High phenylalanine levels directly affect mood and sustained attention in adults with phenylketonuria: A randomised, double-blind, placebo-controlled, crossover trial

Journal of Inherited Metabolic Disease

Volumn 34, Issue 1, 2011, Pages 165-171

  Ten Hoedt, Amber E ^a   De Sonneville, Leo M J ^b   Francois, Baudouin ^c   Ter Horst, Nienke M ^a   Janssen, Mirian C H ^d   Rubio Gozalbo, M Estela ^e   Wijburg, Frits A ^a   Hollak, Carla E M ^a   Bosch, Annet M ^a  

^a UNIVERSITY OF AMSTERDAM   (Netherlands)

^b LEIDEN UNIVERSITY   (Netherlands)

^c Department of Metabolic Diseases   (Belgium)

^d RADBOUD UNIVERSITY MEDICAL CENTER   (Netherlands)

^e MAASTRICHT UNIVERSITY MEDICAL CENTER   (Netherlands)

Author keywords

[No Author keywords available]

Indexed keywords

PHENYLALANINE; PLACEBO;

ADULT; AMINO ACID BLOOD LEVEL; ARTICLE; ATTENTION DISTURBANCE; CONTROLLED STUDY; CROSSOVER PROCEDURE; DIET SUPPLEMENTATION; DIETARY INTAKE; DOUBLE BLIND PROCEDURE; FEMALE; FRIEND; HUMAN; MAJOR CLINICAL STUDY; MALE; MOOD DISORDER; MULTICENTER STUDY; NEUROPSYCHOLOGICAL TEST; PHENYLALANINE BLOOD LEVEL; PHENYLKETONURIA; PROFILE OF MOOD STATES; PROTEIN RESTRICTION; RANDOMIZED CONTROLLED TRIAL; RELATIVE; TREATMENT OUTCOME; AFFECT; ATTENTION; BLOOD; CLINICAL TRIAL; CONTROLLED CLINICAL TRIAL; DRUG EFFECT; PSYCHOLOGICAL ASPECT;

ADULT; AFFECT; ATTENTION; CROSS-OVER STUDIES; DIETARY SUPPLEMENTS; DOUBLE-BLIND METHOD; FEMALE; HUMANS; MALE; NEUROPSYCHOLOGICAL TESTS; PHENYLALANINE; PHENYLKETONURIAS; PLACEBOS; YOUNG ADULT;

EID: 79955842584     PISSN: 01418955     EISSN: 15732665     Source Type: Journal    
DOI: 10.1007/s10545-010-9253-9     Document Type: Article

References (34)

