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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Child's nervous system 9 (1993), S. 23-24 
    ISSN: 1433-0350
    Keywords: Down syndrome ; Reflex seizures ; Closed-circuit electroencephalogram video telemetry ; Treatment
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A unique patient with Down syndrome who developed reflex seizures is described. The patient has had recurrent intermittent seizures for the past 3.5 years. These seizures were usually precipitated by auditory stimuli such as sudden loud noises. While having seizures the patient experienced severe discomfort in the posterior neck area. The seizures usually lasted 10–20 s, and there was no aura, nor was there a postictal phase. Of the numerous investigations performed, closed-circuit electroencephalogram video telemetry was most helpful in arriving at an accurate diagnosis. Carbamazepine administration resulted in total seizure control and reflex stimuli no longer provoke seizure activity. Although it has been suggested that reflex seizures may be due to genetic factors or structural central nervous system defects, we were unable to uncover the cause of the reflex seizures in our patient.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Child's nervous system 9 (1993), S. 452-457 
    ISSN: 1433-0350
    Keywords: Adenotonsillar enlargement ; Sleep-disordered breathing ; Sleep apnea ; Attention deficit disorder ; Down syndrome
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Sleep-related breathing disorders may cause excessive daytime sleepiness, cognitive impairment, and behavior problems in children and adolescents. Adenotonsillar enlargement (AT) is known to be a significant risk factor for these disorders, which have also been reported in several patients with Down syndrome (DS). Children with attention deficit disorder/hyperactivity (ADD) show behavior problems that may be related to disturbed nocturnal sleep in some. To evaluate the relationships among these disorders and symptoms, parents of 29 school-aged children with AT, 70 with DS and 48 of their siblings (DS-SIB), and 21 with ADD completed a 20-item screening questionnaire covering nocturnal sleep symptoms and daytime behavior problems. Nocturnal symptoms of sleep-related breathing disorders — snoring, breathing pauses during sleep — were reported more commonly by parents of AT and DS children. However, parents of two of the ADD children reported significant signs of sleep-related breathing disorders. Daytime behavior problems were more common in ADD and AT than in the DS group. Bedwetting reports did not distinguish groups. Direct comparisons of DS and DS-SIB groups showed that more DS were mouth breathers, snored, stopped breathing at night, and were sleepy in the daytime. These findings underscore the importance of obtaining a history of nocturnal sleep from parents of children with AT and DS, as well as those with disrupted daytime behavior.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Child's nervous system 11 (1995), S. 639-642 
    ISSN: 1433-0350
    Keywords: Zellweger syndrome ; Peroxisomal disorders ; Central nervous system ; Very long chain fatty acids
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract An infant with Zellweger syndrome is reported. A detailed description of the clinical findings is provided. In particular, the neuropathological aspects are highlighted and the underlying biochemical derangements discussed. In addition, some of the known pathogenetic mechanisms that are involved in producing the phenotype of Zellweger syndrome are analyzed.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Child's nervous system 8 (1992), S. 45-48 
    ISSN: 1433-0350
    Keywords: Lowe syndrome ; Central nervous system ; Mental retardation ; Cataracts
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We describe three patients with Lowe (oculocerebrorenal) syndrome, emphasizing primarily the central nervous system and renal pathology. Using magnetic resonance imaging, we noted diffuse high T2 signals periventricularly, indicating significant white matter destruction, which may be responsible in part for the mental retardation, seizure disorder, hypotonia, and areflexia observed in the patients. In contrast to previously published reports, there was minimal renal tubular dysfunction; however, proteinuria was significantly increased in all patients. We believe that the observed proteinuria is primarily the result of glomerular pathology rather than renal tubular dysfunction and may represent a net loss of negative charges within the glomerular filter. This loss of charge may be linked to the increased excretion of glycosaminoglycans in the urine.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Child's nervous system 8 (1992), S. 61-66 
    ISSN: 1433-0350
    Keywords: Myoclonic dystonia ; Therapy ; Rehabilitation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Myoclonic dystonia is a rare disorder that occurs in an hereditary and a sporadic form. The autosomal-dominantly inherited form is responsive to alcohol but not to other drugs. The sporadic form has been relatively resistant to drug treatment. We report a young man with myoclonic dystonia who displayed only little response to alcohol but improved significantly with a combination of sodium valproate for myoclonus and trihexiphenidyl hydrochloride for dystonia. His rehabilitation, however, was confounded by public authorities who thought the patient's appearance was indicative of drug use.
