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Biomedical subjects

U Kramer

Publications and source records attributed to U Kramer.

At least 37 records · Page 2Linked to original sources
PubMed2001

Prognostic significance of hyperechogenic lesions in the basal ganglia and thalamus in neonates.

Neonatal cranial ultrasonography at times reveals hyperechogenic lesions in the basal ganglia and thalamus. These lesions have been attributed to a wide variety of pathologic states, among them toxoplasmosis, rubella, cytomegalovirus, and herpes simplex (TORCH) infections, chromosomal abnormalities, and asphyxia. The clinical significance in terms of the neurodevelopmental outcome of this radiologic abnormality is unknown. We performed a developmental evaluation on 16 children aged 2 to 6 years in whom neonatal cranial ultrasonography had demonstrated hyperechogenic lesions in the basal ganglia or thalamus and had no other neurodevelopmental risk factors. There was no significant difference between the average Developmental Quotient of the target population and the normal population in regard to developmental status. We conclude that in our population, an isolated finding of hyperechogenic lesions in the basal ganglia is probably not a predictor of poor neurodevelopmental outcome.

Basal Ganglia↗
PubMed2001

[Benign childhood epilepsy with centro-temporal spikes].

Benign childhood epilepsy with centro-temporal spikes is a specific pediatric syndrome, characterized by typical clinical symptoms including hemifacial convulsions which tend to generalize in sleep. There is no loss of consciousness. The typical EEG shows blunt diphasic spikes, localized in the rolandic or mid-temporal area. The spikes are high voltage and increase in drowsiness and sleep. In all these patients there is a normalization of the EEG and cessation of seizures in a few years, even without treatment. All these pediatric patients recover before adulthood.

Anticonvulsants↗
PubMed2000

[Phenobarbital coma for psychogenic status epilepticus].

Among patients seen for epileptic seizures, there is a subgroup of those who do not have epilepsy but suffer from psychogenic paroxysmal events which mimic epileptic seizures. The differential diagnosis between them is of the utmost importance, as incorrect diagnosis of seizures subjects the patient to incorrect management. In the case psychogenic status epilepticus, the treatment may endanger the patient. We report 3 women, aged 20, 20 and 28 and a man aged 24 with psychogenic status epilepticus admitted to an intensive care unit and treated by induction of phenobarbital coma when other antiepileptic medication failed.

Adult↗
PubMed2000

Unprovoked seizures after complex febrile convulsions.

Children with complex febrile convulsions bear a higher risk of developing epilepsy than children with simple febrile convulsions. Complex febrile convulsions are defined by the presence of prolonged seizures, partial seizures and multiple seizures occurring during the same day. The aim of this study is to delineate the relative significance of each of the three criteria defining complex febrile convulsions. Fifty-seven out of 477 children (12%) admitted for febrile convulsions had complex febrile convulsions and normal neurological examination. Follow-up was available for 48 (84%) of them. Thirteen of these 48 (27%) had epilepsy at follow-up. The mean age of seizure onset among the patients with subsequent afebrile seizures was significantly lower than the rest (10.8 months versus 16.8 months). The patients with partial febrile convulsions showed a trend toward a higher risk (45%) of developing epilepsy than the patients with multiple febrile convulsions (21%).

Child↗
PubMed2000

Mental retardation subsequent to refractory partial seizures in infancy.

Whether seizures are the direct cause of cognitive deterioration in epileptic children is undetermined. This retrospective study aimed to delineate a subgroup of pediatric patients with cognitive deterioration and refractory seizures in the absence of recognized causes for mental retardation. Of the 80 children identified as having mental retardation and refractory seizure disorder, seven (8.7%) had normal cognitive development until at least 1 year of age. Their metabolic status was normal. Five of them suffered repeated frequent partial seizures with onset in the first year of life and two had repeated episodes of status epilepticus. All seven had similar characteristics of early onset partial seizures, six of them had partial seizures secondarily generalized and one had complex partial seizures. The time of peak cognitive deterioration correlated with increases in seizure frequency during that period. Evaluation revealed a well-defined epileptic focus in the absence of neuroimaging abnormality except for hippocampal atrophy in the two children with complex partial seizures and a small vascular malformation in one child. Uncontrolled partial seizures in the first months of life may result in cognitive deterioration.

Adolescent↗
PubMed2000

Alternating hemiplegia of childhood: clinical manifestations and long-term outcome.

