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  • 1
    Electronic Resource
    Electronic Resource
    Temporäre Amaurose nach Kraniopharyngeomoperation (1998)
    Springer
    Monatsschrift Kinderheilkunde 146 (1998), S. 1067-1069 
    Details
    ISSN: 1433-0474
    Keywords: Schlüsselwörter Kraniopharyngeom ; Amaurose ; Chiasma ; Hormone ; Operation ; Key words Kraniopharyngioma ; Blindness ; Chiasma ; Endocrine dysfunction ; Surgery
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Craniopharyngiomas are the third frequent pediatric brain tumors. Complications after surgical treatment are common. The most frequent complications are endocrine disturbances, vision field defects, deteriorated acuity, cognitive or psychological deficiency as well as significant weight gain. A case of complete vision loss right after surgical removal of craniopharyngioma is reported. Magnetic resonance image showed an edema of the chiasm. No damage of anatomical structure could be detected. Seventeen days after surgery the patient reported intermittent visual impressions for the first time. Over the following weeks his visus improved to 0.5, while the chiasm edema disappeared. Discussion: Even weeks after surgery-induced blindness regeneration of sufficient visus is possible.
    Notes: Zusammenfassung Das Kraniopharyngeom ist der dritthäufigste Hirntumor des Kindesalters. Komplikationen nach Kraniopharyngeomoperationen sind häufig. Zu den häufigsten Komplikationen zählen endokrine Störungen, Visusverschlechterung, Gesichtsfeldausfälle, emotionale und kognitive Veränderungen sowie Gewichtszunahme. Wir berichten über 1 Patientin, bei der unmittelbar nach der Operation eines Kraniopharyngeoms eine vollständige Amaurose auftrat. Bei intakten anatomischen Strukturen zeigte das MRT eine Ödem im Bereich des Chiasmas. 17 Tage nach der Operation traten erstmals wieder Seheindrücke auf, die sich im Verlauf von 6 Wochen bis zu einem Visus von 0,5 steigerten. Parallel dazu wies die Bildgebung einen Rückgang des Chiasmaödems auf. Diskussion: Auch Wochen nach einer eingetretenen operationsbedingten Amaurose kann bei intakten anatomischen Strukturen mit der Wiedererlangung eines ausreichenden Visus gerechnet werden.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s001120050366
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  • 2
    Electronic Resource
    Electronic Resource
    Effect of l-dopa on visual evoked potentials and neuropsychological tests in adult phenylketonuria patients (1996)
    Springer
    European journal of pediatrics 155 (1996), S. S74 
    Details
    ISSN: 1432-1076
    Keywords: Key words Phenylketonuria ; l-dopa therapy ; Neuropsychological tests
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Eight adult, untreated patients with classical phenylketonuria received L-dopa and a decarboxylase inhibitor for 2 weeks. No effect of l-dopa therapy on choice reaction time tasks, sustained attention, frontal lobal function as well as latencies of visual evoked potentials was found. The results raise the question if adult patients with phenylketonuria really suffer from functional dopamine deficiency.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/PL00014256
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  • 3
    Electronic Resource
    Electronic Resource
    Disturbed myelination in patients with treated hyperphenylalaninaemia: evaluation with magnetic resonance imaging (1991)
    Springer
    European journal of pediatrics 150 (1991), S. 185-189 
    Details
    ISSN: 1432-1076
    Keywords: Phenylketonuria ; Adolescents ; Magnetic resonance imaging ; Myelination
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Cranial magnetic resonance imaging (MRI) was performed in nine treated adolescents with hyperphenylalaninaemia (HPA) in order to analyse possible changes in myelination. Three patients suffered from type I HPA, four from type II and two from type III (persistent HPA). Images were obtained with a 1.5T unit using spin-echo-sequences. In all patients with type I or type II HPA, abnormal findings in the cerebral white matter were demonstrated including band-like and/or confluent patchy areas of high signal intensity predominantly in the peritrigonal region, with anterior and posterior periventricular extension and/or involvement of the subcortical white matter. The extent of MRI changes did not correlate with the initiation, duration or quality of dietary treatment. There was also no consistent relationship between electrophysiological changes and white matter abnormalities on MRI. Our findings suggest a disturbance of myelination in patients with treated HPA. These results correspond well with earlier neuropathological and biochemical studies in untreated patients.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01963563
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  • 4
    Electronic Resource
    Electronic Resource
    White matter abnormalities in patients with treated hyperphenylalaninaemia: Magnetic resonance relaxometry and proton spectroscopy findings (1993)
    Springer
    European journal of pediatrics 152 (1993), S. 1012-1020 
    Details
    ISSN: 1432-1076
    Keywords: Phenylketonuria ; Myelination ; Magnetic resonance imaging ; 1H spectroscopy ; T2 relaxometry
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In order to further clarify the pathogenesis and clinical significance of MRI white matter abnormalities in treated hyperphenylalaninaemia (HPA), ten patients (seven type I HPA, two type II and one type III) underwent T2 relaxometry (n=8) and/or1H spectroscopy (n=7) in addition to conventional MR spin-echo imaging at 1.5 T. Two patients with severe MRI abnormalities had repeat examinations during and after a 6-to 8-month period of strict diet control. The clinical evaluation included a detailed neurological examination. In nine out of ten patients visual evoked potentials (VEP) were obtained parallel to the MR examination. MR imaging demonstrated typical symmetrical areas of prolonged T2 relaxation time predominantly in the posterior periventricular white matter in all but one of type I and II patients. There was no consistent relationship between MRI findings and time of diagnosis/initiation of therapy, IQ or visual evoked potential changes. MRI abnormalities tended to be more severe in patients with poor dietary control and high current plasma phenylalanine levels, whereas a normal MRI was found only in patients with plasma phenylalanine levels continuously below 0.36 mmol/l. There was marked regression of MRI abnormalities already after 3 months of strict diet control. T2 relaxometry showed a bi-exponential behaviour of T2 in the affected white matter, with a slow component of about 200–450 ms, indicating an increase in free (extracellular) water.1H spectroscopy revealed no signs of severe neuronal damage. We conclude, that the observed white matter changes in treated HPA probably represent reversible structural myelin changes rather than permanent demyelination.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01957228
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  • 5
    Electronic Resource
    Electronic Resource
    Deficits in selective and sustained attention processes in early treated children with phenylketonuria — result of impaired frontal lobe functions? (1996)
    Springer
    European journal of pediatrics 155 (1996), S. 200-204 
    Details
    ISSN: 1432-1076
    Keywords: Phenylketonuria ; Selective ; sustained attention ; Impaired frontal lobe functions
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Twenty normally intelligent children with early treated phenylketonuria (PKU) (IQ: mean=101.4, SD=10.0; age: mean=10 years 11 months, SD=1.3 years) and 20 healthy controls, matched for age, sex and IQ, were assessed for their selective (Stroop Task) and sustained attention (Test-d-2). Using positron emission tomography an activation of the frontal lobe during the Stroop task had previously been demonstrated. In addition to the Stroop Task and the Test-d-2, a short-term memory test as a “non-frontal-lobe-function-task” was administered to all subjects. Group comparisons demonstrated that PKU children had specific deficits in selective and sustained attention, which were significantly correlated with the concurrent serum phenylalanine concentration. Conclusion The results give evidence that even dietary treated children with PKU were suffering from impaired attentional control mechanisms in spite of a normal IQ. The deficits might be the result of impaired frontal lobe functions.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01953938
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    Paper (German National Licenses)
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