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Biomedical subjects

P Uvebrant

Publications and source records attributed to P Uvebrant.

At least 19 recordsLinked to original sources

[Surgical treatment is efficient in epilepsy].

Epilepsy surgery has a very long tradition, and recent advances in diagnostic and surgical procedures have enabled a number of patients with drug-resistant epilepsy to be treated successfully. In addition to the conventional clinical work-up, candidates for epilepsy surgery undergo evaluation by a multidisciplinary team using a battery of neuroimaging and neurophysiological procedures. Such teams have been established at all six university hospitals in Sweden.

Epilepsy

Dichotic-listening performance and intracarotid injections of amobarbital in children and adolescents. Preoperative and postoperative comparisons.

BACKGROUND: Dichotic listening (DL) to consonant-vowel syllables is frequently used in clinical and experimental studies of brain laterality. However, the paradigm of consonant-vowel syllables has not been thoroughly validated through a comparison with injections of amobarbital sodium (Amytal). OBJECTIVE: To validate the DL test for hemisphere dominance preoperatively vs postoperatively with the results from intracarotid injections of amobarbital (i.e., the Wada test) in epileptic children and adolescents. DESIGN AND MAIN OUTCOME MEASURES: Patients were tested with DL preoperatively and at 6-month follow-up. Correct reports in the DL tests were entered in a stepwise discriminant analysis for calculation of correct classification of hemisphere dominance with the results from the injections of amobarbital as the grouping variable. Correct reports from the right and left ears on the consonant-vowel DL test were compared preoperatively and postoperatively, separated for the subjects with regard to language dominance in the left and right hemispheres. SETTING: The Department of Pediatrics, Ostra Hospital, University of Göteborg, Göteborg, Sweden. PATIENTS: Thirteen children and adolescents between the ages of 10 and 19 years, who were surgically treated for resistant epilepsy, were included in the study. The operated area corresponded with morphological changes and functional dysfunctions according to findings from computed tomography, magnetic resonance imaging, single photon emission computed tomography, and electroencephalography. RESULTS: The results of the Wada tests revealed that 10 subjects had left hemisphere language dominance, with 3 subjects having right hemisphere language dominance. All 3 subjects with right hemisphere language dominance showed a left ear advantage on the DL test preoperatively and postoperatively, with 8 and 7 of the 10 subjects with left hemisphere dominance showing a right ear advantage, preoperatively and postoperatively, respectively. However, according to discriminant analysis, knowledge of the DL performance led to a correct classification according to the Wada test results in 12 (92%) of the 13 subjects. CONCLUSIONS: A quantitative classification procedure like discriminant analysis may be more sensitive when predicting hemisphere speech dominance from DL data than a qualitative procedure based on the ear advantage dichotomy. The ear advantage dichotomy may actually introduce arbitrary left-right categories that do not correspond to the actual clustering of the data.

Adolescent

Motor and sensory dysfunctions in children with mental retardation and epilepsy.

The aim of this study was to assess motor and sensory functions in a population-based series of 88 mentally retarded children with epilepsy. A new standardized physiotherapy protocol was developed for the heterogeneous population of children with epilepsy; the Cailler-Azusa scale was also found to be useful. For children with cerebral palsy, the gross motor function measure was used. Sensorimotor impairments, resulting in disabilities and handicap, were found to be very common but often overlooked and neglected. Sensorimotor dysfunctions need to be identified in order to provide rational training, understanding and care to children with epilepsy and mental retardation.

Cerebral Palsy

Neuronal ceroid lipofuscinoses in Scandinavia. Epidemiology and clinical pictures.

