Regression of neuropsychological deficits in early-treated phenylketonurics during adolescence

Journal of Inherited Metabolic Disease

Volumn 22, Issue 6, 1999, Pages 693-705

  Weglage, J ^a   Pietsch, M ^a   Denecke, J ^a   Sprinz, A ^a   Feldmann, R ^a   Grenzebach, M ^a   Ullrich, K ^b  

^a UNIVERSITY OF MÜNSTER   (Germany)

^b UNIVERSITY OF HAMBURG   (Germany)

Author keywords

[No Author keywords available]

Indexed keywords

PHENYLALANINE;

ADOLESCENT; AGING; AMINO ACID BLOOD LEVEL; ARTICLE; ATTENTION; CLINICAL ARTICLE; CLINICAL TRIAL; CONTROLLED CLINICAL TRIAL; CONTROLLED STUDY; DIET RESTRICTION; FEMALE; HUMAN; INTELLIGENCE TEST; MALE; MOTOR PERFORMANCE; NEUROLOGIC DISEASE; NEUROPSYCHOLOGICAL TEST; PHENYLKETONURIA; SCHOOL CHILD;

ADOLESCENT; AGING; CENTRAL NERVOUS SYSTEM DISEASES; CHILD; FEMALE; HUMANS; INTELLIGENCE; LONGITUDINAL STUDIES; MALE; MENTAL DISORDERS; MOTOR SKILLS; PHENYLALANINE; PHENYLKETONURIAS;

EID: 0032766461     PISSN: 01418955     EISSN: None     Source Type: Journal    
DOI: 10.1023/A:1005587915468     Document Type: Article

References (42)

