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Biomedical subjects

E Hauser

Publications and source records attributed to E Hauser.

At least 19 recordsLinked to original sources
PubMed1996

Prognosis of childhood epilepsy in newly referred patients.

The aim of this study was to investigate the prognosis of childhood epilepsy and to analyze prognostic factors in addition to remission rate in a follow-up of newly referred patients. Two hundred eighty-one patients were followed for a mean period of 5.3 years. Overall, 253 patients (90%) achieved 1-year remission. The beginning of a 1-year seizure-free period was achieved in 77.9% by 1 year, in 84% by 2 years and in 88.6% by 3 years after onset of treatment. Early onset of seizures, symptomatic etiology, and neurologic handicap predicted a worse prognosis. In 44 of 253 children with complete suppression of seizures for 1 year, relapses occurred within the follow-up period. In one child with a relapse, remission could not be achieved in the 2nd year thereafter. In conclusion, our study shows a good prognosis for most children with epilepsy, especially in patients with idiopathic epilepsy and late onset of seizures and without neurologic dysfunction. Moreover, our data strongly suggest that the long-term pattern of seizure control is largely established during the first 2 years of treatment.

Adolescent↗
PubMed1996

Brainstem auditory evoked potentials and visually evoked potentials in young patients with IDDM.

OBJECTIVE: To investigate whether young IDDM patients develop central nervous dysfunction and to establish a possible relationship with various disease parameters. RESEARCH DESIGN AND METHODS: Thirty-two patients, aged 13.5 +/- 2 years, with disease duration of 6 +/- 2.6 years and age of onset of 7.7 +/- 3.2 years (group 1), and 21 patients with short-term disease, age 9.7 +/- 3.5 years, duration of disease < 2 years and age of onset of 9.4 +/- 3.3 years (group 2) were compared with age- and sex-matched control subjects. Exclusion criteria were clinical signs of neuropathy, retinopathy, nephropathy, or hearing impairment. Neurophysiological studies included auditory and visually evoked potentials (EPs). RESULTS: Patients in group 1 revealed increased P100 latencies of visually EPs (103.4 +/- 4.5 vs. 96.8 +/- 3.7 ms) and interpeak latencies I-V of auditory EPs (4.16 +/- 0.10 vs. 3.99 +/- 0.09 ms) and had abnormal latencies (values outside 2.5 SD) in 37%. However, short-term patients (group 2) had results within normal limits compared with control subjects. In group 1, longer disease duration and younger age at onset correlated with an increase of P100 latency (P < 0.001) and IPL I-V (P < 0.001). Patients with a history of severe hypoglycemic episodes had increased latencies compared with patients without hypoglycemia (P < 0.05). Furthermore, metabolic control during the last 2 years was related to P100 latencies (P < 0.05). CONCLUSIONS: EPs noninvasively detect subclinical central nervous system involvement in children and adolescents with IDDM. Most important risk factors are duration of disease and frequency of severe hypoglycemia.

Acoustic Stimulation↗
PubMed1995

Oxyradical damage and mitochondrial enzyme activities in the mdx mouse.

A number of studies have already been undertaken to investigate involvement of oxyradicals in muscle diseases by means of measurements of oxyradical protective enzymes. We investigated o-tyrosine, which is a biomarker for OH radical damage in vivo, in 10 mdx and 10 control mice. We also measured mitochondrial enzymes in muscle homogenates of 10 mdx and 10 control mice. Mdx mice had significantly elevated values for o-tyrosine, succinat-phenacinmetosulfat oxidoreductase. NADH O2 oxidoreductase and cytochrome C oxidoreductase. Our findings confirm the suggestion that elevated oxyradical production occurs in muscular dystrophies with lack of dystrophin. Furthermore, our results demonstrate that OH radical damage does not impair mitochondrial enzyme activities in the mdx mouse.

Animals↗
PubMed1995

Quantitative EEG in young diabetics.

