Neuropsychological and Neuroradiological (MRI) Variations During Phenylalanine Load: Protective Effect of Valine,Leucine,and Isoleucine Supplementation

Abstract

Get full access to this article

View all access options for this article.

References

Pearsen KD , Gean-Marton AD , Levy HL , Davis KR : Phenylketonuria: MR imaging of the brain with clinical correlation . Radiology 1990;177:437-440.

Bick U. , Fahrendorf G. , Ludolph AC , et al: Disturbed myelination in patients with treated hyperphenylalaninaemias: Evaluation with magnetic resonance imaging. Eur J Pediatr 1991;150:185-189.

Shaw DWW , Maravilla KR , Weinberger E. , et al: MR imaging ofphenylketonuria. AJNR Am J Neuroradiol 1991;12:403-406.

Thompson AJ , Smith I. , Kendall BE , et al: Magnetic resonance imaging changes in early treated patients with phenylketonuria, letter. Lancet 1991;337:1224.

Leuzzi V. , Gualdi GF , Trasimeni G. , et al: Neuroradiological (MRI) abnormalities in phenylketonuric subjects: Clinical and biochemical correlations. Neuropediatrics 1993;24:302-306.

Thompson AJ , Tillotson S. , Smith I. , et al: Brain MRI changes in phenylketonuria. Association with dietary status. Brain 1993;116:811-821.

Cleary MA , Walter JH , Wraith JE , et al: Magnetic resonance imaging of the brain in phenylketonuria. Lancet 1994;344:87-90.

Berry HK , Brunner RL , Hunt M. , White PP : Valine, isoleucine, and leucine. A new treatment for phenylketonuria . Am J Dis Child 1990;144:539-543.

Battistini S. , De Stefano N. , Parlanti S. , Federico A. : Unexpected white matter changes in an early treated PKU case and improvement after dietary treatment. Funct Neurol 1991;6:177-180.

10.

Thompson AJ , Smith I. , Brenton D. , et al: Neurological deterioration in young adults with phenylketonuria. Lancet 1990;336:602-605.

11.

Bick U. , Ullrich K. , Stöber U. , et al: White matter abnormalities in patients with treated hyperphenylalaninaemia: Magnetic resonance relaxometry and proton spectroscopy findings. Eur J Pediatr 1993; 152:1012-1020.

12.

Brunner RL , Jordan MK , Berry HK : Early-treated phenylketonuria: Neuropsychologic consequences. Pediatrics 1983;102:831-835.

13.

Lou HC , Güttler F. , Lykkelund C. , et al: Decreased vigilance and neurotransmitter synthesis after discontinuation of dietary treatment for phenylketonuria in adolescents. Eur J Pediatr 1985;144:17-20.

14.

Krause W. , Halminski M. , McDonald L. , et al: Biochemical and neuropsychological effects of elevated plasma phenylalanine in patients with treated phenylketonuria. A model for the study of phenylalanine and brain function in man. J Clin Invest 1985;75:40-48.

15.

Brunner RL , Berch DB , Berry H. : Phenylketonuria and complex spatial visualization: An analysis of information processing. Dev Med Child Neurol 1987;29:460-468.

16.

Butler IJ , O'Flynn ME , Seifert WE Jr , Howell RR : Neurotransmitter defects and treatment of disorders of hyperphenylalaninemia . J Pediatr 1981;98:729-733.

17.

Andersen AE , Avins L. : Lowering brain phenylalanine levels by giving other large neutral amino acids. Arch Neurol 1976;33:684-686.

18.

Brunner RL , Vorhees CV , McLean MS , et al: Benefical effect of isoleucine on fetal brain development in induced phenylketonuria. Brain Res 1978;154:191-195.

19.

Berry HK , Bofinger MK , Hunt MM , et al: Reduction of cerebrospinal fluid phenylalanine after oral administration of valine, isoleucine, and leucine. Brain Res 1982;16:751-755.

20.

Jordan MK , Brunner RL , Hunt MM , Berry HK : Preliminary support for the oral administration of valine, isoleucine and leucine for phenylketonuria. Dev Med Child Neurol 1985;27:33-39.

21.

Lezak MD : Neuropsychological Assessment. New York, Oxford University Press, 1983, pp 447-451.

22.

Plaitakis A. , Smith J. , Mandeli J. , Yahr MD : Pilot trial of branched-chain amino acids in amyotrophic lateral sclerosis. Lancet 1988 ;May 7, 1(8593):1015-1018.