1. Albrecht J, Garbade SF, Burgard P (2009) Neuropsychological speed tests and blood phenylalanine levels in patients with phenylketonuria: a meta-analysis. Neurosci Biobehav Rev 33:414-421
2. Beauchamp MH, Anderson V (2010) SOCIAL: an integrative framework for the development of social skills. Psychol Bull 136:39-64
3. Bik-Multanowski M, Didycz B, Mozrzymas R et al (2008) Quality of life in noncompliant adults with phenylketonuria after resumption of the diet. J Inherit Metab Dis. Short report #133 online
4. Burgard P (2000) Development of intelligence in early treated phenylketonuria. Eur J Pediatr 159:S74-S79
5. Burgard P, Rey F, Rupp A, Abadie V, Rey J (1997) Neuropsychologic functions of early treated patients with phenylketonuria, on and off diet: results of a cross-national and cross-sectional study. Pediatr Res 41:368-374 (Pubitemid 27097904)
6. Christ SE, Huijbregts SCJ, de Sonneville LMJ, White DA (2010a) Executive function in early-treated phenylketonuria: profile and underlying mechanisms. Mol Genet Metab 99:S22-S32
7. Christ SE, Moffitt AJ, Peck D (2010b) Disruption of prefrontal function and connectivity in individuals with phenylketonuria. Mol Genet Metab 99:S33-S340
8. Cohen J (1988) Statistical power analysis for the behavioral sciences, 2nd edn. Erlbaum, Hillsdale, NJ
9. De Sonneville LM (2005) Amsterdam neuropsychological tasks: scientific and clinical applications. Tijdschr Neuropsychol 0:27-41
10. De Sonneville LM (2009) Amsterdam neuropsychological tasks: a computer-aided assessment program. In: Den Brinker BPLM, Beek PJ, Brand AN, Maarse SJ, Mulder LJM (eds) Computers in psychology: cognitive ergonomics, clinical assessment and computer-assisted learning. Swets & Zeitlinger, Lisse, pp 187-203
11. De Sonneville LM, Boringa JB, Reuling IE, Lazeron RH, Ader HJ, Polman CH (2002) Information processing characteristics in subtypes of multiple sclerosis. Neuropsychologia 40:1751-1765 (Pubitemid 34626423)
12. 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.
13. Hargreaves KM, Pardridge WM (1988) Neutral amino acid transport at the human blood-brain barrier. J Biol Chem 263:19392-19397 (Pubitemid 19016415)
14. Huijbregts S, de Sonneville L, Licht R, Sergeant J, Van Spronsen F (2002a) Inhibition of prepotent responding and attentional flexibility in treated phenylketonuria. Dev Neuropsychol 22:481-499 (Pubitemid 36120225)
15. Huijbregts SC, De Sonneville LM, Licht R, Van Spronsen FJ, Sergeant JA (2002b) Short-term dietary interventions in children and adolescents with treated phenylketonuria: effects on neuropsychological outcome of a well-controlled population. J Inherit Metab Dis 25:419-430 (Pubitemid 36104377)
16. Huijbregts SC, De Sonneville LM, Licht R, Van Spronsen FJ, Verkerk PH, Sergeant JA (2002c) Sustained attention and inhibition of cognitive interference in treated phenylketonuria: associations with concurrent and lifetime phenylalanine concentrations. Neuropsychologia 40:7-15 (Pubitemid 32929971)
17. Huijbregts SC, De Sonneville LM, Van Spronsen FJ et al (2003) Motor function under lower and higher controlled processing demands in early and continuously treated phenylketonuria. Neuropsychology 17:369-379 (Pubitemid 37013148)
18. Joseph B, Dyer CA (2003) Relationship between myelin production and dopamine synthesis in the PKU mouse brain. J Neurochem 86:615-626 (Pubitemid 36897442)
19. Nieoullon A, Coquerel A (2003) Dopamine: a key regulator to adapt action, emotion, motivation and cognition. Curr Opin Neurol 16:S3-S9 (Pubitemid 38251045)
20. 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
21. Pietz J, Fatkenheuer B, Burgard P, Armbruster M, Esser G, Schmidt H (1997) Psychiatric disorders in adult patients with early-treated phenylketonuria. Pediatrics 99:345-350 (Pubitemid 27176002)
22. Pietz J, Dunckelmann R, Rupp A et al (1998) Neurological outcome in adult patients with early-treated phenylketonuria. Eur J Pediatr 157:824-830 (Pubitemid 28460744)
23. Pocock SJ, Simon R (1975) Sequential treatment assignment with balancing for prognostic factors in the controlled clinical trial. Biometrics 31:103-115
24. Robertson IH, Manly T, Andrade J, Baddeley BT, Yiend J (1997) 'Oops!': performance correlates of everyday attentional failures in traumatic brain injured and normal subjects. Neuropsychologia 35:747-758
25. Schmidt E, Rupp A, Burgard P, Pietz J, Weglage J, de Sonneville L (1994) Sustained attention in adult phenylketonuria: the influence of the concurrent phenylalanine-blood-level. J Clin Exp Neuropsychol 16:681-688 (Pubitemid 24331907)
26. Schmidt H, Burgard P, Pietz J, Rupp A (1996) Intelligence and professional career in young adults treated early for phenylketonuria. Eur J Pediatr 155:S97-S100
27. Schweitzer-Krantz S, Burgard P (2000) Survey of national guidelines for the treatment of phenylketonuria. Eur J Pediatr 159:S70-S73
28. Scriver R, Levy H, Donlon J (2008) Hyperphenylalaninemia: phenylalanine hydroxylase deficiency. In The Online Metabolic and Molecular Bases of Inherited Disease. Retrieved from: url: www.ommbid.com
29. Tam SY, Roth RH (1997) Mesoprefrontal dopaminergic neurons: can tyrosine availability influence their functions? Biochem Pharmacol 53:441-453
30. Van Spronsen FJ, Ahring KK, Gizewska M (2009) PKU-what is daily practice in various centres in Europe? Data from a questionnaire by the scientific advisory committee of the European Society of Phenylketonuria and Allied Disorders. J Inherit Metab Dis 32:58-64
31. Vermathen P, Robert-Tissot L, Pietz J, Lutz T, Boesch C, Kreis R (2007) Characterization of white matter alterations in phenylketonuria by magnetic resonance relaxometry and diffusion tensor imaging. Magn Reson Med 58:1145-1156 (Pubitemid 350234201)
32. Wald FDM, Mellenbergh GJ (1990) De verkorte versie van de Nederlandse vertaling van de Profile of Mood States (POMS) [The shortened version of the Dutch translation of the Profile of Mood States (POMS)]. Nederlands Tijdschr Psychol 45:86-90
33. Welsh MC (1996) A prefrontal dysfunction model of early-treated phenylketonuria. Eur J Pediatr 155:S87-S89
34. Wechsler D (1981) Manual for the Wechsler Adult Intelligence Scale - Revised. Psychological Corporation, New York