We present our analysis of 44 patients with alternating hemiplegia of childhood. The clinical course usually consisted of three phases. The first was dominated by abnormal eye movements and dystonic episodes, the second by hemiplegic spells and psychomotor regression, and the third by persistent developmental delay and fixed neurologic deficits. The age of onset was 0-54 months (mean = 7.9 +/- 13 months). The presenting signs included abnormal ocular movements in 65%, dystonia in 60%, and hemiplegia in 32%. Patients with an early onset of the disorder and an early appearance of hemiplegic spells faired the poorest developmentally. Developmental delay was present in 91%, ataxia in 68%, choreoathetosis in 50%, and seizures in 18%. Laboratory investigations suggested mitochondrial abnormalities and cerebrovascular dysfunction in several patients. Numerous therapies were largely ineffective. Flunarizine reduced the duration, severity, and frequency of the hemiplegic attacks in 78%. Patients who received flunarizine did not differ developmentally from those who did not. Our data suggest that flunarizine does not adversely affect and may favorably influence the outcome in patients with alternating hemiplegia of childhood. Additionally, the occurrence of autosomal-dominant cases of the syndrome, although rare, suggests that, in addition to mitochondrial dysfunction, genetic factors may be important.

Adolescent↗
PubMed2000

Outcome of infants with unilateral Sturge-Weber syndrome and early onset seizures.

Patients with Sturge-Weber syndrome often present with seizures during the first year of life. Currently, only patients with clinically significant seizures who do not respond to medical treatment are candidates for early epileptic surgery. However, a delay of surgical treatment may result in cognitive deterioration. We studied the correlation between parameters and outcome of seizures to re-examine the criteria for early epilepsy surgery. We performed a retrospective chart review combined with telephone interviews of parents of all Israeli infants with unilateral Sturge-Weber syndrome and early onset seizures, and we examined whether age of seizure onset and seizure intensity were correlated with cognitive level and the degree of hemiparesis at follow-up. We recruited a total of 15 patients with unilateral Sturge-Weber syndrome and early onset seizures, five of whom underwent epilepsy surgery. The mean follow-up period of all the patients was 15 years: six patients had normal intelligence, four had borderline cognitive level, three had mild mental retardation and two had moderate mental retardation. Eight of the ten non-operated patients still experience seizures at follow-up. Cognitive delay was significantly correlated with seizure intensity in the early period, but not with the age of seizures onset, the degree of hemiparesis, or the presence of ongoing seizures. We conclude that high seizure intensity in young patients with Sturge-Weber syndrome is a prognostic marker for mental deterioration.

Age of Onset↗
PubMed2000

[Variability in the MR tomographic determination of myocardial function and perfusion parameters in health subjects].

PURPOSE: The aim of the study was to evaluate myocardial function and perfusion patterns using magnetic resonance imaging in normal subjects. MATERIALS AND METHODS: 22 healthy volunteers were examined. Cine-mode acquisitions along all three axes of the heart were generated and perfusion was measured on a mid-ventricular short-axis view. Myocardial wall thickening was scored and contrast enhancement during the first pass was analysed. RESULTS: For myocardial wall thickening increasing values from base (24-86%) to apex (63-106%) were found. Contrast enhancement patterns showed regional variability. In addition marked individual differences were detectable. CONCLUSION: Perfusion patterns should be interpreted only in combination with functional parameters. Standard values for perfusion measurements cover very large range as a result of the high individual variability.

Adult↗
PubMed2000

Interferon consensus sequence binding protein confers resistance against Yersinia enterocolitica.

Interferon consensus sequence binding protein (ICSBP)-deficient mice display enhanced susceptibility to intracellular pathogens. At least two distinct immunoregulatory defects are responsible for this phenotype. First, diminished production of reactive oxygen intermediates in macrophages results in impaired intracellular killing of microorganisms. Second, defective early interleukin-12 (IL-12) production upon microbial challenge leads to a failure in gamma interferon (IFN-gamma) induction and subsequently in T helper 1 immune responses. Here, we investigated the role of ICSBP in resistance against the extracellular bacterium Yersinia enterocolitica. ICSBP(-/-) mice failed to produce IL-12 and IFN-gamma, but also IL-4, after Yersinia challenge. In addition, granuloma formation was highly disturbed in infected ICSBP(-/-) mice, leading to multiple necrotic abscesses in affected organs. Consequently, ICSBP(-/-) mice rapidly succumbed to acute Yersinia infection. In vitro treatment of spleen cells from ICSBP(-/-) mice with recombinant IL-12 (rIL-12) or rIL-18 in combination with a second stimulus resulted in IFN-gamma induction. In experimental therapy of infected ICSBP(-/-) mice, we observed that administration of rIL-12 induced IFN-gamma production which was associated with improved resistance to Yersinia. In contrast, treatment with rIL-18 failed to enhance endogenous IFN-gamma production but nevertheless reduced bacterial burden in ICSBP(-/-) mice. Although cytokine therapy with rIL-12 or rIL-18 ameliorated the course of Yersinia infection in ICSBP(-/-) mice, both cytokines failed to completely restore impaired immunity. Taken together, the results indicate that the transcription factor ICSBP is essential for efficient host immune defense against Yersinia. These results are important for understanding the complex host immune responses in bacterial infections.

