Literature DB >> 7795351

Neurological findings in early treated phenylketonuria.

J Weglage1, M Pietsch, B Fünders, H G Koch, K Ullrich.   

Abstract

Twenty early treated, normal intelligent patients (IQ: mean 101.4, SD 10.0; age: mean 10.11, SD 1.3 years) with classical phenylketonuria and 20 age-, sex- and IQ-matched healthy controls were investigated for neurological outcome, especially with regard to fine motor ability using the motor performance task ("Motorische Leistungsserie"). No pathological findings were seen on clinical neurological examination. The patient group had significantly poorer results in a concentration task (Test-d-2) as well as in some subtests of the motor performance task. Patients had difficulties in tasks which needed speed and precision of arm-hand-finger movements. High serum phenylalanine concentrations were significantly correlated with these deficiencies in fine motor ability. Our data demonstrated mild neurological impairment even in early and relatively strictly treated patients with phenylketonuria.

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Year:  1995        PMID: 7795351     DOI: 10.1111/j.1651-2227.1995.tb13661.x

Source DB:  PubMed          Journal:  Acta Paediatr        ISSN: 0803-5253            Impact factor:   2.299


  14 in total

Review 1.  Oxidative stress in phenylketonuria: what is the evidence?

Authors:  Graziela S Ribas; Angela Sitta; Moacir Wajner; Carmen R Vargas
Journal:  Cell Mol Neurobiol       Date:  2011-04-23       Impact factor: 5.046

2.  Characterization of tremor in phenylketonuric patients.

Authors:  Belén Pérez-Dueñas; Josep Valls-Solé; Emilio Fernández-Alvarez; Jaon Conill; Maria Antonio Vilaseca; Rafael Artuch; Jaume Campistol
Journal:  J Neurol       Date:  2005-07-05       Impact factor: 4.849

3.  Deficits in selective and sustained attention processes in early treated children with phenylketonuria--result of impaired frontal lobe functions?

Authors:  J Weglage; M Pietsch; B Funders; H G Koch; K Ullrich
Journal:  Eur J Pediatr       Date:  1996-03       Impact factor: 3.183

Review 4.  Human hepatocyte transplantation: current experience and future challenges.

Authors:  Anil Dhawan; Juliana Puppi; Robin D Hughes; Ragai R Mitry
Journal:  Nat Rev Gastroenterol Hepatol       Date:  2010-04-06       Impact factor: 46.802

5.  Investigation of oxidative stress parameters in treated phenylketonuric patients.

Authors:  A Sitta; A G Barschak; M Deon; T Terroso; R Pires; R Giugliani; C S Dutra-Filho; M Wajner; C R Vargas
Journal:  Metab Brain Dis       Date:  2006-12-05       Impact factor: 3.584

6.  Regression of neuropsychological deficits in early-treated phenylketonurics during adolescence.

Authors:  J Weglage; M Pietsch; J Denecke; A Sprinz; R Feldmann; M Grenzebach; K Ullrich
Journal:  J Inherit Metab Dis       Date:  1999-08       Impact factor: 4.982

7.  Untreated non-phenylketonuric-hyperphenylalaninaemia: intellectual and neurological outcome.

Authors:  J Weglage; K Ullrich; M Pietsch; B Fünders; R Zass; H G Koch
Journal:  Eur J Pediatr       Date:  1996-07       Impact factor: 3.183

8.  Motor development skills of 1- to 4-year-old Iranian children with early treated phenylketonuria.

Authors:  Sepideh Nazi; Farzaneh Rohani; Firoozeh Sajedi; Akbar Biglarian; Arya Setoodeh
Journal:  JIMD Rep       Date:  2013-08-06

9.  Alanine prevents the reduction of pyruvate kinase activity in brain cortex of rats subjected to chemically induced hyperphenylalaninemia.

Authors:  Luciane Rosa Feksa; Andrea Renata Cornelio; Virginia Cielo Rech; Carlos Severo Dutra-Filho; Angela Terezinha Souza Wyse; Moacir Wajner; Clóvis Milton Duval Wannmacher
Journal:  Neurochem Res       Date:  2002-09       Impact factor: 3.996

10.  Chemical biomarkers of epileptogenesis and ictogenesis in experimental epilepsy.

Authors:  Hiram Luna-Munguia; Alexander G Zestos; Stephen V Gliske; Robert T Kennedy; William C Stacey
Journal:  Neurobiol Dis       Date:  2018-10-07       Impact factor: 5.996

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