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Biomedical subjects

U Kramer

Publications and source records attributed to U Kramer.

At least 55 records · Page 3Linked to original sources
PubMed1998

Epidemiology of epilepsy in childhood: a cohort of 440 consecutive patients.

This study analyzes the relative frequency and age of onset of the different seizure types in a 20-year cohort of a pediatric neurology outpatient clinic of an urban hospital that serves the majority of the city's population (Tel Aviv Medical Center). Only patients with two or more unprovoked seizures were included. Neonatal seizures were excluded from the analysis. The different seizure types in descending order of frequency were: partial seizures secondarily generalized (20.6%), complex partial seizures (12.5%), West syndrome (9%), simple partial seizures (8.6%), benign rolandic epilepsy of childhood (8%), absence seizures (7%), generalized tonic-clonic seizures (6.6%), generalized tonic seizures (5%), myoclonic seizures (2.2%), benign occipital epilepsy of childhood (2%), mixed type seizures (1.8%), Lennox-Gastaut syndrome (1.5%), juvenile myoclonic epilepsy (0.9%), atypical absence (0.6%), Landau-Kleffner syndrome, Ohtahara syndrome, myoclonic astatic epilepsy, electrical status epilepticus in sleep and startle epilepsy (0.2% each), and unclassified seizures (12%). The findings of this study confirm that there are more pediatric patients with partial seizures (52%) than primary generalized seizures (33%) and that partial seizures secondarily generalized is the most frequent seizure type in this age group.

Adolescent↗
PubMed1998

Neuroimaging of children with partial seizures.

The predictive value of abnormal neurological findings on neuroimaging (NI) of children with partial seizures (PS) was studied. The sample comprised 143 children and adolescents with PS from 1979 to 1996. Fifty patients had the following abnormal NI findings: diffuse atrophy and porencephalic cyst (5.6% each), hemiatrophy, tumors, neurocutaneous syndrome brain lesions, and dysgenesis (4.2% each), and arachnoid cyst and hydrocephalus (2.1% each). There were significantly more NI abnormalities among those with simple PS than among the other groups of complex PS or PS with secondary generalization. All cases of porencephalic cyst (n = 8), and hemiatrophy (n = 6) were in the hemiparetic group, all cases of tumor (n = 6) were in the normal group, while most cases of diffuse atrophy were in the mentally retarded group. Patients with abnormal NIs had a significantly earlier age of seizure onset than the others. The chance of finding a treatable abnormality in neuroimaging was 5% for tumors and 2% for arachnoid cysts in the group of patients with PS and normal neurological findings. Neither an abnormality detected in a neurological examination nor the type of seizure are predictive parameters for suggesting the presence of a resectable brain tumor. All patients with newly diagnosed PS should undergo an MRI.

Adolescent↗
PubMed1998

Generalized tonic and tonic-clonic seizures of childhood.

In a retrospective analysis of all our patients with seizure onset prior to age 16 years, 25 patients with primary generalized tonic (n = 10) or tonic-clonic (n = 15) seizures were identified. These patients constituted 5.7% of the total seizure patient population in our institute between the ages of 1 month and 16 years. The natural history of generalized tonic-clonic seizures is known to be benign; however, that of isolated primary generalized tonic seizures is not clear. Therefore, an attempt was made to characterize the patients suffering from primary generalized tonic seizures and determine their outcome. Analysis of our patient population shows that both seizure types are characterized by early onset of generalized seizures that appear in normally developed children with a normal electroencephalographic background. The children usually respond quickly to antiepileptic drugs. A long-term follow-up (mean period of 7.6 years) was possible in 84% of the patients, and showed that 95% of them were seizure free at the end of the follow-up period. There was no significant difference between the two groups in regard to age of onset, family history, and seizures at follow-up. In conclusion, the natural history of patients with generalized tonic seizures is similar to the benign course of those with generalized tonic-clonic seizures.

Adolescent↗
PubMed1998

[Ceramic cups for hip endoprostheses. 5: Consideration of designs].