The aim of this study was to investigate the influence of metabolic control on quantitative electroencephalographic (EEG) abnormalities in young diabetic patients. We compared quantitative EEGs of 44 insulin-dependent diabetic patients with age-matched controls. Furthermore, differences in EEG variables of diabetic patients and controls were correlated with the age of onset and duration of diabetes, hemoglobin A1c concentrations, and episodes with severe hypoglycemia. We found a correlation between high values of hemoglobin A1c and decreased relative power of the alpha band. Patients with previous ketoacidotic episodes had increased relative power of the delta/theta band and decreased relative power of the alpha band. Patients with hemoglobin A1c values less than 8.5% exhibited slowing of background activity compared with normal controls. We conclude that metabolic control influences the EEG and that improvement of glucose metabolism is an important factor in avoiding EEG abnormalities in young diabetic patients.

Adolescent↗
PubMed1994

Kappa/lambda ratio as parameter for evaluation of therapy-resistant epilepsy of childhood.

In a prospective study we tested the hypothesis whether an alteration in the immunoglobulin light chain kappa and lambda is an expression of an autoimmune mechanism. We investigated 152 children (84 boys, 68 girls, age 12 months to 16 years). Immunoglobulins were analyzed by kinetic nephelometry. The patients were grouped according to clinical and laboratory parameters. A high kappa/lambda ratio caused by high concentration of the kappa-chain was found in children with therapy-resistant epilepsy. 58% showed a high kappa/lambda ratio, in 28% no distinct association was possible and in 14% false-positive results were obtained. In seizure-free children immunological markers were specific in 71%, whilst 25% could not be characterized and 4% showed false-positive results. This alteration of immunoglobulins therefore can be used as a diagnostic parameter.

Adolescent↗
PubMed1994

Comparison of spinal and epidural anesthesia for patients younger than 50 years of age.

To evaluate the respective clinical value of spinal anesthesia with 24-gauge Sprotte needles and epidural anesthesia for younger subjects, 202 patients younger than 50 yr were assigned randomly to undergo one of these two techniques for orthopedic, vascular, urologic, or plastic surgery. Failed blocks occurred in 5% in each group. Spinal anesthesia resulted in significantly less time to achieve sufficient spread of block; a significantly lower incidence of incomplete sensory block at level L5/S1, incomplete motor block, and pain during surgery; and a significantly lower incidence of postlumbar puncture backache (11% vs 30% after epidural anesthesia). The incidence of postdural puncture headache (PDPH) in the spinal and epidural groups was 7% and 4%, respectively (P = not significant), and patient satisfaction was 97% and 93% (P = not significant). Our results demonstrate the effectiveness of both techniques in younger patients, but show that the spinal technique is associated with fewer limitations, suggesting that factors other than PDPH should be considered when choosing between these two techniques.

Adolescent↗
PubMed1994

Moderate exercise improves glucose metabolism in uncontrolled elderly patients with non-insulin-dependent diabetes mellitus.

Exercise should be an integral part of the treatment in non-insulin-dependent (NIDDM) diabetic patients, yet most of these patients' performance is low, mainly because of their obesity and concomitant macrovascular disease. We studied the influence of a moderate exercise training on parameters of glucose control in NIDDM patients. Forty patients aged 56.6 +/- 6.6 years were assigned randomly according to age and sex into exercise and control groups. The exercise group trained for 45 min 3 times weekly for 12 weeks, while the control group did not change their lifestyle. At the end of the study the exercise group had a significant reduction in plasma levels of triglycerides, fructosamine and glycohemoglobin. The improvement in metabolic control persisted significantly in patients who continued to exercise at varying levels at home during 1 year of follow-up.

Aged↗
PubMed1993

Multipoint mapping of the central core disease locus.

