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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Child's nervous system 11 (1995), S. 55-56 
    ISSN: 1433-0350
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1433-0350
    Keywords: Medulloblastoma ; PNET ; Preoperative chemotherapy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Few studies have been carried out regarding preoperative chemotherapy utilized in the treatment of primitive neuroectodermal tumors/medulloblastomas (PNET/MB). In this paper, the authors report 3 cases of children under three years of age, with a presumed diagnosis of PNET/MB, who were preoperatively treated with chemotherapy consisting of high doses of carboplatin alone. The treatment improved the childrens' clinical condition prior to surgery and facilitated tumor removal, resulting in partial regression of the tumor. The preoperative single-drug therapy did not affect the distological diagnosis or prevent the presence of an effective degree of tumor sensitivity to the drug. All three children are still diseasefree, after a mean follow-up period of 42 months.
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  • 3
    ISSN: 1433-0350
    Keywords: Moyamova disease ; Cerebral ischemia ; SPECT ; Bypass surgery ; Cerebral blood flow ; Acetazolamide ; Frontal lobe
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Regional cerebral blood flow (rCBF) and its reactivity to acetazolamide were analyzed in a series of 15 cases of childhood moyamoya disease, using the xenon-133 inhalation method and single photon emission CT (SPECT). Most of the patients had normal mean hemispheric cerebral blood flow (mCBF), comparable to that of age-matched healthy children. However, they had abnormal rCBF distribution and disturbed reactivity to acetazolamide in the territory of the internal carotid artery, especially in the frontal lobe. Surgical revascularization for the anterior circulation, which consisted of STA-MCA anastomosis and indirect synangiosis, dramatically improved theses cerebral hemodynamics in the anterior circulation, including the frontal lobe, and reactivity to acetazolamide in the whole brain. These results suggest that surgical revascularization for the anterior circulation should be carried out in order to improve cerebral hemodynamics as widely as possible, especially in the frontal lobe, and may improve cerebral perfusion reserve in the posterior circulation.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1433-0350
    Keywords: Hydrocephalus ; Transcranial Doppler ; Pulsatility index ; Resistance index ; Intracranial pressure ; Anterior fontanel pressure
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Cerebral hemodynamic changes in infants with progressive hydrocephalus have been studied with the transcranial Doppler (TCD) technique. Several authors have referred to the correlation between the hemodynamic changes and increased intracranial pressure (ICP). Despite conflicting conclusions on the value of pulsatility index (PI) measurements for monitoring infantile hydrocephalus, these pulsatility indices are the most commonly used for this purpose. Although clinical signs of raised ICP are highly variable and unreliable in infants, assumptions have been made in most of the studies about the presence of elevated ICP on the basis of the patient's clinical state. Few studies have reported on actual ICP values, however, and a direct relationship between ICP and TCD changes has never been adequately demonstrated. In the present study, this relationship was investigated in long-term simultaneous TCD/ICP measurements, in an attempt to develop a noninvasive method of monitoring the effect of ICP on intracranial hemodynamics. Two groups of data sets were established. Group I consisted of pre- and postoperative (shunt implantation) TCD/ICP measurements. Group II were long-term simultaneous TCD/ICP recordings showing significant ICP variations. In most of the postoperative measurements there was a decrease in the average PI and RI values. The correlation between PI or RI and ICP in the long-term simultaneous recordings, however, was generally poor. The risk of obtaining false positive or false negative PI or RI values in short-term measurements was also demonstrated. It can be concluded from our results, besides the wide range of reference values for the Doppler indices and extracranial influences upon them, that the present Doppler indices are inadequate for monitoring the complex intracranial dynamic responses in patients with raised ICP.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1433-0350
    Keywords: Nemaline myopathy ; Cardiac involvement ; Nervous system involvement ; Ubiquitin
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Nemaline myopathy belongs to the group of congenital non-progressive myopathies; however, in rare cases death occurs in early infancy. We report two cases of rapidly fatal nemaline myopathy. The first patient, who died at the age of 26 months, showed atrophy of type 1 fibers containing numerous rods in biopsy sections. Biopsy of the second patient, who had died at the age of 5 months, revealed severe maturational arrest and myopathy, but rods were so rare that diagnosis could only be made at the ultrastructural level. Autopsy of both patients showed that atrophy of type 1 fibers and maturational arrest had disappeared in the very same muscles; rods had moved to a central position in the first and significantly increased in number in the second case. Diaphragma muscles contained abundant amounts of rods in both cases. The cardiac musculature showed a few rods only in the first patient, who had developed heart insufficiency 11 months prior to death. Immunohistochemical analysis showed that rods did not contain desmin or ubiquitin.