The epidemiology of neuronal ceroid lipofuscinoses (NCL) in Scandinavia was studied. For juvenile NCL 40 Swedish living patients were identified. The corresponding number for Finland was 61, for Norway 28, for Denmark 16 and for Iceland three. The prevalence of juvenile NCL was thus 4.6, 12.2, 6.5, 3.1 and 11 per million inhabitants in Sweden, Finland, Norway, Denmark, and Iceland, respectively. For calculating incidence the years 1976-85 were used. The incidence was 2.2 per 100,000 live births in Sweden, 4.8 in Finland, 3.7 in Norway, 2.0 in Denmark, and 7.0 in Iceland. Late infantile NCL was found in five Swedish children, including one variant form, CLN5. This gives a prevalence of about 0.6 per million. In Finland 13 cases gave a prevalence of 2.6 per million with the variant form in 80% of cases. In Norway, three children corresponded to a prevalence of 0.7 per million and one case in Iceland to 3.8 per million. No Danish cases were reported. As for infantile NCL, sixteen Swedish cases have been diagnosed during the 27-year period 1968-95, six are presently alive. This gives an estimated prevalence of 0.7 per million and an incidence of 0.6 per 100,000. The prevalence and incidence of infantile NCL in Finland were 5.4 per million and 5 per 100,000, respectively. The prevalence in Norway was 0.2 per million inhabitants. The variability of onset, clinical course and symptoms in the Swedish cases of juvenile and infantile NCL were analysed.

Adolescent

Hidden dysfunction in childhood epilepsy.

Sensory and motor function were evaluated in 84 children and adolescents with epilepsy but not additional neuroimpairments such as mental retardation or cerebral palsy. The influence of sex, age, age at onset of epilepsy, duration, seizure type and frequency and medication was analysed. The Bruininks-Oseretsky Test of Motor Proficiency and the EB (Eva Beckung) test were used. We found significant dysfunction in gross as well as in fine motor functions. The most obvious deviation from normal was noted in gross motor function, mainly in the areas of running speed, balance, response speed and bilateral coordination, but also in upper-limb speed, dexterity and coordination. Boys performed better than girls. Motor function scores improved with age but sensory and motor problems persisted in the teenage years. Early onset and a long duration of the epilepsy were associated with significantly more sensory and motor difficulties. The children on monotherapy performed significantly better than the children on polytherapy and, surprisingly, also better than those without antiepileptic medication. Sensory and motor function should be carefully investigated in the context of a comprehensive rehabilitation programme for children with epilepsy.

Adolescent

Computed tomography and single-photon emission computed tomography as diagnostic tools in acquired brain injury among children and adolescents.

Twenty children with acquired brain injuries were investigated with CT and SPECT. The findings were related to the clinical outcome judged at discharge following acute care after the injury and at follow-up 5 years later. The abnormalities that were found were classified for each lobe on a scale ranging from 0 (normal) to 5 (severe abnormality). The patients were divided into two groups showing mild and severe injury. CT and SPECT revealed similar results in the patients with severe injury, but in the group of mildly injured children the number of affected lobes and scores indicated by SPECT were significantly higher than those indicated by CT. SPECT also differentiated more effectively between the two outcome groups. This supports the hypothesis that SPECT could be used as an instrument to objectivise minor sequelae and that SPECT and CT are both useful methods for forecasting outcome.

Adolescent

Surgical treatment for epilepsy: a retrospective Swedish multicenter study.

The characteristics of patients suffering from drug resistant epilepsy, including the results of the preoperative evaluation and epilepsy surgery were retrospectively analyzed in a Swedish multicenter 10-year cohort of children and adults. Altogether 152 patients (65 children and 87 adults) treated during the period 1980-1990 in three epilepsy centers were included and followed-up 2 years after surgery. Median age at onset of seizures was 4 years for the children and 12 years for the adults. A localization related epilepsy was present in 85% of the children and in 95% of the adults. The mean number of seizure types in the children was 1.7 (range 1-4) and in the adults 1.8 (range 1-4). The median monthly seizure frequency was 52 and 15 for children and adults respectively. Resective surgery was performed in 143 cases (94 temporal, 31 extratemporal, 9 multilobar and 9 major resection procedures) and palliative procedures in 16 cases (13 callosotomies and 3 stereotactic amygdalotomies). Postoperative neurological deficits were detected in 9% of the patients after temporal lobe resections and in 15% of the patients after extratemporal and multilobar resection procedures. Two years after resective surgery 53% of the children and 49% of the adults were seizure free. Another 25% of the patients had a more than 50% reduction of seizure frequency. In the postoperative non seizure free group of patients there was a negative correlation between decrease in weighted seizure severity and decrease in seizure frequency. This finding stresses the need for including other parameters than seizure frequency when evaluating the outcome of epilepsy surgery.

Adolescent

Impairments, disabilities and handicaps in children and adolescents with epilepsy.