A linkage analysis with 12 DNA markers from proximal 19q was performed in eight families with central core disease (CCO). Two-point analysis gave a peak lod score of Z = 4.95 at theta = 0.00 for the anonymous marker D19S190 and of Z = 2.53 at theta = 0.00 for the ryanodine receptor (RYR1) candidate gene. Multipoint linkage data place the CCO locus at 19q13.1, flanked proximally by D19S191/D19S28 and distally by D19S47. This map location includes the RYR1 gene. The results of the linkage study present no evidence for genetic heterogeneity of CCO.

Animals↗
PubMed1993

Quantitative EEG before and after open heart surgery in children. A significant decrease in the beta and alpha 2 bands postoperatively.

Quantitative EEGs of 30 patients undergoing open heart surgery were investigated before, 6 days, 11 days and 44 days after operation. The study was conducted in order to investigate whether quantitative EEGs can show postoperative changes in children after open heart surgery. In 28 children, no new neurological signs of cerebral involvement were seen postoperatively. The most striking feature in these children was a significant decrease in the beta and the alpha 2 bands 6 and 11 days postoperatively. The pattern in the delta band was dominated by an increase 6 days postoperatively. Except for a slight decrease in alpha 2 waves, all variables were restored to preoperative values at 44 days after the operation. We found a significant decrease in plasma sodium and chloride after surgery but children with no or slight declines (1-3 mmol/l) also showed increased slow activity postoperatively. Therefore we conclude that an organic brain syndrome, although usually mild and transient, is a general phenomenon after open heart surgery.

Adolescent↗
PubMed1993

Occurrence of Andermann syndrome out of French Canada--agenesis of the corpus callosum with neuronopathy.

We report on two siblings, a boy and a girl, with agenesis of corpus callosum and neuronopathy. The children show diffuse hypotonia, delayed motor and mental development. Neurophysiological examinations revealed reduction of the motor nerve conduction velocity, absence of sensory nerve action potentials, abnormal somatosensory and visual evoked potentials. Nerve biopsies showed reduced density of myelinated and unmyelinated fibres in both children. We also found signs of hypomyelination and suggest this is secondary to degeneration of peripheral sensory and motor neurons. Our findings are consistent with the diagnosis of Andermann syndrome. This is the first report of the occurrence of Andermann syndrome out of French Canada.

Agenesis of Corpus Callosum↗
PubMed1993

The incidence of Duchenne muscular dystrophy in eastern Austria. The controversy regarding CK screening.

The incidence of Duchenne muscular dystrophy (DMD) in Eastern Austria was investigated. From 1968-1983 302,643 boys were born, 68 of whom were subsequently identified as having Duchenne muscular dystrophy. The overall incidence with respect to live male births is 1:4451 (22.5 x 10(-5)). The mean age of the children at the occurrence of the first symptoms was 3.1 (SD 1.1), at first presentation to a physician 4.8 (SD 2.0), and at diagnosis 5.5 (SD 1.7) years. DMD occurred in 56 families. These families had 73 boys with DMD, 5 of whom were born before or after the observation period. Thirteen families had two affected boys and two families even had three. In 4 families a second boy suffering from DMD was born although the diagnosis of DMD had already been established in another close male relative. If a newborn male screening system had been in function, 13 cases could possibly have been avoided. However, since in 3 cases the affected siblings were not both born within the observation period, they were excluded, leaving 10 cases. Hence, a prevention rate of 14.7% might have been achieved through creatine kinase (CK) screening and genetic counselling.

Austria↗
PubMed1993

A radiation hybrid map of the distal short arm of human chromosome 11, containing the Beckwith-Wiedemann and associated embryonal tumor disease loci.

