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  • 1
    Electronic Resource
    Electronic Resource
    Local vascular CO2 reactivity in the infant brain assessed by functional MRI (1995)
    Springer
    Pediatric radiology 25 (1995), S. 420-424 
    Details
    ISSN: 1432-1998
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The local cerebral vascular response to hyperventilation vas investigated in five distressed, intubated infants by means of a T2* sensitive gradient-echo MRI technique at 1.5 T. In one preterm infant, the MR signal change during hyperventilation was sparse. In four term infants, the mean MR signal of the brain slice investigated decreased by 1.2–2.6% per kPa change inPCO2 as a reflection of decreased cerebral blood flow during hyperventilation. Pixel-wise analysis revealed absence of vascular response in the basal ganglia, the thalamus or in the occipital region. In two adult controls, who hyperventilated voluntarily, the vascular reactivity was homogeneously distributed predominantly over the grey matter. The experiments demonstrate that local impairment of vascular CO2 reactivity in the distressed infant brain can be detected by T2* sensitive gradient-echo MRI, which is also known as functional MRI.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02019053
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    Paper (German National Licenses)
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  • 2
    Electronic Resource
    Electronic Resource
    Magnetic resonance imaging in juvenile Canavan disease (1993)
    Springer
    European journal of pediatrics 152 (1993), S. 750-753 
    Details
    ISSN: 1432-1076
    Keywords: Magnetic resonance imaging ; Spectroscopy ; Imaging, Canavan disease ; Brain
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We present a 2-year-old boy and a 6-year-old girl with mild Canavan disease (CD). Aspartoacylase activity in skin fibroblasts was deficient. Magnetic resonance imaging (MRI) of the brain did not show the prominent leucodystrophy previously reported in CD, but there was a hyperintense signal from the lentiform nuclei and the heads of the caudate nuclei on the T2-weighted MR images. This suggests a specific vulnerability of the corpus striatum in these patients. In the older patient, the white matter became affected at the age of 6 years. Proton magnetic resonance spectroscopy (1H-MRS) of white matter revealed a normal concentration of N-acetyl-l-aspartate (NAA) and a markedly decreased concentration of choline containing compounds (Cho) in the boy but a normal ratio of NAA to Cho in the girl. We conclude that deficient NAA catabolism affects myelin metabolism. This may present as changes in the striatum and/or as a low concentration of Cho before leucodystrophy appears on MRI.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01953994
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    Paper (German National Licenses)
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  • 3
    Electronic Resource
    Electronic Resource
    Brain magnetic resonance imaging in children with optimally controlled hyperphenylalaninaemia (1994)
    Springer
    Journal of inherited metabolic disease 17 (1994), S. 575-583 
    Details
    ISSN: 1573-2665
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary This study was undertaken to investigate whether the white-matter changes on MRI and the EEG abnormalities detectable in treated adolescents and adults with hyperphenylalaninaemia (HPA) can be detected in younger children on an optimally controlled diet. The study included 17 children, 7–12 years of age, with HPA. The MRI of five healthy children were included in the blind evaluation of the MR images. According to mutation genotype and dietary tolerance of phenylalanine, 9 patients have severe HPA and 8 have moderate HPA, all requiring dietary treatment. Mild white-matter hyperintensity was detected in 1 of the 5 healthy children and in 10 of 17 patients. EEG was abnormal in 2 patients. This group of children was compared with a previously reported group of adolescents with HPA who had been treated according to the same dietary regimen. MRI changes and EEG abnormalities were significantly less frequent in the group of children than in the group of adolescents. It is suggested that the more frequent MRI changes and EEG abnormalities seen in adolescents are related to the fact that a relaxation of the dietary treatment after the age of 8 years is often accepted.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00711594
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  • 4
    Electronic Resource
    Electronic Resource
    An occipito-temporal syndrome in adolescents with optimally controlled hyperphenylalaninaemia (1992)
    Springer
    Journal of inherited metabolic disease 15 (1992), S. 687-695 
    Details
    ISSN: 1573-2665
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The study included 16 adolescents with optimally controlled hyperphenylalaninaemia (McKusick 26160), of whom six did not require treatment according to conventional criteria. All except the two patients with lowest median serum phenylalanine level throughout childhood (most values at 200–300 µmol/L) had white matter abnormalities detectable with magnetic resonance imaging. The lesions were particularly prominent in the watershed regions between the posterior and middle cerebral arteries. In most patients with moderate or severe hyperphenylalaninaemia frontal white matter lesions were present as well. Normal proton magnetic resonance spectra indicated that the lesions were stable. Occipital EEG abnormalities were frequent, and deficient performance on a pattern-recognition test was a characteristic neuropsychological finding. Serum phenylalanine levels at about 300 µmol/L or below throughout childhood and early adolescence may be required to avoid lesions. The present study demonstrates the limitations of even an optimally controlled dietary regimen in hyperphenylalaninaemia.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01800008
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  • 5
    Electronic Resource
    Electronic Resource
    Blood-brain barrier transport of amino acids in healthy controls and in patients with phenylketonuria (1995)
    Springer
    Journal of inherited metabolic disease 18 (1995), S. 653-664 
    Details
    ISSN: 1573-2665
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Blood-brain barrier permeability to phenylalanine and leucine in four patients with phenylketonuria and in four volunteers was measured five times by the double-indicator method at increasing plasma concentrations of phenylalanine. Based on the permeability-surface area product (PS) from blood to brain (PS1) and on plasma phenylalanine levels, Vmax and the apparentK m for phenylalanine were determined. Statistically significant relationships between plasma phenylalanine and PS1 were established in three out of four volunteers, the averageV max value being 46.7 nmol/g per min and the apparentK m 0.328 mmol/L. Owing to saturation of the carrier, such a relationship could not be established in the patients. In phenylketonuria, PS1 for phenylalanine and leucine decreased significantly by 55% and 46%, respectively. Transport from brain back to blood, PS2, decreased significantly and cerebral large neutral amino acid net uptake was generally decreased in patients with phenylketonuria. In conclusion, the transport ofl-phenylalanine across the human blood-brain barrier follows Michaelis-Menten kinetics. In phenylketonuria, brain permeability to large neutral amino acids is reduced by about 50% and net uptake appears decreased.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02436753
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    Paper (German National Licenses)
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