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  • 1
    Electronic Resource
    Electronic Resource
    Long-term development of intelligence (IQ) and EEG in 34 children with phenylketonuria treated early (1988)
    Springer
    European journal of pediatrics 147 (1988), S. 361-367 
    Details
    ISSN: 1432-1076
    Keywords: CNS development ; Dietary treatment ; EEG ; IQ ; Phenylketonuria
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In 34 children with phenylketonuria (PKU) treated early the prognostic value of the age on institution of the diet (within the first 3 months of life) and of the quality of dietary treatment was determined in two different ways: 1) following intelligence closely (IQ) and (2) evaluating the EEG development up to their 12th (n=34) and 15th (n=18) years of life as appropriate. In general, IQ scores were found to be normal from the 4th–15th years of life. In our group of patients there was no effect on the IQ of the timing of diet onset. Children with “strict” dietary control showed a significantly higher IQ than those with “loose” control. One hundred and fifty-four EEGs (10/20 system, awake with eyes closed) were recorded at intervals of 2 years and conventionally evaluated. The development of alpha-activity was found to be normal. Beta-activity was enhanced. Abnormal EEG findings like general slowing and generalized paroxysmal activity (GPA) with or without spikes were more frequent in children with PKU than in controls, with the exception of focal abnormalities. EEG abnormalities increased with advancing age independently of IQ development and showed no relation to either the age at the onset nor the quality of dietary treatment.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00496411
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  • 2
    Electronic Resource
    Electronic Resource
    Muscle adenylate kinase in Duchenne muscular dystrophy
    Amsterdam : Elsevier
    Biochimica et Biophysica Acta (BBA)/General Subjects 883 (1986), S. 598-603 
    Details
    ISSN: 0304-4165
    Keywords: (Muscle) ; Adenylate kinase ; Duchenne muscular dystrophy
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Biology , Chemistry and Pharmacology , Medicine , Physics
    Type of Medium: Electronic Resource
    URL: http://linkinghub.elsevier.com/retrieve/pii/0304-4165(86)90303-X
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  • 3
    Electronic Resource
    Electronic Resource
    Comparison of electronically calculated and graphically determined values of the acid-base status
    Amsterdam : Elsevier
    Clinica Chimica Acta 16 (1967), S. 297-303 
    Details
    ISSN: 0009-8981
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://linkinghub.elsevier.com/retrieve/pii/0009-8981(67)90195-7
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  • 4
    Electronic Resource
    Electronic Resource
    Risk factors for latex allergy in patients with spina bifida (1996)
    Oxford, UK : Blackwell Publishing Ltd
    Clinical & experimental allergy 26 (1996), S. 0 
    Details
    ISSN: 1365-2222
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Objective and methods In order to study risk factors for latex allergy in patients with spina bifida, we investigated 165 patients with spina bifida (mean age 9 years). Besides answering a questionnaire, patients underwent skin-prick testing and determination of specific serum IgE to latex as well as a screening test for specific IgE to environmental allergens. A total of 80 patients(49%) were sensitized to latex according to the presence of specific IgE to latex.Results Skin-prick tests (SPT) with high ammonia latex milk were performed in 81 of our patients with spina bifida and were positive in 36 patients (46%). Concordance of SPT with specific IgE in serum was good. Nineteen out of 165 patients suffered from a clinically relevant latex allergy: five patients had a history of systemic reactions to latex (e.g. severe bronchospasm, anaphylactic reactions), mostly during surgery. Fourteen patients reported clinical symptoms while inflating a balloon; all these 19 patients were sensitized to latex. Number of operations ranged from one to 26 (mean 5 operations). Concentration of specific IgE to latex in serum correlated well with increasing numbers of operations. Some 32/76 patients (41%) with spina bifida who were sensitized to latex showed an atopic disposition, while 21 out of 81 latex-negative patients (26%) were atopic. Of 300 consecutive sera (mean age of patients 9 years) sent to our laboratory for routine determination of specific IgE, 144 (48%) were positive in terms of specific IgE to environmental allergens, of which 247144 (17%) were sensitized to latex.Conclusions From our data we conclude that in order to minimize risk of severe systemic clinical reactions, all patients with spina bifida should be screened for their individual risk of latex allergy to plan preventive measures before operations. Main risk factors for latex allergy seem to be: more than five operations, atopic predisposition, history of clinical symptoms while inflating a balloon, and a sensitization with a CAP-class of ± 4.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-2222.1996.tb00629.x
