, 14, 3775-3790.
109. Katz, I., Lloyd, T., & Kaufman, S. (1976). Studies on phenylalanine and tyrosine hydroxylation by rat brain tyrosine hydroxylase. Biochemica et Biophysica Acta, 445, 567-578.
110. Kaufman, S., Berlow, S., Summer, G. K., Milstein, S., Schulman, J. D., Orloff, S., Spielberg, S., & Pueschel, S. (1978). Hyperphenylalaninemia due to a deficiency of biopterin: A variant of phenylketonuria. New England Journal of Medicine, 299, 673-679.
111. Kaufman, S., Kapatos, G., McInnes, R. R., Schulman, J. D., & Rizzo, W. B. (1982). Use of tetrahydropterins in the treatment of hyperphenylalaninemia due to defective synthesis of tetrahydrobiopterin. Pediatrics, 70, 376-380.
112. Koch, R., Azen, C., Friedman, E. G., & Williamson, E. L. (1982). Preliminary report on the effects of diet discontinuation in PKU. Pediatrics, 100, 870-875.
113. Koch, R., Azen, C., Friedman, E. G., & Williamson, M. L. (1984). Paired comparisons between early-treated PKU children and their matched sibling controls on intelligence and school achievement test results at eight years of age. Journal of Inherited Metabolic Diseases, 7, 86-90.
114. Koch, R., & Wenz, E. (1987). Phenylketonuria. Annual Review of Nutrition, 7, 117-135.
115. Kolb, B., & Whishaw, I. (1985). Fundamentals of human neuropsychology. New York: W. H. Freeman.
116. Kowalska, D. M., Bachevalier, J., & Mishkin, M. (1991). The role of the inferior prefrontal convexity in performance of delayed nonmatching-to-sample. Neuropsychologia, 29, 583-600.
117. Krause, W. L., Helminski, M., McDonald, L., Dembure, P., Salvo, R., Freides, D., & Elsas, L. J. (1985). Biochemical and neuropsychological effects of elevated plasma phenylalanine in patients with treated phenylketonuria, a model for the study of phenylalanine in brain function in man. Journal of Clinical Investigation, 75, 40-48.
118. Kubota, K., & Niki, H. (1971). Prefrontal cortical unit activity and delayed alternation performance in monkeys. Journal of Neurophysiology, 34, 337-347.
119. Kupersmith, M. J., Shakin, E., Siegel, I. M., & Lieberman, A. (1982). Visual system abnormalities in patients with Parkinson's disease. Archives of Neurology, 39, 284-286.
120. Lajtha, A., Sershen, H., & Dunlop, D. (1988). Developmental changes in cerebral amino acids and protein metabolism. In G. Huether (Ed.), Amino acid availability and brain function in health and disease. Berlin: Springer.
121. Lamb, M. R., Robertson, L. C., & Knight, R. T. (1989). Attention and inference in the processing of global and local information: Effects of unilateral temporal parietal junction lesions. Neuropsychologia, 27, 471-483.
122. Lamb, M. R., Robertson, L. C., & Knight, R. T. (1990). Component mechanisms underlying the processing of hierarchically organized patterns: Interferences from patients with unilateral cortical lesions. Journal of Experimental Psychology, 16, 471-483.
123. Lang, M. J., Koch, R., Fishler, K., & Baker, R. (1989). Nonphenylketonuric hyperphenylalaninemia. American Journal of Diseases of Children, 143, 1464-1466.
124. Larsen, J. K., & Divac, I. (1978). Selective ablations within the prefrontal cortex of the rat and performance on delayed alternation. Physiological Psychology, 6, 15-17.
125. Ledley, F. D., Levy, H. L., & Woo, S. L. (1986). Molecular analysis of the inheritance of phenylketonuria and mild hyperphenylalaninemia in families with both disorders. New England Journal of Medicine, 314, 1276-1280.
126. Levitt, M., Spector, S., Sjoerdsma, A., & Udenfriend, S. (1965). Journal of Pharmacological Experimental Theory, 148, 1-8.
127. Levy, H. L., Shih, V. E., Karolkewicz, V., French, W. A., Carr, J. R., Cass, V., Kennedy, J. L., & MacCready, R. A. (1971). Persistent mild hyperphenylalaninemia in the untreated state. New England Journal of Medicine, 285, 424-428.