Animals↗
PubMed2000

Alternating hemiplegia of childhood in half-sisters.

We present the family of two girls affected with alternating hemiplegia of childhood who were born to the same mother and different fathers. Previous reports suggested mitochondrial dysfunction as an etiologic mechanism for this disorder. Muscle biopsy, including a measurement of the respiratory chain enzymes, performed in one of the sisters showed no mitochondrial abnormalities. The mode of inheritance is not certain, but an autosomal-dominant gene is most likely.

Adolescent↗
PubMed1999

Characterization and comparison of autistic subgroups: 10 years' experience with autistic children.

To summarize our 10-year experience with autistic children at the Tel Aviv Child Development Center, the files of all 55 children with autism treated at our center over a 10-year period were retrospectively reviewed. Particular attention was addressed to the value of the medical work-up in detecting the etiology of autism and to factors differentiating infantile autism (IA) from autistic-like behavior (ALB). Twenty-four subjects (44%) had IA and 31 (56%) had ALB. These subgroups were compared for demographic, perinatal, familial, neurological, and psychological findings, and outcome at discharge. Associated medical conditions and the yield of metabolic work-up and neuroimaging and electroencephalography studies are discussed. The two subgroups differed only in severity of autistic symptoms and cognitive function (P<0.05), but not in demographic or neurobiological findings. It was concluded that IA and ALB are similar conditions, and autism has a wide continuum of clinical expressions.

Autistic Disorder↗
PubMed1999

Epilepsy in the first year of life: a review.

The prevalence of epilepsy is highest during the first year of life. Approximately 50% of patients with seizure onset between 1 and 12 months of age suffer from West syndrome. The rest have one of 12 well-delineated epilepsy syndromes, or in most cases have what are classified as generalized or partial seizures. The outcome of infants with partial seizures is significantly worse than that of patients with generalized seizures. This is true not only for symptomatic partial seizures but also for a subgroup of infants with cryptogenic partial seizures.

Cross-Sectional Studies↗
PubMed1998

SMA type 2 unrelated to chromosome 5q13.

We describe two brothers with clinical and histological findings of type 2 spinal muscular atrophy (SMA) associated with small head circumference (<2%) and normal cognitive development. No survival motor neuron (SMN) or neuronal apoptosis-inhibitory protein (NAIP) deletions were detected in these sibs, and they were discordant for the haplotypes determined by DNA markers flanking the 5q13 SMA locus. These findings support the presence of a distinct anterior horn disease unrelated to 5q13. This entity may have either autosomal recessive or X-linked inheritance.

Child, Preschool↗
PubMed1998

Electroencephalographic discharges of temporal lobe seizures in children and young adults.

We investigated the discharge morphology and propagation patterns of electroencephalographic seizures of temporal lobe onset in 21 children and young adults who underwent invasive long-term EEG monitoring (LTM). Of those, 15 subsequently underwent anterior temporal lobectomy. The onset was focal in 63%. The most frequent discharge morphology was low amplitude beta (30%) or rhythmic/semirhythmic theta discharge (30%). Thirteen patients displayed several sequences of propagation with different spreading stages along a fixed path. Initial spreading to the ipsilateral frontal lobe was associated with a higher frequency of secondary generalization than initial spreading to the contralateral temporal lobe (P = 0.18). A comparison of 13 patients older than 18 years of age with 8 patients younger than 14 years showed a trend towards a lower rate of propagating from the temporal lobe (P = 0.13) in the younger age group. Discharge morphology was not correlated with age, focality, or outcome of surgery.

Adolescent↗