Tackling frontal lobe-related functions in PKU through functional brain imaging: A Stroop task in adult patients

Journal of Inherited Metabolic Disease

Volumn 34, Issue 3, 2011, Pages 711-721

  Sundermann, Benedikt ^a   Pfleiderer, Bettina ^a   Möller, Harald E ^b   Schwindt, Wolfram ^a   Weglage, Josef ^a   Lepsien, Jöran ^b   Feldmann, Reinhold ^a  

^a UNIVERSITY HOSPITAL MÜNSTER   (Germany)

^b MAX PLANCK INSTITUTE FOR HUMAN COGNITIVE AND BRAIN SCIENCES   (Germany)

Author keywords

[No Author keywords available]

Indexed keywords

PHENYLALANINE; PLACEBO;

ACCURACY; ADULT; AMINO ACID BLOOD LEVEL; ARTICLE; CLINICAL ARTICLE; CONTROLLED STUDY; EXECUTIVE FUNCTION; FRONTAL LOBE; FUNCTIONAL MAGNETIC RESONANCE IMAGING; HUMAN; MALE; NEUROIMAGING; PHENYLKETONURIA; RESPONSE TIME; STROOP TEST; TASK PERFORMANCE;

ADULT; BEHAVIOR; BRAIN; FRONTAL LOBE; HUMANS; INTELLIGENCE TESTS; MAGNETIC RESONANCE IMAGING; MALE; NEUROPSYCHOLOGICAL TESTS; PHENYLALANINE; PHENYLKETONURIAS; PLACEBOS; YOUNG ADULT;

EID: 79959788921     PISSN: 01418955     EISSN: 15732665     Source Type: Journal    
DOI: 10.1007/s10545-011-9318-4     Document Type: Article

References (70)