128. Levy, H. L., Waisbren, S. E., Lobbregt, D., Allred, E., Schuler, A., Trefz, F. K., Schweitzer, S. M., Sardharwalla, I. B., Walter, J. H., Barwell, B. E., Berlin, C. M., Jr., & Leviton, A. (1994). Maternal mild hyperphenylalaninaemia: An intentional survey of offspring outcome. Lancet, 344, 1589-1594.
129. Lewis, D. A., Campbell, M. J., Foote, S. L., & Morrison, J. H. (1986). The monoaminergic innervation of primate neocortex. Human Neurobiology, 5, 181-188.
130. Lewis, D. A., & Morrison, J. H. (1989). The noradrenergic innervation of monkey prefrontal cortex: A dopamine-ß-hydroxylase immunohistochemical study. Journal of Comparative Neurology, 282, 317-330.
131. L'Hermitte, F. (1983). "Utilization behavior" and its relation to lesions of the frontal lobes. Brain, 106, 237-255.
132. Lidsky, A. S., Law, M. L., Morse, H. G., Kao, F. T., & Woo, S. L. C. (1985). Regional mapping of the human phenylalanine hydroxylase gene and the PKU locus on chromosome 12. Proceedings of the National Academy of Science, 82, 6221-6225.
133. Lou, H. C., Lykkelund, C., Gerdes, A. M., Udesen, H., & Bruhn, P. (1987). Increased vigilance and dopamine synthesis by large doses of tyrosine or phenylalanine restriction in phenylketonuria. Acta Paediatrica, 76, 560-565.
134. Lovenberg, W., Jequier, E., & Sjoerdsma, A. (1968). Tryptophan hydroxylation in mammalian systems. In S. Garratini & P. A. Shore (Eds.), Advances in pharmacology (Vol. 6A). New York: Academic.
135. 2 dopamine receptor agonist. Journal of Cognitive Neuroscience, 4, 58-68.
136. Luria, A. R. (1966). Higher cortical functions in man (1st ed.). New York: Basic.
137. Luria, A. R. (1980). Higher cortical functions in man (2d ed.). New York: Basic.
138. Mangels, J. A. (1997). Strategic processing and memory for temporal order in patients with frontal lobe lesions. Neuropsychology, 11(2), 207-221.
139. Martin, M. (1979). Local and global processing: The role of sparsity. Memory and Cognition, 7, 476-484.
140. Mazzocco, M. M. M., Nord, A. M., van Doorninck, W., Greene, C. L., & Kovar, C. G. (1994). Cognitive development among children with early-treated phenylketonuria. Developmental Neuropsychology, 10, 133-151.
141. Mazzocco, M. M. M., Yannicelli, S., Nord, A. M., van Doorninck, W., Davidson-Mundt, A. J., Greene, C. L., & Pennington, B. F. (1992). Cognition and tyrosine supplementation among school-aged children with phenylketonuria. American Journal of Diseases of Children, 146, 1261-1264.
142. McAndrews, M. P., & Milner, B. (1991). The frontal cortex and memory for temporal order. Neuropsychologia, 9, 849-859.
143. hph-5: A mouse mutant deficient in phenylalanine hydroxylase. Proceedings of the National Academy of Science, 87, 1965-1967.
144. McKean, C. M. (1972). The effects of high phenylalanine concentrations on serotonin and catecholamine metabolism in the human brain. Brain Research, 47, 469-476.
145. McKee, R. D., & Squire, L. R. (1993). On the development of declarative memory. Journal of Experimental Psychology: Learning, Memory, and Cognition, 19, 397-404.
146. Medical Research Council. (1993). Phenylketonuria due to phenylalanine hydroxylase deficiency: An unfolding story. British Medical Journal, 306, 115-119.
147. Medical Research Council Working Party on Phenylketonuria. (1993). Recommendations on the dietary management of phenylketonuria. Archives of Disease in Childhood, 68, 426-427.
148. Mesulam, M.-M. (1995, May). Overview of human frontal lobes. Paper presented at the meeting of the American Academy of Neurology, Seattle.
149. Meunier, M., Bachevalier, J., Mishkin, M., & Murray, E. A. (1993). Effects on visual recognition of combined and separate ablations of the entorhinal and perirhinal cortex in rhesus monkeys. Journal of Neuroscience, 13, 5418-5432.
150. Miller, L., Braun, L. D., Pardridge, W. M., & Oldendorf, W. H. (1985). Kinetic constants for blood-brain barrier amino acid transport in conscious rats. Journal of Neurochemistry, 45, 1427-1432.