Using the combination ceramic on ceramic for hip prostheses, the wear rate can be reduced to less than 1 micron per million cycles. More than 20 years of experience are now available for various concepts involving alumina ceramics in THR. In the early days, monolithic ceramic cups were employed. However, since alumina ceramic has an unsatisfactory potential for bone integration, soft tissue forms at the interface, with the result that the sockets migrate and penetrate. To improve bony integration, monoblock cups surfaced with ceramic beads were introduced. For the past 10 years, there has been a trend towards the use of modular acetabular components comprising a metal shell and a liner made of Biolox forte ceramic. Two concepts for the fixation of the ceramic liners are employed: locking of the ceramic in the metal shell (CeraLock) and the sandwich concept with a polyethylene layer interposed between liner and shell. The basic design and important aspects such as diameter, range of motion, and the possibility for revision, clinical experience, and trends are discussed.

Ceramics↗
PubMed1997

Focal features in West syndrome indicating candidacy for surgery.

A subgroup of patients with West syndrome (WS) also have focal features, and some of these patients respond to surgical intervention. To determine the incidence of focal features in patients with WS and to establish the correlation of these features with outcome, all patients with WS treated in the Epilepsy Unit of the Children's Hospital (Boston, MA) during the years 1989 to 1992 were retrospectively evaluated. Focal features included partial seizures, hemiparesis, focal radiologic findings, asymmetric spasms, and lateralized hypsarrhythmia. The different focal features were later correlated with the natural history of the disease. Sixty-seven patients were included in the study. Sixty-six percent had asymmetric manifestations. Asymmetric spasms, hemihypsarrhythmia, and partial seizures combined with infantile spasms had significant association with asymmetric brain pathology. Focal features did not correlate with age of onset and outcome, but were associated with etiology. Eight of the nine patients who underwent epilepsy surgery had at least two focal features or more. Two of the operated children had focal features in the absence of magnetic resonance imaging abnormalities, thus indicating the necessity for positron emission tomography, the results of which justified surgery.

Brain↗
PubMed1997

Clinical characteristics of complex partial seizures: a temporal versus a frontal lobe onset.

The site of origin of complex partial seizures (CPS) is predominantly in the temporal lobe, but frontal, parietal, and occipital lobes may also be involved. In order to correlate clinical behaviours with either a temporal or frontal site of origin, we analysed 211 seizures occurring during invasive monitoring in 26 patients with temporal lobe epilepsy (TLE) and in eight patients with frontal lobe epilepsy (FLE). Although leg movements, defined as thrashing, pedalling and kicking, hand posturing, facial twitching, sitting up, and tonic-clonic movements occurred more frequently in FLE, and hand automatisms were more frequent in TLE, no statistically significant difference was found between the two groups. When analysing only electrographic seizures that did not spread or propagated only to homologous controlateral lobes, leg movements and hand posturing were seen only with FLE, and oral automatisms only in TLE. Seizures of temporal lobe origin were longer in duration and had a higher frequency of auras. We therefore conclude that the reliability of clinical behaviour alone to predict the site of origin of an epileptic discharge is limited when the surface EEG is equivocal or the neuroradiologic evidence of a focus is not clear.

Adolescent↗
PubMed1997

A family with hemiplegic migraine and focal seizures.

Familial hemiplegic migraine is a distinctive form of migraine with autosomal dominant inheritance. The patients undergo attacks of migraine complicated by hemiplegia. Seizures have not been reported as comprising a part of this syndrome. We describe three generations of a family with hemiplegic migraine and focal seizures occurring concurrently with the migrainous attacks. There were five affected family members whose clinical features included unilateral headache and transient hemiplegia. Two family members also had focal seizures during the migrainous attacks. One of the patients was treated with carbamazepine with good results. The only associated neurological finding was ataxia which was found in the oldest patient. The presence of focal seizures during an episode of hemiplegic migraine suggests that the two phenomena of migraine and focal seizures may share the same underlying pathophysiology.