1. Alvarez JA, Emory E (2006) Executive function and the frontal lobes: a meta-analytic review. Neuropsychol Rev 16:17-42 (Pubitemid 43954075)
2. Bäumler G (1985) Farbe-Wort-Interferenztest nach J. R. Stroop (FWIT). Hogrefe Verlag für Psychologie, Göttingen
3. Beglinger LJ, Gaydos B, Tangphao-Daniels O et al (2005) Practice effects and the use of alternate forms in serial neuropsychological testing. Arch Clin Neuropsychol 20:517-529 (Pubitemid 40719554)
4. Behrmann M, Geng JJ, Shomstein S (2004) Parietal cortex and attention. Curr Opin Neurobiol 14:212-217 (Pubitemid 38479939)
5. Bick U, Ullrich K, Stober U et al (1993) White matter abnormalities in patients with treated hyperphenylalaninaemia: magnetic resonance relaxometry and proton spectroscopy findings. Eur J Pediatr 152:1012-1020 (Pubitemid 23361979)
6. Bradberry CW, Karasic DH, Deutch AY, Roth RH (1989) Regionally-specific alterations in mesotelencephalic dopamine synthesis in diabetic rats: association with precursor tyrosine. J Neural Transm Gen Sect 78:221-229 (Pubitemid 19264986)
7. Brass CA, Greengard O (1982) Modulation of cerebral catecholamine concentrations during hyperphenylalaninaemia. Biochem J 208:765-771 (Pubitemid 13119423)
8. Brass M, Derrfuss J, Forstmann B, von Cramon DY (2005) The role of the inferior frontal junction area in cognitive control. Trends Cogn Sci 9:314-316 (Pubitemid 40910328)
9. Brumm VL, Azen C, Moats RA et al (2004) Neuropsychological outcome of subjects participating in the PKU adult collaborative study: a preliminary review. J Inherit Metab Dis 27:549-566 (Pubitemid 39295097)
10. Carter CS, van Veen V (2007) Anterior cingulate cortex and conflict detection: an update of theory and data. Cogn Affect Behav Neurosci 7:367-379
11. Channon S, German E, Cassina C, Lee P (2004) Executive functioning, memory, and learning in phenylketonuria. Neuropsychology 18:613-620 (Pubitemid 39363146)
12. Channon S, Mockler C, Lee P (2005) Executive functioning and speed of processing in phenylketonuria. Neuropsychology 19:679-686 (Pubitemid 41429115)
13. Chiodo LA, Bannon MJ, Grace AA, Roth RH, Bunney BS (1984) Evidence for the absence of impulse-regulating somatodendritic and synthesis-modulating nerve terminal autoreceptors on subpopulations of mesocortical dopamine neurons. Neuroscience 12:1-16 (Pubitemid 14077673)
14. Choi JK, Chen YI, Hamel E, Jenkins BG (2006) Brain hemodynamic changes mediated by dopamine receptors: role of the cerebral microvasculature in dopamine-mediated neurovascular coupling. Neuroimage 30:700-712
15. Derrfuss J, Brass M, Neumann J, von Cramon DY (2005) Involvement of the inferior frontal junction in cognitive control: meta-analyses of switching and Stroop studies. Hum Brain Mapp 25:22-34 (Pubitemid 40656190)
16. Derrfuss J, Brass M, von Cramon DY, Lohmann G, Amunts K (2009) Neural activations at the junction of the inferior frontal sulcus and the inferior precentral sulcus: interindividual variability, reliability, and association with sulcal morphology. Hum Brain Mapp 30:299-311
17. Devlin JT, Jamison HL, Gonnerman LM, Matthews PM (2006) The role of the posterior fusiform gyrus in reading. J Cogn Neurosci 18:911-922 (Pubitemid 44066129)
18. Diamond A (2007) Consequences of variations in genes that affect dopamine in prefrontal cortex. Cereb Cortex 17(Suppl 1):i161-i170
19. Diamond A, Ciaramitaro V, Donner E, Djali S, Robinson MB (1994) An animal model of early-treated PKU. J Neurosci 14:3072-3082 (Pubitemid 24138994)
20. Diamond A, Prevor MB, Callender G, Druin DP (1997) Prefrontal cortex cognitive deficits in children treated early and continuously for PKU. Monogr Soc Res Child Dev 62(i-v):1-208
21. Feldmann R, Denecke J, Pietsch M, Grenzebach M, Weglage J (2002) Phenylketonuria: no specific frontal lobe-dependent neuropsychological deficits of early-treated patients in comparison with diabetics. Pediatr Res 51:761-765 (Pubitemid 34547342)
22. Feldmann R, Denecke J, Grenzebach M, Weglage J (2005) Frontal lobe-dependent functions in treated phenylketonuria: blood phenylalanine concentrations and long-term deficits in adolescents and young adults. J Inherit Metab Dis 28:445-455 (Pubitemid 40753672)
23. Forman SD, Cohen JD, Fitzgerald M, Eddy WF, Mintun MA, Noll DC (1995) Improved assessment of significant activation in functional magnetic resonance imaging (fMRI): use of a cluster-size threshold. Magn Reson Med 33:636-647