1. APS (Stellungnahme der Arbeitsgemeinschaft für Pädiatrische Stoffwechselstörungen) (1990) Diätetische Behandlung der Phenylketonurie. Monatsschr Kinderheilkd 138: 636.
2. Bäumler G (1985) Farbe Wort Interferenz Test (FWIT) nach JR Stroop. Göttingen: Verlag für Psychologie, 1-75.
3. Bench CJ, Frith CJ, Gasby PM, Friston KJ, Frankowiak RSJ, Dolan RJ (1993) Investigations of the functional anatomy of attention using the Stroop test. Neuropsychology 31: 907-922.
4. Bortz J (1983) Statistik. Berlin: Springer-Verlag.
5. Brickenkamp R (1994) Test d 2 Aufmerksamkeits-Belastungs-Test. Göttingen: Verlag für Psychologie, 1-98.
6. Brunner RL, Berry HK (1987) Phenylketonuria and sustained attention: the continuous performance test. Int J Clin Neuropsychol 9: 68-70.
7. Burgard P, Schmidt E, Rupp A, Schneider W, Bremer HJ (1996) Intellectual development of the patients of the German Collaborative Study of patients treated for phenylketonuria. Eur J Pediatr 155 (supplement 1): 33-38.
8. Burgard P, Rey F, Rupp A, Abadie V, Rey J (1997) Neuropsychological functions in early treated patients with phenylketonuria on and off diet. Results of a cross-national and cross-sectional study. Pediatr Res 41: 368-374.
9. Cleary MA, Walter JH, Wraith JE, et al (1994) Magnetic resonance imaging of the brain in phenylketonuria. Lancet 344: 87-90.
10. Diamond A (1994) Phenylalanine levels of 6 to 10mg/dl may not be as benign as once thought. Acta Paediatr (Suppl) 407: 89-91.
11. Faust D, Libon D, Pueschel S (1986) Neuropsychological functioning in treated phenylketonuria. Int J Psychiatry Med 16: 169-177.
12. German Paediatric Society (1990) Stellungnahme der Arbeitsgemeinschaft für Pädiatrische Stoffwechselstörungen APS zur diätetischen Behandlung der Phenylketonurie. Monatsschr Kinderheilkd 138: 636.
13. Griffiths P, Campbell R, Robinson P (1998) Executive function in treated phenylketonuria as measured by the one-back and two-back versions of the continuous performance task. J Inher Metab Dis 21: 125-135.
14. Holtzman NA, Kronmal RA, 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. N Engl J Med 314: 593-598.
15. Hommes FA (1994) Loss of neurotransmitter receptors by hyperphenylalaninemia in the MPH-5 mouse brain. Acta Paediatr (Suppl) 407: 120-121.
16. Huttenlocher PR (1975) Synaptic density in frontal lobe cortex: developmental stages and effects of aging. Brain Res 163: 195-205.
17. Kirkcaldy B (1986) Differential psychomotor skills in a clinical group. Pers Indiv Diff 7: 853-859.
18. 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.
19. Lou HC, Lykkelund C, Gerdes AM, Udesen H, Bruhn P (1987) Increased vigilance and dopamine synthesis by large doses of tyrosine or phenylalanine restriction in phenylketonuria. Acta Paediatr 76: 560-565.
20. McKean CM (1972) The effects of high phenylalanine concentrations on serotonin and catecholamine metabolism in the human brain. Brain Res 47: 469-476.
21. Medical Research Council Working Party on Phenylketonuria (1993) Recommendations on the dietary management of phenylketonuria. Arch Dis Child 68: 426-427.
22. Milner JD, Wurtman RJ (1986) Effects of phenylalanine on the release of endogenous dopamine from rat striatal slices. J Neurochem 47: 1444-1448.
23. Pardo JV, Pardo PJ, Janer KW, Raichle ME (1990) The anterior cingulate cortex mediates processing selection in the Stroop attentional conflict paradigm. Proc Natl Acad Sci USA 87:256-259.
24. Perret E (1974) The left frontal lobe of man and the suppression of habitual responses in verbal categorical behaviour. Neuropsychology 12: 323-330.
25. Pietz J, Fätkenheuer B, Burgard P, Armbruster M, Esser G, Schmidt H (1997) Psychiatric disorders in adult patients with early treated phenylketonuria. J Pediatr 99: 345-350.
26. Pratt OE (1992) Transport inhibition in the pathology of phenylketonuria and other inherited metabolic diseases. J Inher Metab Dis 2 (supplement 2): 75-81.
27. Quatember R, Maly J (1980) Neuropsychologische Untersuchungsmethoden altersspezifischer Leistungsparameter. Wien Med Wochenschr 30: 688-692.
28. Rey F, Abadie V, Plainguet F, Rey J (1996) Long-term follow up of patients with classical phenylketonuria after diet relaxation at 5 years of age. The Paris Study. Eur J Pediatr 155 (supplement 1): 39-44.
29. Ris MD, Weber AM, Hunt MM, Berry HK, Williams SE, Leslie N (1997) Adult psychosocial outcome in early-treated phenylketonuria. J Inher Metab Dis 20: 499-508.
30. Schmidt E, Rupp A, Burgard P, Pietz J, Weglage J, Sonneville de L (1994) Sustained attention in adult phenylketonuria: the influence of the concurrent phenylalanine blood level. J Clin Exp Neuropsychol 16: 681-688.
31. Schmidt E, Burgard P, Rupp A (1996) Effects of concurrent phenylalanine levels on sustained attention and calculation speed in patients treated early for phenylketonuria. Eur J Pediatr 155 (supplement 1): 82-88.
32. Schoppe KJ (1974) Das MLS-Gerät: Ein neuer Testapparat zur Messung feinmotorischer Leistungen. Diagnostica 20: 43-46.
33. Scriver CR, Kaufman S, Eisensmith RC, Woo SLC (1995) The hyperphenylalaninemias. In: Scriver CR, Beaudet AL, Sly WS, Valle D, eds. The Metabolic and Molecular Bases of Inherited Diseases, 7th edn. New York: MacGraw-Hill, 1015-1076.
34. Smith I (1994) Treatment of phenylalanine hydroxylase deficiency. Acta Paediatr (Suppl) 407: 60-65.
35. Smith I, Beaseley MG, Ades AE (1991) Effect on intelligence of relaxing the low phenylalanine diet in phenylketonuria. Arch Dis Child 65: 311-316.
36. Waisbren SE, Brown MJ, Sonneville de LMJ, Levy HL (1994) Review of neuropsychological functioning in treated phenylketonuria: an information processing approach. Acta Paediatr (Suppl) 407: 98-103.
37. Weglage J, Fünders B, Wilken B, et al (1992) Psychological and social findings in adolescents with phenylketonuria. Eur J Pediatr 151: 522-525.
38. Weglage J, Fünders B, van Teeffelen-Heithoff A, Ullrich K (1993) Behandlung der Phenylketonurie: Wunsch und Wirklichkeit. Monatsschr Kinderheilkd 141: 670-674.
39. Weglage J, Pietsch M, Fünders B, Koch HG, Ullrich K (1995) Neurological findings in early treated phenylketonuria. Acta Paediatr 84: 411-415.
40. 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.
41. Weiss RH (1987) Grundintelligenztest Skala 2 CFT 20. Göttingen: Verlag für Psychologie, pp 1-67.
42. Welsh MC, Pennington BF, Ozonoff S, Rouse B, McCabe ERB (1990) Neuropsychology of early treated phenylketonuria: specific executive function deficits. Child Dev 61: 1697-1713.