Adolescent↗
PubMed1997

Outcome of seizures in the first year of life.

AIM: To determine the frequency and natural history of seizures having an onset during the first year of life. METHODS: A retrospective analysis of the files of all patients treated in the paediatric neurology clinic of our medical centre during 1975-1995. RESULTS: Of our 482 patients with seizure onset prior to age 16 years, the first seizure occurred at age 1-12 months in 80 (16.6%). Of those, 38 (48%) had West syndrome and the rest were classified as follows--partial seizures with or without secondary generalization: 19%, generalized tonic seizures: 8.5%, generalized tonic-clonic seizures: 7.5%, myoclonic: 7.5%, unclassified: 7.5%, and mixed-type seizures: 2%. Follow-up was possible in 91% of the patients. Mean follow-up period from seizure onset was 10 years. Of the non-West syndrome patients who were followed, 19 (50%) were still experiencing seizures at follow-up. Eleven of the 15 patients (73%) with partial seizures and four of the 13 patients (31%) with generalized tonic or tonic-clonic seizures had symptomatic seizures. Of the 14 followed patients with partial seizures, 10 (71%) still had seizures at follow-up, as did three (25%) of the 12 followed patients with generalized tonic or tonic-clonic seizures (p = 0.023). CONCLUSIONS: The partial seizures were most often symptomatic with unfavourable prognosis, while the generalized seizures were either symptomatic and severe or cryptogenic and rapidly responsive to anti-epileptic drugs with good outcome.

Adolescent↗
PubMed1997

L-type calcium channels in insulin-secreting cells: biochemical characterization and phosphorylation in RINm5F cells.

Opening of dihydropyridine-sensitive voltage-dependent L-type Ca2+-channels (LTCCs) represents the final common pathway for insulin secretion in pancreatic beta-cells and related cell lines. In insulin-secreting cells their exact subunit composition is unknown. We therefore investigated the subunit structure of (+)-[3H]isradipine-labeled LTCCs in insulin-secreting RINm5F cells. Using subunit-specific antibodies we demonstrate that alpha1C subunits (199 kDa, short form) contribute only a minor portion of the total alpha1 immunoreactivity in membranes and partially purified Ca2+-channel preparations. However, alpha1C forms a major constituent of (+)-[3H]isradipine-labeled LTCCs as 54% of solubilized (+)-[3H]isradipine-binding activity was specifically immunoprecipitated by alpha1C antibodies. Phosphorylation of immunopurified alpha1C with cAMP-dependent protein kinase revealed the existence of an additional 240-kDa species (long form), that remained undetected in Western blots. Fifty seven percent of labeled LTCCs were immunoprecipitated by an anti-beta-antibody directed against all known beta-subunits. Isoform-specific antibodies revealed that these mainly corresponded to beta1b- and beta3-subunits. We found beta2- and beta4-subunits to be major constituents of cardiac and brain L-type channels, respectively, but not part of L-type channels in RINm5F cells. We conclude that alpha1C is a major constituent of dihydropyridine-labeled LTCCs in RINm5F cells, its long form serving as a substrate for cAMP-dependent protein kinase. beta1b- and beta3-Subunits were also found to associate with L-type channels in these cells. These isoforms may therefore represent biochemical targets for the modulation of LTCC activity in RINm5F cells.

Calcium Channels↗
PubMed1997

Hypsarrhythmia: frequency of variant patterns and correlation with etiology and outcome.

