Journal article
MR imaging-based volumetry in patients with early-treated phenylketonuria
American journal of neuroradiology : AJNR, Vol.26(7), pp.1681-1685
2005
PMID: 16091513
Abstract
Background and purpose: Our purpose was to specify the most severely affected brain structures in early treated phenylketonuria regarding volume loss and establish possible correlations between volume loss and plasma levels of phenylalanine (Phe).
Methods: In 31 patients with early treated phenylketonuria and in 27 healthy volunteers, we acquired volumetric MR imaging data. Serum Phe concentrations at different times were measured as well. Semiautomatic volumetric postprocessing of the cerebellum, cerebrum (supratentorial brain tissue), hippocampus, intracranial volume, lateral ventricles, nucleus caudatus, nucleus lentiformis, pons, and thalamus, as well as the two-dimensional extension of the corpus callosum, was performed using the software BRAINS2. For each separate brain structure, the relative differences between the normal and the phenylketonuria group (delta(rel)) were calculated.
Results: The cerebrum, corpus callosum, hippocampus, intracranial volume, and pons were significantly smaller in patients with phenylketonuria than in healthy patients. The volume of the lateral ventricles was significantly larger in patients with phenylketonuria than in healthy ones. The most severely affected structures were the pons (delta(rel) = 16%), hippocampus (delta(rel) = 14.5%), cerebrum (delta(rel) = 13%), and corpus callosum (delta(rel) = 10%). No significant differences were found for the basal ganglia, cerebellum, and thalamus. There were no significant correlations found between the volume of any of the different brain structures and the metabolic parameters.
Conclusion: The most severely affected brain structures in early-treated patients with phenylketonuria regarding volume loss are the cerebrum, corpus callosum, hippocampus, and pons.
Details
- Title: Subtitle
- MR imaging-based volumetry in patients with early-treated phenylketonuria
- Creators
- Nadine H PFAENDNER - Division of Neuroradiology, Department of Neurology, University of Heidelberg Medical Center, Heidelberg, GermanyGitta REUNER - Department of Pediatric Neurology, University of Heidelberg Medical Center, Heidelberg, GermanyJoachim PIETZ - Department of Pediatric Neurology, University of Heidelberg Medical Center, Heidelberg, GermanyGregor JOST - Division of Neuroradiology, Department of Neurology, University of Heidelberg Medical Center, Heidelberg, GermanyDietz RATING - Department of Pediatric Neurology, University of Heidelberg Medical Center, Heidelberg, GermanyVincent A MAGNOTTA - Department of Psychiatry, Iowa Mental Health Clinical Research Center, Univer sity of Iowa, Iowa City, IA, United StatesAlexander MOHR - Division of Neuroradiology, Department of Neurology, University of Heidelberg Medical Center, Heidelberg, GermanyBodo KRESS - Division of Neuroradiology, Department of Neurology, University of Heidelberg Medical Center, Heidelberg, GermanyKlaus SARTOR - Division of Neuroradiology, Department of Neurology, University of Heidelberg Medical Center, Heidelberg, GermanyStefan HÄHNEL - Division of Neuroradiology, Department of Neurology, University of Heidelberg Medical Center, Heidelberg, Germany
- Resource Type
- Journal article
- Publication Details
- American journal of neuroradiology : AJNR, Vol.26(7), pp.1681-1685
- Publisher
- American Society of Neuroradiology; Oak Brook, IL
- PMID
- 16091513
- ISSN
- 0195-6108
- eISSN
- 1936-959X
- Language
- English
- Date published
- 2005
- Academic Unit
- Roy J. Carver Department of Biomedical Engineering; Radiology; Psychiatry; Iowa Neuroscience Institute
- Record Identifier
- 9984051893102771
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