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Biomedical subjects

S F Berkovic

Publications and source records attributed to S F Berkovic.

At least 19 recordsLinked to original sources
PubMed1997

Retrospective study of concussive convulsions in elite Australian rules and rugby league footballers: phenomenology, aetiology, and outcome.

OBJECTIVES: To study the ictal phenomenology, aetiology, and outcome of convulsions occurring within seconds of impact in violent collision sport. DESIGN: Retrospective identification of convulsions associated with concussive brain injury from case records from medical officers of football clubs over a 15 year period. SUBJECTS: Elite Australian rules and rugby league footballers. MAIN OUTCOME MEASURES: Neuroimaging studies, electroencephalography, neuropsychological test data, and statistics on performance in matches to determine presence of structural or functional brain injury. Clinical follow up and electroencephalography for evidence of epilepsy. RESULTS: Twenty two cases of concussive convulsions were identified with four events documented on television videotape. Convulsions began within 2 seconds of impact and comprised an initial period of tonic stiffening followed by myoclonic jerks of all limbs lasting up to 150 seconds. Some asymmetry in the convulsive manifestations was common, and recovery of consciousness was rapid. No structural or permanent brain injury was present on clinical assessment, neuropsychological testing, or neuroimaging studies. All players returned to elite competition within two weeks of the incident. Epilepsy did not develop in any player over a mean (range) follow up of 3.5 (1-13) years. CONCLUSIONS: These concussive or impact convulsions are probably a non-epileptic phenomenon, somewhat akin to convulsive syncope. The mechanism may be a transient traumatic functional decerebration. In concussive convulsions the outcome is universally good, antiepileptic treatment is not indicated, and prolonged absence from sport is unwarranted.

Adult↗
PubMed1997

Hippocampal atrophy is not a major determinant of regional hypometabolism in temporal lobe epilepsy.

PURPOSE: The pathophysiologic basis for the [18F]fluorodeoxyglucose positron-emission tomography (FDG-PET) temporal lobe hypometabolism in patients with hippocampal sclerosis (HS) is uncertain. We tested the hypothesis that hippocampal atrophy, which is strongly correlated with hippocampal cell loss, is largely responsible for the regional hypometabolism in HS. METHODS: Regions of interest (ROIs) on FDG-PET scanning were determined in the medial, lateral, and posterior temporal lobe, thalamus, and basal ganglia. A right/left asymmetry index for each ROI was calculated. These results were correlated with hippocampal magnetic resonance imaging (MRI) volume ratios. RESULTS: There was no correlation between the magnitudes of the FDG-PET asymmetry index and the MRI volume ratio for the mesial or lateral temporal regions (r = -0.09, r = -0.04). When the right/left asymmetry index was compared with the right/left hippocampal volume ratio, correlations for the mesial temporal ROI (r = 0.79, p < 0.0001) and lateral temporal ROI (r = 0.57, p < 0.0005) were found. These, however, simply indicated that both tests accurately reflect the side of the epileptogenic region. The concordance of the side of relative hypometabolism of the FDG-PET with the side of the hippocampal atrophy was higher for the mesial temporal region (100%) than for the lateral (77.5%). CONCLUSIONS: The lack of correlation between the magnitudes of the ratios argues against hippocampal atrophy and cell loss having a central role in the FDG-PET temporal hypometabolism.

Adult↗
PubMed1996

Familial temporal lobe epilepsy: a common disorder identified in twins.

We describe a new syndrome of familial temporal lobe epilepsy in 38 individuals from 13 unrelated white families. The disorder was first identified in 5 concordant monozygotic twin pairs as part of a large-scale twin study of epilepsy. When idiopathic partial epilepsy syndromes were excluded, the 5 pairs accounted for 23% of monozygotic pairs with partial epilepsies, and 38% of monozygotic pairs with partial epilepsy and no known etiology. Seizure onset for twin and nontwin subjects usually occurred during adolescence or early adult life. Seizure types were simple partial seizures with psychic or autonomic symptoms, infrequent complex partial seizures, and rare secondarily generalized seizures. Electroencephalograms revealed sparse focal temporal interictal epileptiform discharges in 22% of subjects. Magnetic resonance images appeared normal. Nine affected family members (24%) had not been diagnosed prior to the study. Pedigree analysis suggested autosomal dominant inheritance with age-dependent penetrance. The estimated segregation ratio was 0.3, indicating an overall penetrance of 60% assuming autosomal dominant inheritance. The mild and often subtle nature of the symptoms in some family members may account for lack of prior recognition of this common familial partial epilepsy. This disorder has similarities to the El mouse, a genetic model of temporal lobe epilepsy with a major gene on mouse chromosome 9, which is homologous with a region on human chromosome 3.

Adolescent↗
PubMed1996

Periventricular heterotopia: an X-linked dominant epilepsy locus causing aberrant cerebral cortical development.