To determine the frequency and significance of the EEG features of hypsarrhythmia, we analyzed the pre-ACTH records of 53 consecutive patients with infantile spasms for the severity of the following abnormalities: disorganization of background, slowing, high amplitude, spike activity, and for the presence or absence of each of the following patterns and variants: electrodecremental discharges, absence of normal sleep activity, relative normalization, hemihypsarrhythmia, burst suppression (BS), occipital hypsarrhythmia, interhemispheric asymmetry, and interhemispheric synchronization. We calculated a total score indicating the severity of the hypsarrhythmia for each record. The hypsarrhythmia variant patterns occurred frequently in up to 69% of the records. Patients with cerebral dysgenesis were more likely to have hemihypsarrhythmia or BS pattern persistent throughout the EEG. Patients with history of perinatal hypoxia-ischemia were more likely to have absence of normal sleep activity. The occurrence of each of the other variant patterns did not correlate with etiology. Favorable outcome did not correlate with the occurrence, or absence, of any of the variant patterns but was associated with faster background activity (< 75% delta), a lower total hypsarrhythmia score (< or = 10), and with absence of electrodecremental discharges on the pre-ACTH EEG. We conclude that variant patterns of hypsarrhythmia are frequent, generally do not correlate with prognosis, and thus are best included within the definition of hypsarrhythmia. The severity of the hypsarrhythmia, however, does have significant prognostic implications.

Adrenocorticotropic Hormone↗
PubMed1997

Electroencephalography in the evaluation of headache patients: a review.

The intention of this paper was to review the literature on the use of EEG in headache evaluation and recommend clinical applications of this technique. The consensus of published findings was that EEG can differentiate migraineurs from controls but that it is not justified for routine diagnosis of headache etiology. We conclude that neuroimaging is a superior technique for detecting underlying structural lesions.

Brain↗
PubMed1996

Differential diagnosis of staring spells in children: a video-EEG study.

Staring is frequently a nonepileptic manifestation in children. To differentiate epileptic versus nonepileptic staring, we reviewed clinical and video-EEG findings in 143 patients, aged 5 months to 43 years, monitored for staring episodes. In 79 patients staring was of epileptic origin; 46 had partial seizures and 33 atypical absence. Thirty-five had behavioral staring, 8 psychogenic seizures, 1 a migraine equivalent, and in 20 no staring spells were recorded. In all patients with epileptic staring, epilepsy was suspected clinically. Only 22 of the admissions for behavioral staring and 3 for pseudoseizures were to exclude a possible nonepileptic phenomenon. Review of their clinical histories revealed that certain findings strongly support a nonepileptic origin. In conclusion, a careful clinical history will differentiate between epileptic and nonepileptic staring episodes in most patients. Video-monitoring is helpful to adjust treatment or to exclude nonepileptic events in patients with refractory staring spells.

Adolescent↗
PubMed1996

Clinical characteristics of children referred to a child development center for evaluation of speech, language, and communication disorders.

Speech, language, and communication disorders are prominent reasons for referrals to a child development center. From 1984 to 1988, 1,090 preschool children were referred to our child development center, which serves the Tel Aviv metropolitan area. Of all referrals, 432 (41%) were primarily due to speech, language, and communication problems. After exclusion of those with IQ < 50 and those with non-language-related disabilities, 323 children remained. The children were classified into different subtypes of developmental language disorders and autistic spectrum disorders. The main developmental language disorder subtypes were combined expressive-receptive (49%) and expressive (44%). Central processing deficits were less common, occurring in 20 (7%) of the children. Parents of children with developmental language disorders had educational levels similar to those of parents of children referred to the child development center for other causes. However, parents of children with infantile autism had higher educational levels than parents of children with developmental language disorder or parents of children referred for other causes (P < .001). Our results reflect the distribution of language and related problems in an unselected population of preschool children referred to a child development center.

Autistic Disorder↗
PubMed1996

Pharyngeal dysesthesia as an aura in temporal lobe epilepsy.

PURPOSE: Because oral, buccal, and sometimes oral-pharyngeal manifestations in epilepsy are linked to the central-temporal region, we studied 3 patients with childhood-onset partial seizures that consistently began with pharyngeal dysesthesias, described as either throat tingling or burning, to localize seizure onset. METHODS: Because of an intractable clinical course, each patient underwent invasive video-EEG monitoring, which localized the epileptogenic zone to the mesial temporal lobe. The 3 patients underwent temporal lobe resections. RESULTS: All 3 patients achieved remission of the pharyngeal auras and a 90-100% reduction in the frequency of their seizures. CONCLUSIONS: Pharyngeal dysesthesias can be the initial manifestation of complex partial seizures (CPS) of temporal lobe origin.