151. Milner, B. (1972). Disorders of learning and memory after temporal lobe lesions in man. Clinical Neurosurgery, 19, 421-446.
152. Milner, B. (1982). Some cognitive effects of frontal lobe lesions in man. Philosophical Transactions of the Royal Society of London, B298, 211-226.
153. Milner, B., Corsi, P., & Leonard, G. (1991). Frontal-lobe contribution to recency judgements. Neuropsychologia, 29, 601-618.
154. Milner, J. D., Irie, K., & Wurtman, R. J. (1986). Effects of phenylalanine on the release of endogenous dopamine from rat striatal slices. Journal of Neurochemistry, 47, 1444-1448.
155. Mishkin, M. (1978). Memory in monkeys severely impaired by combined but not separate removal of amygdala and hippocampus. Nature, 273, 297-298.
156. Mishkin, M., Prockop, E. S., & Rosvold, H. E. (1962). One trial object discrimination learning in monkeys with frontal lesions. Journal of Comparative and Physiological Psychology, 7, 357-364.
157. Murray, E. A., Bachevalier, J., & Mishkin, M. (1989). Effects of rhinal cortical lesions on visual recognition memory in rhesus monkeys. Society for Neuroscience Abstracts, 15, 342.
158. Navon, D. (1977). Forest before trees: The precedence of global features in visual perception. Cognitive Psychology, 9, 353-383.
159. Niki, H. (1974). Prefrontal unit activity during delayed alternation in the monkey. Brain Research, 68, 185-204.
160. Nord, A. M., McCabe, L., & McCabe, E. R. B. (1988). Biochemical and nutritional status of children with hyperphenylalaninemia. Journal of Inherited Metabolic Disease, 11, 431-432.
161. O'Driscoll, G. A., Alpert. N. M., Matthysse, S. W., Levy, D. L., Rauch, S. L., & Holzman, P. S. (1995). Functional neuroanatomy of antisaccade eye movements investigated with positron emission tomography. Proceedings of the National Academy of Science, 92, 925-929.
162. O'Flynn, M. E., & Hsia, D. (1968). Some observations on the dietary treatment of phenylketonuria. Neuropsychologia, 72, 260-262.
163. Oldendorf, W. H. (1973). Stereospecificity of blood brain barrier permeability to amino acids. American Journal of Physiology, 224, 967-969.
164. Overman, W. H., Bachevalier, J., Turner, M., & Peuster, A. (1992). Object recognition versus object discrimination: Comparison between human infants and monkey infants. Behavioral Neurosrience, 106, 15-29.
165. Owen, A. M., Morris, R. G., Sahakian, B. J., Polkey, C. E., & Robbins, T. W. (1996). Double dissociations of memory and executive functions in working memory tasks following frontal lobe excision, temporal lobe excisions or amygdala-hippocampectomy in man. Brain, 119, 1597-1615.
166. Pardo, J. V., Pardo, V. J., Janer, K. W., & Raichle, M. E. (1990). The anterior cingulate cortex mediates processing selection in the Stroop attentional conflict paradigm. Proceedings of the National Academy of Science, 87, 256-259.
167. Pardridge, W. M. (1977). Regulation of amino acid availability to the brain. In R. J. Wurtman &J. J. Wurtman (Eds.), Nutrition and the brain. New York: Raven.
168. Pardridge, W. M. (1988). Phenylalanine transport at the human blood-brain barrier. In R. J. Wurtman & E. Ritter-Walker (Eds.), Dietary phenylalanine and brain function. Boston: Birkhäuser.
169. Pardridge, W. M., & Oldendorf, W. H. (1977). Transport of metabolic substrates through the blood-brain barrier. Journal of Neurochemistry, 28, 5-12.
170. Passingham, R. (1995). The frontal lobes and voluntary action. Oxford: Oxford University Press.
171. Passingham, R. E. (1996). Attention to action. Philosophical Transactions of the Royal Society of London, B351, 1473-1479.
172. Pennington, B. F., van Doorninck, W., McCabe, L. L., & McCabe, E. R. B. (1985). Neuropsychological deficits in early treated phenylketonuric children. American Journal of Mental Deficiency, 89, 467-474.
173. Perret, E. (1974). The left frontal lobe of man and the suppression of habitual responses in verbal categorical behaviour. Neuropsychologia, 16, 527-537.
174. Petrides, M. (1993). Dissociations of human mid-dorsolateral from posterior dorsolateral frontal cortex in memory processing. Proceedings of the National Academy of Science, 90, 873-877.