Periventricular heterotopia (PH) involves dramatic malformations of the human cerebral cortex. Here we show that PH is closely linked to markers in distal Xq28 (maximal two-point lod score = 4.77 for F8C at theta = 0; maximal multipoint lod score = 5.37), so that affected females are obligatory mosaics for the mutation; that PH is lethal to at least some affected males; that PH malformations consist of well-differentiated cortical neurons filling the adult subependymal zone; and that individuals with PH are at high risk for epilepsy, though they have no other neurological or external stigmata. The PH gene may represent an important epilepsy susceptibility locus in addition to playing a key role in normal cortical development.

Abortion, Habitual↗
PubMed1996

A double-blind, placebo-controlled crossover study of vigabatrin 2 g/day and 3 g/day in uncontrolled partial seizures.

The efficacy and tolerability of vigabatrin as add-on therapy was assessed in patients with uncontrolled partial seizures. Ninety-seven patients entered this seven-centre, double-blind, placebo-crossover study. Vigabatrin (2 g or 3 g) or placebo was administered daily. Vigabatrin was well-tolerated and did not cause clinically significant adverse drug effects when added to established anticonvulsant therapy. No significant differences were observed between dose groups for the overall incidence of adverse events, although drowsiness and visual disturbances (diplopia, ataxia, visual abnormalities) showed a dose-related increase with vigabatrin treatment. The results of this study indicate that vigabatrin, given in a daily dose of either 2 g or 3 g is significantly more effective than placebo in reducing seizure frequency among patients with partial seizures.

Adolescent↗
PubMed1996

Does naming contribute to memory self-report in temporal lobe epilepsy?

The present study evaluated the hypothesis (Mayeux et al., 1980) that visual confrontation naming deficits may underlie the memory complaint in patients with temporal lobe epilepsy (TLE). Thirty-nine patients with medically refractory left (n = 23) and right (n = 16) TLE were compared with an epilepsy control group with idiopathic primary generalized epilepsy (n = 38). All subjects completed selected subtests of the Multilingual Aphasia Examination and Wechsler Memory Scale (Form 1) together with a measure specifically designed for quantification of the memory complaint in TLE. Objective verbal memory test performance, confrontation naming, repetition, and comprehension were unrelated to memory self-report. Controlled Oral Word Association was the only measure to exert an influence on memory self-ratings, and this relationship was specific to the TLE group. The hypothesis of Mayeux et al. (1980) was not specifically supported, but the present findings do suggest that cognitive processes reflected in orthographically based and internally generated word retrieval play a role in memory self-report.

Adult↗
PubMed1996

Temporal lobe epilepsy subtypes: differential patterns of cerebral perfusion on ictal SPECT.

PURPOSE: We studied cerebral perfusion patterns in the various subtypes of TLE, as determined by pathology and good outcome after temporal lobectomy (as confirmation of temporal origin). METHODS: We studied clinical features and ictal technetium 99m hexamethyl-propyleneamineoxime (99mTc-HM-PAO) single-photon emission-computed tomography (SPECT) in four subgroups of patients with intractable temporal lobe epilepsy (TLE) treated with surgery: hippocampal sclerosis (group 1, n = 10), foreign-tissue lesion in mesial temporal lobe (group 2, n = 8), foreign-tissue lesion in lateral temporal lobe (group 3, n = 7), and normal temporal lobe tissue with good surgical outcome (group 4, n = 5). RESULTS: No major clinical differences in auras, complex partial seizures or postictal states were identified among the groups. Ictal SPECT showed distinct patterns of cerebral perfusion in these subtypes of TLE. In groups 1 and 2, hyperperfusion was seen in the ipsilateral mesial and lateral temporal regions. In group 3, hyperperfusion was seen bilaterally in the temporal lobes with predominant changes in the region of the lesion. Hyperperfusion was restricted to the ipsilateral anteromesial temporal region in group 4. Ipsilateral temporal hyperperfusion in mesial onset seizures can be explained by known anatomic projections between mesial structures and ipsilateral temporal neocortex. Bilateral temporal hyperperfusion in lateral onset seizures can be explained by the presence of anterior commissural connections between lateral temporal neocortex and the contralateral amygdala. CONCLUSIONS: We conclude that the perfusion patterns seen on ictal SPECT are helpful for subclassification of temporal lobe seizures, whereas clinical features are relatively unhelpful. These perfusion patterns provide an insight into preferential pathways of seizure propagation in the subtypes of TLE.

Adolescent↗
PubMed1996

Suggestion of a major gene for familial febrile convulsions mapping to 8q13-21.