The patterns of motor and sensory impairments, disabilities and handicap were assessed in 217 children and adolescents with epilepsy. Motor and sensory dysfunctions were found to be common even in children without major additional neurodisorders such as cerebral palsy and mental retardation. Handicap was most severe in the dimensions of physical independence and orientation and increased significantly with duration of epilepsy. It was more severe when the onset of seizures was early and when secondary generalized seizures were present. Handicap was significantly reduced after epilepsy surgery.

Adolescent

Regional cerebral blood flow abnormalities in Rett syndrome.

Sixteen Rett syndrome (RS) girls and 1 24 year old RS woman were compared with 16 neurologically healthy children as to the regional cerebral blood flow (rCBF). Hypoperfusion of the frontal lobes and parts of the midbrain was found in RS, with significant differences to controls at latest by the age of three to four years. The findings were in accordance with previous reports of morphological, neuroradiological and functional abnormalities in these areas, compatible with a statement that structures in the midbrain and in the frontal lobes may have particular pathophysiological relevance in RS.

Adolescent

Disorders of the cerebral white matter in children. The spectrum of lesions.

The use of magnetic resonance imaging (MRI) has resulted in the detection of an increasing number of children with an apparently leukodystrophic white matter. Laboratory tests and the clinical presentation, however, often do not correspond to any known entity and the course is sometimes not progressively deteriorating. Such children with white-matter changes and no known diagnosis were the subject of this Swedish multicentre study, in which MRI findings and clinical data from 100 children considered to have white-matter abnormalities were assessed during the period 1992-1995. At re-evaluation of MR images by an established "white-matter group" of neuroradiologists, paediatric neurologists, neurologists and neurochemists, the MRI signal of the white matter was considered normal in eleven children and eleven had mainly a grey matter affection. Of the remaining 78 children with white matter abnormalities, a diagnosis was found in 32, but in 46 children no diagnosis could be established. A progressive downhill course characterised 17, probably representing hitherto undefined types of leukodystrophies. Five children had a relapsing-remitting course, and in 11 it was difficult to establish whether the course was progressive or stationary. The disease was non-progressive in 13. This group of non-leukodystrophic white-matter changes obviously represents maldevelopments of myelin formation, thus dys- or hypomyelination rather than demyelination.

Adolescent

Autism and epilepsy (and tuberous sclerosis?) in two pre-adolescent boys: neuropsychiatric aspects before and after epilepsy surgery.

We report on two pre-adolescent boys with a combination of severe seizure disorders and severe-moderate autism who underwent brain surgery for their epilepsy at the ages of 9 and 10 years, respectively. Both boys became seizure-free and initially improved dramatically with regard to autism symptoms. One of the boys continued to improve, but the other had a relapse to his pre-operative state in conjunction with his pubertal growth spurt. Several years after surgery, one of the boys remained much improved with respect to his autism. The other subject showed some improvement with respect to self-injury and aggression, and had slightly lower scores on screens for autism symptoms than in the year preceding epilepsy surgery. The histopathological examination of the brain tissue that was removed at surgery suggested a diagnosis of tuberous sclerosis in both cases.

Autistic Disorder

The light-flash-evoked response as a possible indicator of increased intracranial pressure in hydrocephalus.

Surgical treatment of infantile hydrocephalus by shunt implantation may result in suboptimal intracranial pressure. Major neurological impairments and death are usually prevented by shunt treatment, but minor sequelae may persist or develop. The introduction of adjustable shunts has improved the possibilities of optimizing shunt function and minimizing the risk of such impairments. However, it is still impossible to determine the intracranial pressure without invasive measurements. Clinical findings and procedures such as computed tomography (CT) are not always enough to allow a conclusion as to whether a child's signs and symptoms are the result of suboptimal intracranial pressure (shunt dysfunction) or are of another etiology. With the aim of reducing the number of invasive pressure measurements and CT scans, we investigated the effect of increased intracranial pressure on the visual evoked response (VER). Binocular light flash stimuli of supramaximal intensity were used and VER recordings were performed from Oz and Cz. The VER results from a group of 31 infants and children with hydrocephalus and 2 children with pseudotumor cerebri were compared with responses from a control group of 35 healthy children. The results show that a subpotential, P' (P-prime), usually just preceding P1 (P100), had an increased latency ( > 96 ms) in all hydrocephalic children before surgery. The P' latency in this group was usually even above 110 ms. The latencies of other VER potentials were also increased but not as consistently as P'. After surgical intervention the VER latencies decreased and usually normalized. The P' latency in four children in the control group was just above the borderline latency, but was less than 110 ms.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent

Dendritic morphology in epileptogenic cortex from TRPE patients, revealed by intracellular Lucifer Yellow microinjection and confocal laser scanning microscopy.