175. Petrides, M. (1995). Impairments on nonspatial self-ordered and externally ordered working memory tasks after lesions of the mid-dorsal part of the lateral frontal cortex in the monkey. Journal of Neuroscience, 15, 359-375.
176. Petrides, M. (1996). Specialized systems for the processing of mnemonic information within the primate frontal cortex. Philosophical Transactions of the Royal Society of London, B351, 1455-1462.
177. Petrides, M., Alivisatos, B., Evans, A. C., & Meyer, E. (1993). Dissociation of human middorsolateral frontal cortex in memory processing. Proceedings of the National Academy of Science, 90, 875-877.
178. Petrides, M., Alivisatos, B., Meyer, E., & Evans, A. C. (1993). Functional activation of the human frontal cortex during performance of verbal working memory tasks. Proceedings of the National Academy of Science, 90, 878-882.
179. Petrides, M., & Milner, B. (1982). Deficits in subject-ordered tasks after frontal- and temporallobe lesions in man. Neuropsychologia, 220, 249-262.
180. Phelps, E. A., Hyder, F., Blamire, A. M., Rothman, D. L., & Shulman, R. G. (1994). Functional magnetic resonance imaging of frontal lobes during overt fluency. Society for Neuroscience Abstracts, 20, 5.
181. Piaget, J. (1954). The construction of reality in the child. New York: Basic. (Original work published 1937)
182. Pietz, J., Landwehr, R., Kutscha, A., Schmidt, H., de Sonneville, L. N. J., & Trefz, F. K. (1995). Effect of high-dose tyrosine supplementation on brain function in adults with phenylketonuria. Journal of Pediatrics, 127(6), 936-943.
183. Raichle, M. E., Fiez, J. A., Videen, T. O., Macleod, A. M. K., Pardo, J. V., Fox, P. T., & Petersen, S. E. (1994). Practice-related changes in human brain functional anatomy. Cerebral Cortex, 4, 8.
184. Rapoport, S. I. (1976). Sites and functions of the blood-aqueous and blood-vitreous barriers of the eye. In S. I. Rapoport (Ed.), Blood-brain barrier in physiology and medicine. New York: Raven.
185. Reason, J., & Mycielska, K. (1982). Absent-minded? The psychology of mental lapses and everyday errors. Englewood Cliffs, NJ: Prentice-Hall.
186. Regan, D., & Neima, D. (1984). Low-contrast letter charts in early diabetic retinopathy, ocular hypertension, glaucoma, and Parkinson's disease. British Journal of Ophthalmology, 68, 885-889.
187. Reynolds, R., Burri, R., Mahal, S., & Herschokowitz, N. (1992). Disturbed myelinogenisis and recovery in hyperphenylalaninemia in rats: An immunohistochemical study. Experimental Neurology, 115, 347-367.
188. Richer, F., Decary, A., Lapierre, M. F., Rouleau, I., & Bouvier, G. (1993). Target detection deficits in frontal lobotomy. Brain and Cognition, 21, 203-211.
189. Ris, M. D., Williams, S. E., Hunt, M. M., Berry, H. K., & Leslie, N. (1994). Early-treated phenylketonuria: Adult neuropsychologic outcome. Journal of Pediatrics, 124, 388-392.
190. Robbins, T. W. (1996). Dissociating executive functions of the prefrontal cortex. Philosophical Transactions of the Royal Society of London, B351, 1463-1472.
191. Roberts, A. C., de Salvia, M. A., Muir, J. L., Wilkinson, L. S., Everitt, B. J., & Robbins, T. W. (1991). The effects of selective prefrontal dopamine (DA) lesions on cognitive tests of frontal function in primates. Society for Neuroscience Abstracts, 17, 501.
192. Roberts, S., & Morelos, B. S. (1982). Inhibition of cerebral protein kinase activity and cyclic AMP-dependent ribosomal protein phosphorylation in experimental hyperphenylalaninemia. Biochemical Journal, 202, 343-351.
193. Robertson, L. C., & Delis, D. (1986). Part-whole processing in unilateral brain-damaged patients: Dysfunction of hierarchical organization. Neuropsychologia, 24, 363-370.
194. Robertson, L. C., Lamb, M. R., & Knight R. T. (1988). Effects of lesions of temporal-parietal junction on perceptual and attentional processing in humans. Journal of Neurosciences, 8, 3757-3769.