Febrile convulsions affect 2 to 5% of all children under the age of 5 years. These convulsions probably have a variety of causes, but a genetic component has long been recognised. A large and remarkable family is described in which febrile convulsions appear to result from autosomal dominant inheritance at a single major locus. A gene for febrile convulsions was excluded from regions of previously mapped epilepsy genes and extension of exclusion mapping, using microsatellite markers, to the entire genome implied that a locus on chromosome 8q13-21 may be involved. Linkage analysis of markers on chromosome 8 gave a multipoint lod score of 3.40, maximised over different values of penetrance and phenocopy rate, for linkage between the gene for febrile convulsions and the region flanked by markers D8S553 and D8S279. This lod score was calculated assuming the disease has a penetrance of 60% and a phenocopy rate of 3%. Although there was no indication of linkage other than to markers on chromosome 8, linkage remains suggestive rather than significant because of the maximisation procedure applied. The support for linkage involving a major gene, as opposed to an alternative hypothesis of a complex inheritance pattern, relied upon the assumption of low penetrance.

Adolescent↗
PubMed1996

Plasmapheresis in Rasmussen's encephalitis.

Rasmussen's encephalitis (RE) is a progressive childhood disorder characterized by intractable focal seizures, hemiplegia, dementia, and inflammatory histopathology. The process is typically limited to one cerebral hemisphere. We report four patients with pathologically confirmed RE who were treated with repeated plasmapheresis. Three patients exhibited repeated, dramatic, transient responses to plasmapheresis, manifested by reduced seizure frequency and improved neurologic function. One patient exhibited marginal improvement after treatment with plasmapheresis. These observations indicate that circulating factors, likely autoantibodies, are pathogenic in at least some patients with RE and suggest that RE is an autoimmune disease. Plasmapheresis may be a useful adjunctive therapy in status epilepticus, and can also aid in assessment of residual function in the diseased hemisphere before surgical resection.

Adolescent↗
PubMed1995

Spatiotemporal alterations of central alpha 1-adrenergic receptor binding sites following amygdaloid kindling seizures in the rat: autoradiographic studies using [3H]prazosin.

Noradrenergic neurons are thought to be involved in the process of seizure development and long-term central nervous system plasticity associated with kindling and epilepsy. These processes involve actions of noradrenaline at alpha 1-, alpha 2- and beta 1-adrenergic receptors. In this study, quantitative in vitro autoradiography was used to investigate possible changes in the density of brain alpha 1-adrenergic receptors in a kindling model of epilepsy in the rat. Kindling was produced by daily unilateral stimulation of the amygdala. The alpha 1A+alpha 1B subtypes of adrenergic receptors were labelled with the alpha 1-selective antagonist, [3H]prazosin and alpha 1B receptors, detected in the presence of 10 nM WB4101 to selectively occupy alpha 1A receptors, accounted for 50% of total alpha 1 receptors in cerebral cortex. Autoradiographic studies identified significant and long-lasting, ipsilateral increases in specific [3H]prazosin binding throughout layers I-III of the cortex in sham-operated, unstimulated rats, presumably caused by the surgical implantation of the stimulating electrode within the basolateral amygdaloid nucleus. Binding to alpha 1A + alpha 1B receptors and alpha 1B receptors was increased by an average of 35 and 60%, respectively under these conditions. Stimulation-evoked seizures produced dramatic bilateral increases in specific [3H]prazosin binding to alpha 1A + alpha 1B receptors and particularly to alpha 1B receptors in layers I-III of all cortical areas examined. These changes were rapidly induced and the largest increases (range alpha 1A + alpha 1B 80-340%; alpha 1B 165-380%) occurred at 0.5-2 h after the last stage 5 kindled seizure. At 1 and 3 days after the last seizure, increases were measured for both alpha 1A + alpha 1B and alpha 1B receptors in layers I-III of particular cortical regions, but not overall (e.g. 60-210% increase in perirhinal cortex at both times, with increases also in retrosplenial, hindlimb, occipital, parietal and temporal cortices). Between 2-8 wk post-stimulation specific receptor binding levels were equivalent to those in sham-operated, unstimulated rats. In contrast to the large and widespread increases in outer cortical [3H]prazosin binding, smaller increases were detected in the inner cortex (layer V-VI) at individual times (65-75% increase at 30 min), while no significant changes occurred in several other brain regions examined, including thalamus, which contained a high density of alpha 1A and alpha 1B receptors, or hippocampus which has a low density of both alpha 1 receptor subtypes.(ABSTRACT TRUNCATED AT 400 WORDS)

Amygdala↗
PubMed1995

Comparison of ictal SPECT and interictal PET in the presurgical evaluation of temporal lobe epilepsy.

