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  • 1
    Electronic Resource
    Electronic Resource
    Lissencephaly syndromes: clinical aspects (1993)
    Springer
    Child's nervous system 9 (1993), S. 380-386 
    Details
    ISSN: 1433-0350
    Keywords: Type I lissencephaly ; Type II lissencephaly ; Walker- ; Warburg syndrome ; EEG
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report clinical and neurophysiological findings in six children (three female, three male) with type I lissencephaly and three children (all female) with type II lissencephaly (Walker-Warburg syndrome). In type I lissencephaly the diagnosis is based only on electroencephalographic (EEG) signs, whereas in type II lissencephaly the diagnosis rests on clinical signs. In type I lissencephaly the EEG typically shows high α-β activity, which is not seen in type II lissencephaly.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00306188
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  • 2
    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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  • 3
    Electronic Resource
    Electronic Resource
    Psychological and social findings in adolescents with phenylketonuria (1992)
    Springer
    European journal of pediatrics 151 (1992), S. 522-525 
    Details
    ISSN: 1432-1076
    Keywords: Adolescent phenylketonuria ; Psychological characteristics ; Social findings ; Dietary control
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In a retrospective study, 34 early treated, normally intelligent adolescents with phenylketonuria (PKU) and their parents were tested with several psychometric personality inventories and self-developed questionaires concerning their psychosocial situation and their disease-and diet-specific knowledge. Results show that the patients are characterized by less autonomy, a more negative evaluation of their scholastic ability, less achievement motivation, less extraversion and impulsiveness, a feeling of not being quite healthy, more grave and a higher level of dependency from their families. The patients saw their whole social situation as being destinctly restricted. Their knowledge concerning disease and diet was alarmingly poor and the majority had great difficulties in managing the diet satisfactorily without parental help. Up to the age of 15 years the serum phenylalanine levels were persistently above the desired range.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01957759
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  • 4
    Electronic Resource
    Electronic Resource
    Growth in patients with phenylketonuria (1994)
    Springer
    European journal of pediatrics 153 (1994), S. 537-538 
    Details
    ISSN: 1432-1076
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01957014
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  • 5
    Electronic Resource
    Electronic Resource
    No white matter abnormalities in untreated patients with persistent hyperphenylalaninaemia: Findings in magnetic resonance imaging (1994)
    Springer
    European journal of pediatrics 153 (1994), S. 918-919 
    Details
    ISSN: 1432-1076
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01954748
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  • 6
    Electronic Resource
    Electronic Resource
    Psychosocial aspects in phenylketonuria (1996)
    Springer
    European journal of pediatrics 155 (1996), S. S101 
    Details
    ISSN: 1432-1076
    Keywords: Key words Phenylketonuria ; Psychological characteristics ; Social ; findings
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Psychosocial aspects in phenylketonuric (PKU) patients are reported. In two separate studies patients with PKU differing in age (children versus adolescents), were assessed. The main message of the first prospective study on 58 10-year-old patients is that normally intelligent PKU patients who were treated early and strictly did not show a higher risk for severe emotional and behavioural maladjustment compared with healthy controls at the age of 10 years. The data were obtained in the course of the German PKU Collaborative Study by the “Personality Questionnaire for Children (PFK 9–14)”. All patients received nutritional, medical, and psychological counselling every 6 months. In the second retrospective study, 34 early treated, normally intelligent adolescents with PKU (age: mean = 14.6, SD = 2.0, range = 11–18 years) and their mothers were assessed with several psychometric personality inventories and self-developed questionnaires concerning their psychosocial situation and their disease- and diet-specific knowledge. Using the Mannheimer Biographic Inventory (MBI), the Personality Questionnaire for Children (PFK 9–14), and the Freiburger Personality Inventory (FPI) the adolescent patients described their social life and their emotional development as being distinctly restricted. Their knowledge concerning disease and diet was alarmingly poor and the majority had great difficulties in satisfactory dietetic management without parental help. In addition to the burdensome diet, developmental crises like puberty may cause more frequently emotional and behavioural problems in PKU patients.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/PL00014225
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  • 7
    Electronic Resource
    Electronic Resource
    Untreated non-Phenyketonuric-hyperphenylalaninaemia: intellectual and neurological outcome (1996)
    Springer
    European journal of pediatrics 155 (1996), S. S26 
    Details
    ISSN: 1432-1076
    Keywords: Key words Phenylketonuria ; Non-PKU HPA ; intellectual and neurological outcome
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The intellectual, neurological, and neuropsychological outcome of patients with non-phenylketonuric-hyperphenylalaninaemia (PKU-HPA) (serum phenylalanine levels under free diet 〈 600 μmol/l) has not been systematically studied so far. We therefore tested 28 patients (mean age = 21.8, SD = 4.2 years) for IQ (WAIS-R/WISC-R), school performance, job career, clinical neurological examination, fine motor performance (motor performance task), and selective and sustained attention (stroop task, Dot Pattern Exercise from the Sonneville visual attention task). In addition, cranial MRI (1.5 T unit) was obtained in 10 of these patients. Clinical-neurological examination revealed no significant abnormalities in the non-PKU-HPA patients. They also had a normal IQ (mean = 101.9, SD = 13.6). Compared to their healthy siblings, they attended a normal school and had a normal job career. The motor performance task revealed no deficits in fine motor abilities. The patients performed normally in the stroop task and the dot pattern exercise. Their MRIs were normal. Our results indicate that patients with non-PKU-HPA are not at risk for developing intellectual, neurological, and neuropsychological impairment, as described for patients with treated mild or classical phenylketonuria. From this point of view a dietary treatment is not necessary in patients with hyperphenylalaninaemia.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/PL00014244
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  • 8
    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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  • 9
    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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  • 10
    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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