195. Robertson, L. C., Lamb, M. R., & Knight, R. T. (1991). Normal global-local analysis in patients with dorsolateral frontal lobe lesions. Neuropsychologia, 29, 939-967.
196. Roth, R. H. (1984). CNS dopamine autoreceptors: Distribution, pharmacology, and function. Annals of the New York Academy of Sciences, 430, 27-53.
197. Sahakian, B. J., Sarna, G. S., Kantamaneni, B. D., Jackson, A., Hutson, P. H., & Curzon, G. (1985). Association between learning and cortical catecholamines in non-drug-treated rats. Psychopharmacology, 86, 339-343.
198. Saint-Cyr, J. A., Wan, R. O., Doudet, D., & Aigner, T. G. (1988). Impaired detour reaching in rhesus monkeys after MPTP lesions. Society for Neuroscience Abstracts, 14, 389.
199. Saunders, R. C. (1989). Monkeys demonstrate high level of recognition memory in delayed non-matching to sample with retention intervals of 6 weeks. Society for Neuroscience Abstracts, 15, 342.
200. Sawaguchi, T., & Goldman-Rakic, P. S. (1991). D1 dopamine receptors in prefrontal cortex: Involvement in working memory. Science, 251, 947-950.
201. Schmidt, E., Rupp, A., Burgard, P., Pietz, J., Weglage, J., & de Sonneville, L. N. J. (1994). Sustained attention in adult phenylketonuria: The influence of the concurrent phenylalanine-blood-level. Journal of Clinical and Experimental Neuropsychology, 16(5), 681-688.
202. Schneider, J. S., & Kovelowski, C. J., II. (1990). Chronic exposure to low doses of MPTP: 1. Cognitive deficits in motor asymptomatic monkeys. Brain Research, 519, 122-128.
203. Shedden, J. M., Christoforou, N., Nahmias, C., Hahn, M. C., & Noll, D. C. (1997, March). Attending to global and local elements of form: fMRI of hemisphere asymmetries in normal subjects. Paper presented at the meeting of the Cognitive Neuroscience Society, Boston.
204. Shedlovsky, A., McDonald, J. D., Symula, D., & Dove, W. F. (1993). Mouse models of human phenylketonuria. Genetics, 134, 1205-1210.
205. Shimamura, A. P., Janowsky, J. S., & Squire, L. R. (1990). Memory for the temporal order of events in patients with frontal lobe lesions and amnesic patients. Neuropsychologia, 28, 803-813.
206. Simon, H., Scatton, B., & LeMoal, M. (1980). Dopaminergic AlO neurones are involved in cognitive functions. Nature, 286, 150-151.
207. Skrandies, W., & Gottlob, I. (1986). Alterations of visual contrast sensitivity in Parkinson's disease. Human Neurobiology, 5, 255-259.
208. Smith, I., & Beasley, M. (1989). Intelligence and behaviour in children with early treated phenylketonuria. European Journal of Clinical Nutrition, 43, 1-5.
209. Smith, I., Beasley, M. G., & Adee, A. E. (1991). Effect on intelligence of relaxing the low phenylalanine diet in phenylketonuria. Archives of Disease in Childhood, 66, 311-316.
210. Smith, I., Beasley, M. G., Wolff, O., & Adee, A. (1988). Behavior disturbance in 8-year-old children with early-treated phenylketonuria. Journal of Pediatrics, 112, 403-408.
211. Smith, M. L., Klim, P., Mallozzi, E., & Hanley, W. B. (1996). A test of the frontal-specificity hypothesis in the cognitive performance of adults with phenylketonuria. Developmental Neuropsychology, 12(3), 327-341.
212. Spreen, O., & Benton, A. L. (1969). Neurosensory center comprehensive examination for aphasia. Victoria: University of Victoria Neuropsychology Lab.
213. Squire, L. R., & Shimamura, A. P. (1986). Characterizing amnesic patients for neuro-behavioral study. Behavioral Neuroscience, 100, 866-877.
214. Squire, L. R., Zola-Morgan, S., & Chen, K. S. (1988). Human amnesia and animal models of amnesia: Performance of amnesic patients on tests designed for the monkey. Behavioral Neuroscience, 102, 210-221.
215. Stuss, D. T., & Benson, D. F. (1986). The frontal lobes. New York: Raven.
216. Stuss, D. T., & Benson, D. F. (1987). The frontal lobes and control of cognition and memory. In E. Perecman (Ed.), The frontal lobes revisited. New York: IRBN.