Adolescent↗
PubMed1995

EEG prior to hemispherectomy: correlation with outcome and pathology.

Hemispherectomy, for the treatment of seizures, is highly successful but has a significant morbidity rate. The procedure is usually restricted to patients with an intractable seizure disorder and hemiparesis. Because of the inherent risk of surgery, patient selection is a critical issue. This report describes the evaluation of background activity and ictal patterns on surface and invasive EEG in 12 children who underwent both anatomical (7) and functional (5) hemispherectomy in order to determine the role of electroencephalography in the selection of patients for hemispherectomy, and to correlate EEG findings with underlying pathology and outcome. A favorable outcome was predicted by an interictal EEG with two or more of the following: suppression over the abnormal hemisphere, absence of contralateral slowing, absence of generalized discharges and absence of bilateral independent spiking; or by unilateral onset of ictal discharges on invasive intracerebral EEG recording. Outcome did not correlate with the underlying pathology. Hemispherectomy can be successful in patients with a variety of predominantly unilateral pathologic entities.

Brain↗
PubMed1995

Psychogenic seizures: video telemetry observations in 27 patients.

Psychogenic seizures are unusual during the first decade of life. To compare the clinical features of psychogenic seizures in young children with those of teenagers, the long-term electroencephalographic and video monitoring studies of all patients younger than 18 years of age with recorded episodes diagnosed as psychogenic seizures were reviewed from a single hospital during the past 7 years. The 27 patients were divided into 2 age groups: group A, 6-9 years (n = 5), and group B, 10-17 years (n = 22). All patients had habitual episodes recorded during monitoring. Although the adolescents displayed clinical patterns similar to adult patients with psychogenic seizures, the children demonstrated a clinical pattern characterized mainly by prolonged staring and unresponsiveness. The most common behaviors in the adolescent group were tremor (45%), intermittent stiffening (41%), and out-of-phase movements of the extremities (36%). Fifteen percent of the patients had a history of seizures. This study suggests that young children with psychogenic seizures have clinical profiles different from that of teenagers.

Adolescent↗
PubMed1995

Unprovoked seizures and developmental disabilities: clinical characteristics of children referred to a child development center.

Few data are available on the risk of seizures in young children with developmental problems. A retrospective evaluation of 1,946 children 0-5 years of age referred to the Tel Aviv Child Development Center (CDC) between 1981 and 1990 was performed. The study was undertaken to determine the cumulative risk of unprovoked seizures in children referred to a CDC and to assess the risk factors associated with seizures in these children. The center serves the Tel Aviv area for a variety of developmental disabilities. Cumulative risk of seizures and risk factors were assessed using Kaplan-Meier methodology. Unprovoked seizures occurred in 58 patients (3%), including 10 with a single seizure and 48 with two or more seizures. Risk factors for seizures included cerebral palsy (CP) (relative risk [RR] = 28.7), neonatal seizures (RR = 15.2), mental retardation (MR) (RR = 7.8), febrile seizures (RR = 7.7), autism (RR = 3.2), and prematurity (RR = 2.7). The cumulative risk of seizures by age 5 years in children with MR, CP, and MR plus CP was 8%, 47%, and 68%, respectively, compared with 1% in those without MR or CP. On multivariate analysis, CP, MR, prior febrile seizures, and prematurity were associated with an increased risk of seizures. The risk of experiencing unprovoked seizures by age 5 in children with developmental disabilities is 3%, which is fourfold greater than that of the general population. Much of this increased risk is limited to selected subgroups with major disabilities. However, if neither MR nor CP is present, the 1% risk of developing unprovoked seizures by age 5 in children with other developmental problems is not substantially different from that expected in the general population.

Cerebral Palsy↗