Biopsy material was obtained from cortical epileptogenic zones (eight temporal, one occipital, one parietal and one frontal) of eleven patients aged 1.5-47 years with therapy-resistant partial epilepsy (TRPE) undergoing epilepsy surgery. Control autopsy material (two temporal, two occipital, one parietal and one frontal) was removed from six neurologically healthy cases within 6-10 hours postmortem delay. In each specimen, 100-300 pyramidal and non-pyramidal neurons were visualized by intracellular Lucifer Yellow microinjection. Single neurons were imaged using CLSM generated serial optical sections; 2-D reconstruction of each neuron was made using z-projection of serial optical images, and 3-D reconstructions and rotations were computerized. Neuronal maps from TRPE biopsies, compared to control autopsies, show markedly increased numbers of dendritic abnormalities of single pyramidal and non-pyramidal neurons in layers I, II-III, V-VII, and in the subcortical white matter. The abnormalities include: (1) increased number of non-pyramidal cells in layer I; (2) many pyramidal cells with two or three dendrites originating apically, rather than one single apical dendrite, in layers II-III; (3) atypical orientation of oblique apical and basal dendrites in pyramidal neurons of layers II-VII; (4) increased number of atypical 'dinosaur-like' and fusiform cells in layers V-VII; (5) numerous neurons in the white matter. These abnormalities may be etiological in cases with early onset, and predisposing in cases with late onset.

Adolescent

Intractable epilepsy in children. The efficacy of lamotrigine treatment, including non-seizure-related benefits.

Fifty children and adolescents with intractable epilepsy were treated with lamotrigine. Most of the children had other neuro-impairments, such as mental retardation, cerebral palsy and autism, added to their epilepsy. Five stopped lamotrigine treatment within four months because of side effects. In the 45 children who continued treatment for a mean of 14 months, five became seizure-free and in 16 the seizure frequency was reduced more than 30 percent. Absences and complex partial seizures responded best. In 24 of the 45 children, the parents reported an improvement in the mental state of their child, with better contact, longer attention span and improved alertness. In eight of 13 autistic children, the autistic symptoms decreased during lamotrigine treatment. This also occurred in children with an unchanged seizure situation, indicating a specific positive psychotropic effect of lamotrigine in mentally retarded and autistic children.

Adolescent

The effects of epilepsy surgery on the sensorimotor function of children.

The motor and sensory functions of 50 children were investigated before and six months after epilepsy surgery; 34 infants were assessed 24 months after surgery. Postoperatively, 20 children were seizure-free and 22 had a significant reduction of seizures. Epilepsy surgery was found to be an effective mode of treatment for intractable seizures in childhood, even in multiply handicapped individuals. Motor and sensory functions did not deteriorate after surgery; in fact, significant improvements were found in more than half of the children, including those with multiple handicaps. Improvements were most obvious in the seizure-free group, but were also noted in those with reduced seizure frequency. The younger children benefited more from surgery as regards sensorimotor function than did older children and adolescents.

Adolescent

Verbal and non-verbal function of children with right- versus left-hemiplegic cerebral palsy of pre- and perinatal origin.

Eighteen children with right- and 13 with left-sided congenital hemiplegia were compared with 19 normal age-matched controls for verbal and non-verbal function. CT scans were obtained from 27 of the 31 hemiplegic children. The two hemiplegic groups were impaired in non-verbal function compared with controls. The right-hemiplegic group was more impaired in verbal function than the left-hemiplegic group and controls; however, impairments were restricted to the girls in the right-hemiplegic group. The results are discussed in terms of cerebral plasticity and functional reorganisation of cognitive functions after an early unilateral injury. It is argued that girls with left-hemisphere lesions may be more limited in cerebral plasticity than boys.

Adolescent