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1433-0350
    Keywords: Butorphanol ; Dorsal rhizotomy ; Epidural ; Morphine ; Spasticity
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The authors prospectively evaluated 15 patients who had undergone selective dorsal rhizotomy who were given a continuous morphine/butorphanol infusion, to determine whether variations in the postoperative pain control and side effects seen using a bolus technique could be reduced. Patients had an epidural catheter placed at the end of the operative procedure through which 50–60 μg/kg preservative-free morphine and 15–20 μg/kg butorphanol was administered. A continuous epidural infusion of 5 μg/kg h morphine and 1.2 μg/kg h butorphanol was then initiated. Postoperatively, mean pain scores were excellent. No patient required additional systemic analgesics during the 72-h investigational period. A low incidence of nausea, and no vomiting, pruritus, or respiratory depression was reported by the cohort. All patients maintained oxygen saturations above 95%. This indicates that the use of a continuous epidural infusion provides excellent pain control, decreases the occurrence of untoward side effects, and allows the early initiation of occupational and physical therapy postoperatively.
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  • 7
    ISSN: 1433-0350
    Keywords: Severe myoclonic epilepsy in infancy ; Myoclonic epilepsy ; Mitochondrial cytopathy Infants
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We describe a case in which severe myoclonic epilepsy of infancy is associated with a disturbance in mitochondrial function. EEG traces showed diffuse spike-wave patterns inducible by intermittent photic stimulation. Laboratory analyses revealed high lactic acid levels in cerebrospinal fluid and urine, without metabolic acidosis or high lacticacidaemia. Muscle biopsy showed a slight increase in the number of mitochondria, which had a tendency towards subsarcolemmal locations, and clefts in the myofibrillar membrane that contained granular material staining positive for oxidative enzymes and red with modified Gomori stain. Quantification of the enzymatic activities of homogenized muscle showed partial deficiency of the mitochondrial respiratory chain complexes III and IV. Severe myoclonic epilepsy associated with mitochondrial cytopathy was diagnosed, but the possibility cannot be ruled out that the myoclonic epilepsy (or perhaps simply nonspecific epileptic encephalopathy) was secondary to the mitochondrial cytopathy. Thorough diagnostic analysis in severe myoclonic epilepsy cases is called for with a view to elucidation of a possible metabolic aetiology.
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  • 8
    ISSN: 1433-0350
    Keywords: Hydrocephalus ; Infants ; Ventriculoperitoneal shunt ; Shunt infection ; Risk factors
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Infection remains a major cause of morbidity and mortality following CSF shunt procedures. In this study 191 shunt procedures carried out from January 1981 to December 1992 in a series of 81 infants (less than 6 months old) were retrospectively analyzed for possible risk factors. The overall surgical infection rate was 7.8%, with 15 infections occurring in 14 patients (17.2%). No significant difference in the rate of infections was found in relation to sex, birth weight, gestational age, and type of shunt procedure (primary insertion/revision). The occurrence of other infections during the period of shunt surgery did not influence the infection risk either. Intraventricular hemorrhage and central nervous system infections as causes of the hydrocephalus were found to correlate with septic risk. Young age (less than 6 months) seems to represent the main risk factor, and this is related both to the immunologic deficiency and to the particular features of residential bacterial flora in this age group.
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  • 9
    ISSN: 1433-0350
    Keywords: Meningioma ; Radiation therapy ; Medulloblastoma
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report a case of radiation-induced meningioma that appeared 12 years after the discovery and treatment of a posterior fossa medulloblastoma. The features of the case that support the diagnosis of radiation-induced meningioma are discussed.
    Type of Medium: Electronic Resource
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  • 10
    ISSN: 1433-0350
    Keywords: SPET ; PET ; EEG ; MRI ; Drug-resistant epilepsy ; Epilepsy surgery
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Magnetic resonance imaging (MRI), single photon emission tomography (SPET), and positron emission tomography (PET) using [18F]fluorodeoxyglucose were used in combination with scalp and scalp-video EEGs in a group of 30 pediatric patients with drug resistant epilepsy (DRE) in order to identify patients who could benefit from neurosurgical approach. Seizures were classified according to the consensus criteria of The International League Against Epilepsy. In three patients infantile spasms (IS) were diagnosed; 13 subjects were affected by different types of generalized seizures, associated with complex partial seizures (CPS) in three. In the other 14 patients partial seizures, either simple (SPS) or complex, were present. A localized abnormality was demonstrated in one patient with IS and in three patients with generalized seizures. Of the group of 14 subjects with CPS, MRI and CT were normal in 7, but SPET or PET indicated focal hypoperfusion or hypometabolism concordant with the localization of the EEG abnormalities. In 5 of the other 7 patients anatomical and functional imaging and EEG findings were concordant for a localized abnormality. It can be concluded that functional imaging combined with scalp EEGs appears to be superior to the use of only CT and MRI for selecting children with epilepsy in whom a surgical approach can be considered, in particular when CPS resistant to therapy are present.
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