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    Applied microbiology and biotechnology 27 (1987), S. 229-234 
    ISSN: 1432-0614
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Process Engineering, Biotechnology, Nutrition Technology
    Notes: Summary The formation of acetic acid by the thermophilic nonsporeforming homoacetogenic bacterium Acetogenium kivui was studied under various conditions. In pH-controlled batch fermentation at pH 6.4 this bacterium was able to produce up to 625 mM of acetic acid from glucose within 50–60 h. The value of μ max obtained was about 0.17 h-1, the yield was about 2.55 mol of acetic acid per mol of glucose utilized. In continuous fermentation both substrate concentration and dilution rate (D) influenced the yield of acetate and the stationary concentration: a glucose concentration of 67 mM at D=0.09 h-1 resulted in 2.82 mol acetate/mol glucose and 190 mM acetate at a production rate of 17.1 mM/1 h. When the dilution rate was increased the production rate reached a maximal value of 43.2 mM/1 h at D=0.32 h-1. At a glucose concentration of 195 mM the dependence of yield upon dilution rate followed a similar pattern and an acetate concentration of 420 mM could be obtained. Enzymatic studies indicate that in A. kivui pyruvate ferredoxin-oxidoreductase and acetate kinase are inhibited at acetate concentrations higher than 800 mM. Based on these results a fed-batch fermentation was developed, which allowed to produce more than 700 mM acetic acid within 40–50 h.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    Archives of microbiology 159 (1993), S. 484-490 
    ISSN: 1432-072X
    Keywords: Zymomonas ; Glucose catabolism ; Ethanol inhibition ; 31P NMR in vivo
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology
    Notes: Abstract Ethanol inhibition of glucose catabolism in Zymomonas mobilis was investigated using 31P NMR spectroscopy in vivo and of perchloric acid extracts from cell suspensions incubated with 0, 5 and 10% (w/v) ethanol. In vivo 31P NMR experiments revealed slower glucose utilization and decreased levels of nucleoside triphosphates in the presence of 10% ethanol as compared to controls. Using 31P NMR spectroscopy of perchloric acid extracts, intracellular accumulation of 3.4 mM 3-phosphoglycerate was found when 10% ethanol was present in the medium. No accumulation of this metabolite occurred in cells incubated with 0 and 5% ethanol. Enzyme assays confirmed that phosphoglycerate-mutase and enolase were inhibited 31 and 40%, respectively, in the presence of 10% ethanol in the test system. Therefore, under the conditions used the decrease in the fermentative activity of Z. mobilis at high ethanol concentrations is due to inhibition of phosphoglycerate-mutase and enolase.
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Oxford, UK : Munksgaard International Publishers
    ISSN: 1600-0501
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Abstract: It is generally assumed that passively fitting superstructures are a prerequisite for long-lasting implant success. In the study presented, the strain development of three-unit implant fixed partial dentures (FPDs) was evaluated at the bone surrounding the implant and on the superstructure using a strain gauge technique. Six groups of three-unit FPDs representing the commonly used techniques of bridge fabrication were investigated with 10 samples each, in order to quantify the influence of impression technique, mode of fabrication and retention mechanism on superstructure fit. Two ITI implants (Straumann, Waldenburg, Switzerland) were anchored in a measurement model according to a real-life patient situation and strain gauges were fixed mesially and distally adjacent to the implants and on the bridge pontics. The developing strains were recorded during cement setting and screw fixation. For statistical analysis, multivariate two sample tests were performed setting the level of significance at P=0.1. None of the investigated bridges revealed a truly passive fit without strains occurring. About 50% of the measured strains were found to be due to impression taking and model fabrication, whereas the remaining 50% were related to laboratory inaccuracies. The two impression techniques used did not reveal any significant differences in terms of precision. Both modes of fixation – i.e. cement and screw retention – provoked equally high stress levels. In the fabrication of screw-retained FPDs, similar results were obtained from the use of burn-out plastic copings and the technique of casting wax moulds to premachined components. Bonding bridge frames onto gold cylinders directly on the implants significantly reduces strain development.
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Springer
    ISSN: 1617-5786
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Chemistry and Pharmacology , Medicine
    Type of Medium: Electronic Resource
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  • 10
    Electronic Resource
    Electronic Resource
    Springer
    Clinical autonomic research 10 (2000), S. 317-321 
    ISSN: 1619-1560
    Keywords: nociception ; irritation ; mucosal blood flow ; laser Doppler ; oral cavity ; aging
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The aim of the current pilot study was to establish a procedure that would allow the investigation of microcirculatory changes in the oral cavity. The authors studied the effects of painful stimulation using dry ice (CO2). To investigate potential regional differences in the change of blood flow, recordings were made for the tongue and at the mucosa of the hard palate, lip, and oral vestibule. The authors investigated 26 patients divided into groups of younger subjects (10 men, 3 women; age range 21–31 y) and older patients (2 men, 11 women; age range 54–74 y). Mucosal blood flow (mBF) was obtained at the hard palate, at the tip of the tongue, on the midline of the oral vestibule, and at the lip. Measurements were made during rest and for 2 minutes after application of dry ice for a 10-second duration, using a pencil-shaped apparatus. Blood pressure, heart rate, cutaneous blood flow, transcutaneous partial pressure of carbon dioxiode (Pco2) and partial pressure of oxygen (Po2) were recorded. Mucosal blood flow increased at all sites in response to application of dry ice (p〈0.001), with peak flow at 0.5 minute to 1.5 minutes after onset of stimulation. During the 1.5 minutes to 2 minutes, blood flow decreased at all measurement sites with a tendency to return to baseline. Heart rate, blood pressure, pCO2, pO2, and cutaneous blood flow did not show significant changes. Overall, responses in older patients showed more variance when compared with younger patients. Stimulation by dry ice appears to be an effective, noninvasive, and tolerable means to investigate mucosal blood flow at different mucosal sites. Preliminary data indicate different levels of responsiveness to painful cold stimulation at different sites on the oral and perioral mucosa; particularly, mucosal blood flow response at the tongue was least pronounced. Therefore, assessment of stimulated mucosal blood flow appears to be a promising tool to investigate the pathophysiology of a number of neurologic symptoms, eg, the burning mouth syndrome.
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