We retrospectively compared ictal technetium 99m hexamethylpropyleneamineoxime single-photon emission computed tomography (SPECT) and interictal 18F-fluorodeoxyglucose positron emission tomography (PET) in 35 patients with well-lateralized temporal lobe epilepsy (TLE). Based on SPECT scans the two observers correctly lateralized seizure foci with certainty in 89% of patients; interobserver agreement was excellent. Both observers incorrectly lateralized the seizure focus on two SPECT scans; one error was explained by rapid electroencephalographic spread to the contralateral side and for the other patient, isotope was injected during a brief aura. Based on PET scans, observers correctly lateralized the foci with certainty in 63% and with lesser confidence in 83%; four incorrect lateralizations were made by one observer and none by the other. PET interobserver disagreement was explained by differences between observers in weighting the relative hypometabolism in medial and lateral temporal regions. The detection rate for PET was lower in the absence of structural imaging abnormalities (60 vs 87%). PET yielded correct lateralizations in the 2 patients for whom SPECT interpretation was difficult. We conclude that both ictal SPECT and interictal PET are sensitive methods for the lateralization of TLE, but SPECT can be interpreted with greater certainty and is more sensitive when magnetic resonance imaging findings are negative. False lateralization is rare with ictal SPECT and can be explained when interpreted in conjunction with electroclinical data. Both investigations have complementary roles when localization is difficult.

Adolescent↗
PubMed1995

Autosomal dominant rolandic epilepsy and speech dyspraxia: a new syndrome with anticipation.

We describe a family of 9 affected individuals in three generations with nocturnal oro-facio-brachial partial seizures, secondarily generalized partial seizures, and centro-temporal epileptiform discharges, associated with oral and speech dyspraxia and cognitive impairment. The speech disorder was prominent, but differed from that of Landau-Kleffner syndrome and of epilepsy with continuous spike and wave during slow-wave sleep. The electroclinical features of this new syndrome of autosomal dominant rolandic epilepsy resemble those of benign rolandic epilepsy, a common inherited epilepsy of childhood. This family shows clinical anticipation of the seizure disorder, the oral and speech dyspraxia, and cognitive dysfunction, suggesting that the genetic mechanism could be expansion of an unstable triplet repeat. Molecular studies on this syndrome, where the inheritance pattern is clear, could also be relevant to identifying a gene for benign rolandic epilepsy where anticipation does not occur and the mode of inheritance is uncertain.

Adult↗
PubMed1995

Intractable partial epilepsy following low-dose scalp irradiation in infancy.

We report the development of intractable epilepsy in 3 patients treated with irradiation to "strawberry" scalp nevi in infancy. Low-dose radiation was used (12 and 13 Gy in 2 of the patients). The clinical evolution suggested a recognizable and distinctive postradiation syndrome. There was concordance between the site of radiation as shown by localized alopecia, the clinical features of the partial seizures, and electrographic abnormalities. The clinical picture was unlike delayed cerebral radiation necrosis of adulthood, which is not thought to occur at doses below 50 Gy, in 2-Gy fractions. Neurological deficits were not progressive and in 2 patients there was no evidence of parenchymal injury on cranial magnetic resonance imaging scanning. These differences suggest pathogenetic differences to cerebral radiation injury of adulthood, probably relating to the interaction between nervous system development, individual susceptibility, and the low doses of radiation employed.

Adolescent↗
PubMed1995

Clinical applications: MRI, SPECT, and PET.

MRI, PET, and SPECT are all used to image abnormalities in the epileptic brain. Comparison of the techniques is difficult because they measure different aspects of the epileptic process--structure, metabolism, and perfusion. SPECT is the only one that can be systematically applied during seizures, while all three are used to image interictal abnormalities. Literature review suggests that of interictal techniques, PET has the highest diagnostic sensitivity in temporal lobe epilepsy (TLE) (84% vs. 66% for SPECT, 55% for qualitative MRI, 71% for quantitative MRI) while SPECT has the highest sensitivity in extratemporal epilepsy (ETE) (60% vs. 43% for MRI and 33% for PET). The highest diagnostic sensitivity and specificity were achieved by ictal imaging with SPECT (90% in TLE, 81% in ETE). The techniques, however, were not always redundant. One reason for the wide discrepancy of results in TLE and ETE might be the differing pathologic substrates. A literature review of imaging findings associated with mesial temporal sclerosis (MTS), developmental lesion or tumor as the underlying abnormality associated with epilepsy supports this explanation. PET and MRI are much more sensitive to MTS than SPECT (100%, 95% vs. 70%). On the other hand, in developmental lesions the three techniques are equally sensitive (88-92%) and in tumors, MRI was most sensitive (96%) and SPECT least (82%). A study at NIH explains the differing sensitivities: using PET to measure both blood flow and metabolism revealed discrepant findings in the same patients. Preliminary evidence also indicates that the distribution of hyperperfusion on ictal SPECT can differentiate subtypes of TLE. Combining the results of refined imaging techniques holds great promise in epilepsy localization and diagnosis.

Brain↗