We describe a high-resolution radiation hybrid (RH) map of the distal short arm of human chromosome 11 containing the Beckwith-Wiedemann gene and the associated embryonal tumor disease loci. Thirteen human 11p15 genes and 17 new anonymous probes were mapped by a statistical analysis of the cosegregation of markers in 102 rodent-human radiation hybrids retaining fragments of human chromosome 11. The 17 anonymous probes were generated from lambda phage containing human 11p15.5 inserts, by using ALU-PCR. A comprehensive map of all 30 loci and a framework map of nine clusters of loci ordered at odds of 1,000:1 were constructed by a multipoint maximum-likelihood approach by using the computer program RHMAP. This RH map localizes one new gene to chromosome 11p15 (WEE1), provides more precise order information for several 11p15 genes (CTSD, H19, HPX, ST5, RNH, and SMPD1), confirms previous map orders for other 11p15 genes (CALCA, PTH, HBBC, TH, HRAS, and DRD4), and maps 17 new anonymous probes within the 11p15.5 region. This RH map should prove useful in better defining the positions of the Beckwith-Wiedemann and associated embryonal tumor disease-gene loci.

Animals↗
PubMed1992

Increased hypothalamic [3H]flunitrazepam binding in hypothalamic-pituitary-adrenal axis hyporesponsive Lewis rats.

We have previously demonstrated that susceptibility of Lewis (LEW/N) rats to inflammatory disease, compared to relatively resistant Fischer (F344/N) rats, is related to deficient glucocorticoid counter-regulation of the immune response resulting from deficient corticotropin-releasing hormone (CRH) responsiveness to inflammatory and other stress mediators. The GABA/benzodiazepine receptor complex is an important negative modulator of CRH secretion and responsiveness to excitatory stimuli. In this study, we have examined in vitro binding of [3H]flunitrazepam to hypothalamic membrane preparations from LEW/N and F344/N rats. LEW/N rats had significantly more hypothalamic benzodiazepine binding sites (Bmax) than F344/N rats, but there were no differences in benzodiazepine binding affinities (Kd) between these two strains. The differences in benzodiazepine receptor number were consistent with the respective plasma corticosterone levels in the two strains, and with previous work indicating a negative correlation between corticosterone levels and benzodiazepine binding site number. Adrenalectomy of F344/N rats increased benzodiazepine binding to levels comparable to LEW/N animals and treatment of adrenalectomized F344/N rats with DEX resulted in lowering of benzodiazepine Bmax to levels that did not differ significantly from those of intact F344/N rats. There was no significant change in receptor number in either adrenalectomized or DEX-treated LEW/N rats. These findings suggest that basal benzodiazepine receptor differences between these strains may be partially related to strain differences in corticosterone levels, however that additional factors may contribute to maintenance of these differences in LEW/N rats. Since benzodiazepines attenuate hypothalamic CRH secretion through GABAergic inhibition, we suggest that strain differences in receptor number could also augment strain differences in hypothalamic-pituitary-adrenal axis function through differential sensitivity to GABA-mediated feedback.

Adrenalectomy↗
PubMed1990

[Computerized tomography in epilepsy in children].

We present a retrospective study of 160 children attending our epilepsy out-patient department. Computed tomography (CT) was performed on 123 (77%) patients, namely all children with the exception of those with febrile seizures, typical absence seizures, and benign Rolandic epilepsy. Incidence of CT abnormalities and their correlation with clinical features were evaluated. The CT scan was normal in 84 and abnormal in 39 patients. Although in general, the detection of abnormality on CT will not alter the management of the child, in a small percentage (2%) of our cases a lesion treatable by surgery was discovered. The indications for CT are summarized.

Anticonvulsants↗
PubMed1987

[Circulatory distribution of intravenously injected metallic mercury].

The mode of distribution and sites of deposition of mercury are discussed on the basis of the example of a 21-year old female nurse who had injected 4.5 ml of metallic mercury into her left cubital vein with suicidal intent, the findings being compared with the reports by 11 other authors. In particular, the influence of gravity on the distribution in the organism is emphasised. The proof of the decrease of an intrapulmonarily deposited amount of mercury within the observation period of 14 months furnishes ample evidence of the importance of a chronic absorptive mercury intoxication.

Adult↗