24. Gonzalez F, Relova JL, Prieto A, Peleteiro M, Romero MC (2006) Hemifield dependence of responses to colour in human fusiform gyrus. Vis Res 46:2499-2504
25. Hanley WB (2004) Adult phenylketonuria. Am J Med 117:590-595
26. Huettel SA, Song AW, McCarthy G (2009) Functional magnetic resonance imaging. Sinauer Associates, Sunderland
27. Iadecola C (1998) Neurogenic control of the cerebral microcirculation: is dopamine minding the store? Nat Neurosci 1:263-265
28. Kastner S, Ungerleider LG (2000) Mechanisms of visual attention in the human cortex. Annu Rev Neurosci 23:315-341 (Pubitemid 30350046)
29. Krimer LS, Muly EC 3rd, Williams GV, Goldman-Rakic PS (1998) Dopaminergic regulation of cerebral cortical microcirculation. Nat Neurosci 1:286-289
30. Lancaster JL, Woldorff MG, Parsons LM et al (2000) Automated Talairach atlas labels for functional brain mapping. Hum Brain Mapp 10:120-131 (Pubitemid 30456920)
31. Landvogt C, Mengel E, Bartenstein P et al (2008) Reduced cerebral fluoro-L-dopamine uptake in adult patients suffering from phenylketonuria. J Cereb Blood Flow Metab 28:824-831 (Pubitemid 351432786)
32. Lemay S, Bedard MA, Rouleau I, Tremblay PL (2004) Practice effect and test-retest reliability of attentional and executive tests in middle-aged to elderly subjects. Clin Neuropsychol 18:284-302 (Pubitemid 39180732)
33. Leuzzi V, Seri S, Cerquiglini A, Carducci C, Carducci C, Antonozzi I (2000) Derangement of the dopaminergic system in phenylketonuria: study of the event-related potential (P300). J Inherit Metab Dis 23:317-320 (Pubitemid 30364760)
34. Leuzzi V, Tosetti M, Montanaro D et al (2007) The pathogenesis of the white matter abnormalities in phenylketonuria. A multimodal 3.0 Tesla MRI and magnetic resonance spectroscopy (1H MRS) study. J Inherit Metab Dis 30:209-216 (Pubitemid 46488003)
35. Logothetis NK, Wandell BA (2004) Interpreting the BOLD signal. Annu Rev Physiol 66:735-769 (Pubitemid 40614461)
36. Lou HC, Güttler F, Lykkelund C, Bruhn P, Niederwieser A (1985) Decreased vigilance and neurotransmitter synthesis after discontinuation of dietary treatment for phenylketonuria in adolescents. Eur J Pediatr 144:17-20 (Pubitemid 15059789)
37. MacLeod CM (1991) Half a century of research on the Stroop effect: an integrative review. Psychol Bull 109:163-203
38. McKean CM (1972) The effects of high phenylalanine concentrations on serotonin and catecholamine metabolism in the human brain. Brain Res 47:469-476
39. McKeefry DJ, Zeki S (1997) The position and topography of the human colour centre as revealed by functional magnetic resonance imaging. Brain 120:2229-2242 (Pubitemid 28014599)
40. Menz MM, Neumann J, Müller K, Zysset S (2006) Variability of the BOLD response over time: an examination of within-session differences. Neuroimage 32:1185-1194 (Pubitemid 44278709)
41. Möller HE, Weglage J, Wiedermann D, Vermathen P, Bick U, Ullrich K (1997) Kinetics of phenylalanine transport at the human blood-brain barrier investigated in vivo. Brain Res 778:329-337 (Pubitemid 28037413)
42. Möller HE, Weglage J, Wiedermann D, Ullrich K (1998) Blood-brain barrier phenylalanine transport and individual vulnerability in phenylketonuria. J Cereb Blood Flow Metab 18:1184-1191 (Pubitemid 28509832)
43. Möller HE, Weglage J, Bick U, Wiedermann D, Feldmann R, Ullrich K (2003) Brain imaging and proton magnetic resonance spectroscopy in patients with phenylketonuria. Pediatrics 112:1580-1583 (Pubitemid 37521701)
44. Möller HE, Feldmann R, Santer R, Ullrich K, Weglage J (2006) Phenylketonuria and blood-brain barrier competition investigated by magnetic resonance spectroscopy. In: Blau N, Christ SE, Finger S (eds) PKU and BH4 - advances in phenylketonuria and tetrahydrobiopterin. SPS-Verlagsgesellschaft, Heilbronn, pp 137-160
45. Moyle JJ, Fox AM, Arthur M, Bynevelt M, Burnett JR (2007) Meta-analysis of neuropsychological symptoms of adolescents and adults with PKU. Neuropsychol Rev 17:91-101 (Pubitemid 46889563)
46. Norris DG, Zysset S, Mildner T, Wiggins CJ (2002) An investigation of the value of spin-echo-based fMRI using a Stroop color-word matching task and EPI at 3T. Neuroimage 15:719-726
