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  Neurocognitive functioning in adults with phenylketonuria: Results of a long term study

Weglage, J., Fromm, J., van Teeffelen-Heithoff, A., Möller, H. E., Koletzko, B., Marquardt, T., et al. (2013). Neurocognitive functioning in adults with phenylketonuria: Results of a long term study. Molecular Genetics and Metabolism, 110(Suppl.), S44-S48. doi:10.1016/j.ymgme.2013.08.013.

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Item Permalink: http://hdl.handle.net/11858/00-001M-0000-0014-6898-4 Version Permalink: http://hdl.handle.net/21.11116/0000-0003-9E8B-F
Genre: Journal Article

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 Creators:
Weglage, J.1, Author
Fromm, J.2, Author
van Teeffelen-Heithoff, A.1, Author
Möller, Harald E.3, Author              
Koletzko, B.4, Author
Marquardt, T.1, Author
Rutsch, F.1, Author
Feldmann, R.1, Author
Affiliations:
1University of Münster, Department of Pediatrics, Albert-Schweitzer-Campus, Münster, Germany, ou_persistent22              
2University of Münster, Department of Clinical Radiology, Albert-Schweitzer-Campus, Münster, Germany, ou_persistent22              
3Methods and Development Unit Nuclear Magnetic Resonance, MPI for Human Cognitive and Brain Sciences, Max Planck Society, ou_634558              
4University of Munich, Department of Pediatrics, Munich, Germany, ou_persistent22              

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Free keywords: IQ; Attention; Information processing; White matter alterations; Imaging; Adolescence; PKU
 Abstract: Objectives A controlled long-term study was performed to assess the neurological and neuropsychological performance in adult patients with early-treated phenylketonuria (PKU). Methods We investigated 57 patients with early-treated classical PKU aged 19 to 41 years (mean age 31 years) and 46 matched healthy controls, matched for age and socioeconomic status. Patients and controls were assessed for their intelligence quotient (IQ), and attention and information-processing abilities. Magnetic resonance imaging (MRI) of the brain was performed in all patients. Neuropsychological assessments and MRI were repeated at a five-year-follow-up. Results In the five-year interval IQ, information processing and attention of patients and controls remained constant. At both assessment times the IQ scores were significantly lower in patients compared to controls. Older adult patients (> 32 years) showed poorer information processing and attention at both assessment times compared to young adult patients (< 32 years) and controls. IQ, information processing and attention showed no correlation to imaging results but were significantly correlated to blood phenylalanine (Phe) levels in patients' childhood and adolescence, and Phe levels had been higher in the adolescent years of older adult patients. Conclusions Cognitive performance in adult patients with early-treated PKU does not seem to be subject to deterioration observable in a five-year interval. Neuropsychological assessment in adults with PKU revealed neurocognitive impairment particularly in older adult patients. This seems to refer to an early relaxation of diet that was recommended when the older patients were adolescents. Results indicate a benefit of dietary control during adolescence in PKU.

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Language(s): eng - English
 Dates: 2013-08-262013-08-122013-08-262013-08-312013
 Publication Status: Published in print
 Pages: -
 Publishing info: -
 Table of Contents: -
 Rev. Method: Peer
 Identifiers: DOI: 10.1016/j.ymgme.2013.08.013
PMID: 24071437
Other: Epub 2013
 Degree: -

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Title: Molecular Genetics and Metabolism
Source Genre: Journal
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Publ. Info: Orlando, FL : Academic Press
Pages: - Volume / Issue: 110 (Suppl.) Sequence Number: - Start / End Page: S44 - S48 Identifier: ISSN: 1096-7192
CoNE: /journals/resource/954922651185