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  • 5
    Electronic Resource
    Electronic Resource
    Clinical, radiological and histological findings in desmoplastic infantile ganglioglioma (1994)
    Springer
    Child's nervous system 10 (1994), S. 458-463 
    Details
    ISSN: 1433-0350
    Keywords: Desmoplastic infantile ganglioglioma ; Magnetic resonance imaging ; Immunocytochemistry
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The clinical course and radiological and histological findings in a 30-month-old boy suffering from desmoplastic infantile ganglioglioma are reported. The child's development was normal until a series of complex partial seizures occurred at the age of 7 months. Cranial computed tomography and magnetic resonance imaging revealed a cystic mass with intensive ring-shaped contrast enhancement in the right temporal fossa without shift of intracranial structures. Histologically, the firm, grayish tumor showed an enormous amount of connective tissue, cystic areas, and some mitoses. Glial and neuronal cell lines were identified by immunocytochemical methods. Eighteen months after surgery the boy had developed well without any neurological dysfunction; no radiation or chemotherapy was given. For the first time a synopsis of radiological findings in this rare brain tumor is correlated with the results of multiple histological and immunocytochemical studies. Despite some malignant characteristics, the prognosis of this dysontogenetic brain tumor is good.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00303613
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  • 6
    Electronic Resource
    Electronic Resource
    Verlaufsformen kindlicher Epilepsien mit Spike wave-Absencen (1965)
    Springer
    European archives of psychiatry and clinical neuroscience 207 (1965), S. 394-415 
    Details
    ISSN: 1433-8491
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Zusammenfassung Anhand von klinischen und elektrencephalographischen Verlaufs beobachtungen bei 149 Kindern, die zu irgendeinem Zeitpunkt ihres epileptischen Leidens Absencen boten, wird die Nosologie der durch diese Anfallsform ausgestalteten Epilepsien untersucht. Es werden folgende Verlaufsformen unterschieden: 1. Frühinfantile Petit mal-Grand mal-Epilepsien. Sie sind den anderen frühkindlichen centrencephalen Epilepsien, dem myoklonischastatischen Petit mal und dem Grand mal centrencephalen Charakters zuzuordnen. 2. Spätinfantile Absence-Epilepsien (Pyknolepsie) und Absence-Grand mal-Epilepsien. Über die Häufigkeit einer sekundären Grand mal-Komplikation der Pyknolepsie kann dabei keine Aussage gemacht werden, da die Krankheitsverläufe in dieser Gruppe durch eine früh einsetzende, regelmäßige, oft eine Grand mal-Prophylaxe einschließende Therapie beeinflußt sind. In diesem Punkt ist ein Vergleich unserer Ergebnisse mit denen anderer Autoren nicht möglich. 3. Juvenile Absence- und Absence-Grand mal-Epilepsien. Die verschiedenen Verlaufsformen unterscheiden sich hinsichtlich Erkrankungsalter, Geschlechtsgebundenheit, Häufigkeit organischer Hirnschäden, bestimmter EEG-Veränderungen wie vor allem abnormer Rhythmisierungen, Verlaufstendenz des Leidens und anderes. Es wird vermutet, daß die verschiedenen Verlaufsformen der Absence-Epilepsien nicht nur alters- und geschlechtsgebundene Modi fikationen eines pathophysiologisch einheitlichen Geschehens sind, sonder daß auch unterschiedliche genetische Faktoren von Bedeutung sein könnten.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00361231
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  • 7
    Electronic Resource
    Electronic Resource
    Schwangerschaftsverlauf, aktueller Geburtszustand und neurologische Entwicklung bei Risikokindern (1977)
    Springer
    Archives of gynecology and obstetrics 224 (1977), S. 244-246 
    Details
    ISSN: 1432-0711
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00679539
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  • 8
    Electronic Resource
    Electronic Resource
    Cranial ultrasound findings in aspartoacylase deficiency (Canavan disease) (1993)
    Springer
    Pediatric radiology 23 (1993), S. 395-397 
    Details
    ISSN: 1432-1998