47. Pietz J, Schmidt E, Matthis P, Kobialka B, Kutscha A, de Sonneville L (1993) EEGs in phenylketonuria. I: Follow-up to adulthood. II: Short-term diet-related changes in EEGs and cognitive function. Dev Med Child Neurol 35:54-64
48. Pietz J, Kreis R, Schmidt H, Meyding-Lamade UK, Rupp A, Boesch C (1996) Phenylketonuria: findings at MR imaging and localized in vivo H-1 MR spectroscopy of the brain in patients with early treatment. Radiology 201:413-420 (Pubitemid 26357636)
49. Pietz J, Kreis R, Rupp A et al (1999) Large neutral amino acids block phenylalanine transport into brain tissue in patients with phenylketonuria. J Clin Invest 103:1169-1178 (Pubitemid 29207251)
50. Pietz J, Rupp A, Ebinger F et al (2003) Cerebral energy metabolism in phenylketonuria: findings by quantitative in vivo 31P MR spectroscopy. Pediatr Res 53:654-662 (Pubitemid 36406737)
51. Puglisi-Allegra S, Cabib S, Pascucci T, Ventura R, Cali F, Romano V (2000) Dramatic brain aminergic deficit in a genetic mouse model of phenylketonuria. Neuroreport 11:1361-1364 (Pubitemid 30262988)
52. Raichle ME, Snyder AZ (2007) A default mode of brain function: a brief history of an evolving idea. Neuroimage 37:1083-1090 (Pubitemid 47385262)
53. Roesch-Ely D, Scheffel H, Weiland S et al (2005) Differential dopaminergic modulation of executive control in healthy subjects. Psychopharmacology (Berl) 178:420-430 (Pubitemid 40486982)
54. Starrfelt R, Gerlach C (2007) The visual what for area: words and pictures in the left fusiform gyrus. Neuroimage 35:334-342
55. Strauss GP, Allen DN, Jorgensen ML, Cramer SL (2005) Test-retest reliability of standard and emotional Stroop tasks: an investigation of color-word and picture-word versions. Assessment 12:330-337
56. Stroop JR (1935) Studies of interference in serial verbal reactions. J Exp Psychol 18:643-662
57. Surtees R, Blau N (2000) The neurochemistry of phenylketonuria. Eur J Pediatr 159(Suppl 2):S109-S113
58. Talairach JTP (1988) Co-planar stereotaxic atlas of the human brain. Thieme Medical, New York
59. Tam SY, Roth RH (1997) Mesoprefrontal dopaminergic neurons: can tyrosine availability influence their functions? Biochem Pharmacol 53:441-453
60. Tam SY, Elsworth JD, Bradberry CW, Roth RH (1990) Mesocortical dopamine neurons: high basal firing frequency predicts tyrosine dependence of dopamine synthesis. J Neural Transm Gen Sect 81:97-110 (Pubitemid 20175065)
61. Vernaleken I, Buchholz HG, Kumakura Y et al (2007) 'Prefrontal' cognitive performance of healthy subjects positively correlates with cerebral FDOPA influx: an exploratory [18F]-fluoro-L-DOPA-PET investigation. Hum Brain Mapp 28:931-939
62. Weglage J, Pietsch M, Fünders B, Koch HG, Ullrich K (1996) Deficits in selective and sustained attention processes in early treated children with phenylketonuria-result of impaired frontal lobe functions? Eur J Pediatr 155:200-204
63. Weglage J, Pietsch M, Denecke J et al (1999) Regression of neuropsychological deficits in early-treated phenylketonurics during adolescence. J Inherit Metab Dis 22:693-705 (Pubitemid 29389349)
64. Weglage J, Moller HE, Feldmann R (2010) Self-assessed and objective blood phenylalanine levels in patients with early treated phenylketonuria. Acta Paediatr 99:944-945
65. Welsh MC (1996) A prefrontal dysfunction model of early-treated phenylketonuria. Eur J Pediatr 155(Suppl 1):S87-S89
66. Welsh MC, Pennington BF, Ozonoff S, Rouse B, McCabe ER (1990) Neuropsychology of early-treated phenylketonuria: specific executive function deficits. Child Dev 61:1697-1713
67. White DA, Nortz MJ, Mandernach T, Huntington K, Steiner RD (2001) Deficits in memory strategy use related to prefrontal dysfunction during early development: evidence from children with phenylketonuria. Neuropsychology 15:221-229 (Pubitemid 32878634)
68. Wiersema JR, van der Meere JJ, Roeyers H (2005) State regulation and response inhibition in children with ADHD and children with early- and continuously treated phenylketonuria: an event-related potential comparison. J Inherit Metab Dis 28:831-843 (Pubitemid 43168919)
69. Zysset S, Müller K, Lohmann G, von Cramon DY (2001) Color-word matching Stroop task: separating interference and response conflict. Neuroimage 13:29-36 (Pubitemid 34188241)
70. Zysset S, Schroeter ML, Neumann J, Yves von Cramon D (2007) Stroop interference, hemodynamic response and aging: an event-related fMRI study. Neurobiol Aging 28:937-946 (Pubitemid 46589745)