217. Suzuki, W. A., Zola-Morgan, S., Squire, L. R., & Amaral, D. G. (1993). Lesions of the perirhinal and parahippocampal cortices in the monkey produce long-lasting memory impairment in the visual and tactual modalities. Journal of Neuroscience, 13, 2430-2451.
218. Tarn, S.-Y., Elsworth, J. D., Bradberry, C. W., & Roth, R. H. (1990). Mesocortical dopamine neurons: High basal firing frequency predicts tyrosine dependence of dopamine synthesis. Journal of Neural Transmission, 81, 97-110.
219. Taylor, J. R., Roth, R. H., Sladek, J. R., Jr., & Redmond, D. E., Jr. (1990). Cognitive and motor deficits in the performance of the object retrieval detour task in monkeys (Cercopithecus aethiops sabaeus) treated with MPTP: Long-term performance and effect of transparency of the barrier. Behavioral Neuroscience, 104, 564-576.
220. Thierry, A. M., Tassin, J. P., Blanc, A., Stinus, L., Scatton, B., & Glowinski, J. (1977). Discovery of the mesocortical dopaminergic system: Some pharmacological and functional characteristics. Advanced Biomedical Psychopharmacology, 16, 5-12.
221. Tornquist, P., & Aim, A. (1986). Carrier-mediated transport of amino acids through the blood-retinal and blood-brain barriers. Graefe's Archive for Clinical and Experimental Ophthalmology, 224, 21-25.
222. Tourian, A. Y., & Sidbury, J. B. (1978). Phenylketonuria. In J. D. Stanbury, J. B. Wyngaarden, & D. Fredrickson (Eds.), The metabolic basis of inherited disease. New York: McGraw-Hill.
223. van der Schot, L. W., Doesburg, W. H., & Sengers, R. C. (1994, May). Thephenylalanine response curve in relation to growth and mental development in the first years of life. Paper presented at the International Symposium on the Occasion of the Sixtieth Anniversary of Følling's Discovery of Phenylketonuria, Elsinore, Denmark.
224. Warrington, E. K., James, M., & Kinsbourne, M. (1966). Drawing disability in relation to laterality of cerebral lesion. Brain, 89, 53-82.
225. Weglage, J., Pietsch, M., Fünders, B., Koch, H. G., & Ullrich, K. (1995). Neurological findings in early treated phenylketonuria. Acta Paediatrica, 84, 411-415.
226. Weglage, J., Schmidt, E., Fünders, B., Pietsch, M., & Ullrich, K. (1996). Sustained attention in untreated non-PKU-hyperphenylalaninemia. Journal of Clinical and Experimental Neuropsychology, 18, 343-348.
227. Welsh, M. C., Pennington, B. F., Ozonoff, S., Rouse, B., & McCabe, E. R. B. (1990). Neuropsychology of early-treated phenylketonuria: Specific executive function deficits. Child Development, 61, 1697-1713.
228. Wikmark, R. G. E., Divac, I., & Weiss, R. (1973). Delayed alternation in rats with lesions in the frontal lobes: Implications for a comparative neuropsychology of the frontal system. Brain, Behavior and Evolution, 8, 329-339.
229. Williamson, M. L., Koch, R., Azen, C., & Chang, C. (1981). Correlates of intelligence test results in treated phenylketonuric children. Pediatrics, 68, 161-167.
230. Woo, S. L. C., Lidsky, A. S., Güttler, F., Chandra, T., & Robson, K. J. H. (1983). Cloned human phenylalanine hydroxylase gene allows prenatal diagnosis and carrier detection of classical phenylketonuria. Nature, 306, 151-155.
231. Wurtman, R. J., Lorin, F., Mostafapour, S., & Fernstrom, J. D. (1974). Brain catechol synthesis: Control by brain tyrosine concentration. Science, 185, 183-184.
232. Zelazo, P. D., Frye, D., & Rapus, T. (1996). An age-related dissociation between knowing rules and using them. Cognitive Development, 11, 37-63.
233. Zinchenko, V. P., Chzhi-Tsin, V., & Tarakanov, V. V. (1963). The formation and development of perceptual activity. Soviet Psychology, 2, 3-12.
234. Zola-Morgan, S., Squire, L. R., & Amaral, D. G. (1989). Lesions of the perirhinal and parahippocampal cortex that spare the amygdala and hippocampal formation produce severe memory impairment. Journal of Neuroscience, 9, 4355-4370.