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Canavan disease (CD) is a rare leukodystrophy which is lethal in infancy or early childhood. The underlying biochemical abnormality in CD is a hereditary deficiency ofN-aspartoacylase transmitted in an autosomal recessive fashion. We report on the ultrasound (US), CT, and MRI findings of three unrelated boys with biochemically confirmed CD. At 6 and 9 months of age, two CD patients with rapid neurological deterioration showed markedly enhanced acoustic attenuation of the white matter with the exception of the corpus callosum, giving the appearance of a reversed pattern of echogenicity of cortical gray and subcortical white matter. While gyri and sulci had an almost normal US appearance, the periventricular gray matter featured prominently with increased echogenicity. In contrast another CD patient with a more protracted course had ventricular enlargement when examined by US at 5 and 9 months but no alteration in white matter echogenicity. MRI showed impaired myelinization in all three patients with Canavan disease.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02011970
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  • 9
    Electronic Resource
    Electronic Resource
    Severe transitory encephalopathy with reversible lesions of the claustrum (1996)
    Springer
    Pediatric radiology 26 (1996), S. 769-771 
    Details
    ISSN: 1432-1998
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Reversible bilateral lesions of the claustrum and external capsule in a 12-year-old girl suffering from a severe, transitory encephalopathy are reported. After a prodromal stage of feeling uncomfortable a sudden onset of status epilepticus occurred, followed by recurrent complex partial and myoclonic seizures for 3 weeks, with psychotic symptoms and temporary loss of vision, speech and hearing. After treatment with phenytoin the patient became free of seizures and recovered completely without neurological deficit. The initial cranial CTwas normal; however, cranial MRI 7 days later showed bilateral selective lesions of the claustrum and external capsule, which disappeared completely 5 weeks later. The aetiology of these lesions remains obscure; repeated cerebrospinal fluid and blood tests were negative for herpes simplex virus and other infectious agents. The clinical and radiological improvement were concomitant. This may indicate a functional disturbance of the claustrum grey matter, rather than lesions of the white matter of the external and extreme capsules.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01396197
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  • 10
    Electronic Resource
    Electronic Resource
    Über die Beziehungen zwischen Absencen, psychomotorischen und fokalen Anfällen (1965)
    Springer
    European archives of psychiatry and clinical neuroscience 206 (1965), S. 504-524 
    Details
    ISSN: 1433-8491
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Zusammenfassung Nach klinischen und elektrencephalographischen Verlaufsbeobachtungen bei 101 Patienten werden die Beziehungen zwischen Absencen, psychomotorischen und fokalen Epilepsien dargestellt. 1. Die vielfach als Kriterium für die differentialdiagnostische Trennung von Absencen und psychomotorischen Anfällen angesehenen Automatismen (orale, gestikulatorische usw.) sind unspezifisch. Zwischen ihrem Auftreten und bestimmten Verlaufsformen und EEG-Befunden der Absence-Epilepsie bestehen keinerlei Beziehungen. 2. Für die pathogenetische Deutung von Krankheitsbildern, in denen reine Absencen und Anfälle fokaler Prägung nebeneinander oder im Verlauf nacheinander vorkommen, werden zwei Möglichkeiten erörtert: a) Eine primär generalisierte, „centrencephale“ Epilepsie im Sinne Penfields kann im Gefolge gehäufter großer Anfälle sekundär zu fokalen Symptomen oder EEG-Herden führen. b) Die Petit mal-Epilepsie kann sich auf dem Boden einer organischen Hirnschädigung entwickeln. Verlaufsbeobachtungen und Untersuchungen des Erbumkreises sprechen dafür, daß in solchen Fällen dem organischen Hirnschaden lediglich die Bedeutung einer Auslösung für die anlagebedingte Spike-wave-Epilepsie zukommt. 3. Die Gesamtheit der klinischen und elektrencephalographischen Beobachtungen läßt aus der Vielfalt der Erscheinungsbilder, unter denen „gemischte“ Epilepsien mit fokalen und Entladungsformen vom Spitze-Welle-Komplex-Typ auftreten, die sogenannten „centrencephale“ Epilepsieformen als eigene Gruppe hervortreten. Die Ergebnisse kommen somit den ursprünglichen Vorstellungen Penfields von der sogenannten „centrencephalen“ Epilepsie entgegen und ergänzen andererseits vom klinischen Bereich her die erbbiologischen Ergebnisse von Metrakos u. Metrakos.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00343652
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