Rasmussen encephalitis with ipsilateral brain stem involvement in an adult patient.
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Adult↗
Biomedical subjects
C E Elger
Publications and source records attributed to C E Elger.
The effect of word concreteness on recognition memory.
Concrete words that are readily imagined are better remembered than abstract words. Theoretical explanations for this effect either claim a dual coding of concrete words in the form of both a verbal and a sensory code (dual-coding theory), or a more accessible semantic network for concrete words than for abstract words (context-availability theory). However, the neural mechanisms of improved memory for concrete versus abstract words are poorly understood. Here, we investigated the processing of concrete and abstract words during encoding and retrieval in a recognition memory task using event-related functional magnetic resonance imaging (fMRI). As predicted, memory performance was significantly better for concrete words than for abstract words. Abstract words elicited stronger activations of the left inferior frontal cortex both during encoding and recognition than did concrete words. Stronger activation of this area was also associated with successful encoding for both abstract and concrete words. Concrete words elicited stronger activations bilaterally in the posterior inferior parietal lobe during recognition. The left parietal activation was associated with correct identification of old stimuli. The anterior precuneus, left cerebellar hemisphere and the posterior and anterior cingulate cortex showed activations both for successful recognition of concrete words and for online processing of concrete words during encoding. Additionally, we observed a correlation across subjects between brain activity in the left anterior fusiform gyrus and hippocampus during recognition of learned words and the strength of the concreteness effect. These findings support the idea of specific brain processes for concrete words, which are reactivated during successful recognition.
Adult↗
Sensory gating of auditory evoked and induced gamma band activity in intracranial recordings.
Oscillatory activity in the gamma band range (30-50 Hz) and its functional relation to auditory evoked potentials (AEPs) is yet poorly understood. In the current study, we capitalized on the advantage of intracranial recordings and studied gamma band activity (GBA) in an auditory sensory gating experiment. Recordings were obtained from the lateral surface of the temporal lobe in 34 epileptic patients undergoing presurgical evaluation. Two kinds of activity were differentiated: evoked (phase locked) and induced (not phase locked) GBA. In 18 patients, an intracranial P50 was observed. At electrodes with maximal P50, evoked GBA occurred with a similar peak latency as the P50. However, the intensities of P50 and evoked GBA were only modestly correlated, suggesting that the intracranial P50 does not represent a subset of evoked GBA. The peak frequency of the intracranial evoked GBA was on average relatively low (approximately 25 Hz) and is, therefore, probably not equivalent to extracranially recorded GBA which has normally a peak frequency of approximately 40 Hz. Induced GBA was detected in 10 subjects, nearly exclusively in the region of the superior temporal lobe. The induced GBA was increased after stimulation for several hundred milliseconds and encompassed frequencies up to 200 Hz. Single-trial analysis revealed that induced GBA occurred in relatively short bursts (mostly <<100 ms), indicating that the duration of the induced GBA in the averages originates from summation effects. Both types of gamma band activity showed a clear attenuation with stimulus repetition.
Acoustic Stimulation↗
Finding the missing link between ictal bradyarrhythmia, ictal asystole, and sudden unexpected death in epilepsy.
Basic science studies of the human brain have supported the cortical representation of cardiovascular responses, including heart rate variability. Clinical observations of ictal bradyarrhythmia may be mechanistically explained by the influence of the central autonomic network, although the localization and lateralization issues need to be considered in the light of patterns of seizure spread, hand dominance, and presence of lesions. Ictal bradyarrhythmia also offers a mechanistic explanation of sudden unexpected death in epilepsy (SUDEP), though it may explain only some but not all cases of SUDEP. The missing links are (1) clinical evidence of common factors shared by patients with ictal bradyarrhythmia and patients who die from SUDEP, (2) evidence of arrhythmia as a risk factor for SUDEP from epidemiological studies, and, (3) determination of the importance of ictal bradyarrhythmia in SUDEP with respect to other proposed mechanisms including apnea and intrinsic cardiac abnormalities. There remains a need to review the seizure mechanisms in cases of SUDEP and to step up the amount of concurrent ECG/intracranial EEG analysis in both ictal bradyarrhythmia and SUDEP cases.
Autonomic Nervous System↗
Correlation of health-related quality of life after surgery for mesial temporal lobe epilepsy with two seizure outcome scales.
PURPOSE: The objective of this study was to correlate health-related quality of life (HRQOL) after surgery for mesial temporal lobe epilepsy, as revealed by a postoperative screening tool, to different modalities of seizure outcome classification (Engel, International League Against Epilepsy (ILAE)). METHOD: One hundred twenty-eight of one hundred forty consecutive patients returned a HRQOL questionnaire at a mean of 36 months after selective amygdalohippocampectomy. Patients answered in two ways: with an absolute estimation (values 1-4) and with a self-rated relative change (-1, 0, +1) after surgery. RESULTS: Eighty patients were seizure- and aura-free (63.3% ILAE 1), 16 continued to have auras (12.5% ILAE 2), and 13 experienced 1-3 seizure days per year after surgery (10.2% ILAE 3). Ninety-two patients were classified seizure-free (71.9% Engel I), and 17 had two or fewer seizures per year (13.3% Engel II). Of 110 patients in ILAE 1-3, 100 (91%) stated good or even very good postoperative HRQOL, and 99 (90.0%) reported improvements in HRQOL. Only 9 of the remaining 18 (50%) reported good or very good HRQOL after surgery (P=0.01). Corresponding results were obtained with Engel classes I and II, suggesting a trend toward ILAE 1-3 and Engel I and II as overall satisfactory outcomes. A more detailed HRQOL assessment yielded lowest scores in the cognitive domain, and a significant correlation of self-rated changes in cognitive functioning with seizure control (P=0.01). Changes in physical capabilities and mood were significantly better with satisfactory seizure outcome (P=0.006 and P<0.001, respectively), whereas the social aspects were not significantly dependent on seizure outcome (P=0.06). CONCLUSION: Correlation of HRQOL and seizure control suggested that ILAE 1-3 and Engel I and II most likely represent overall satisfactory outcome. Subdomain analyses revealed cognitive abilities as the most critical feature associated with seizure control, whereas social aspects remained mainly stable.
Adolescent↗
Shift-back of right into left hemisphere language dominance after control of epileptic seizures: evidence for epilepsy driven functional cerebral organization.
Atypical, i.e. right hemisphere language dominance is frequently observed in early onset left hemisphere epilepsies. In left mesial temporal lobe epilepsy, where eloquent cortex is not directly involved, it is a matter of debate, to which degree atypical language dominance is driven not only by morphological lesions but also by epileptic dysfunction, and whether atypical dominance is hardwired or not. Taking this as the background this study evaluated the hypothesis that epilepsy driven atypical dominancy might be reversible when seizures are successfully controlled. This was evaluated in patients with left mesial temporal lobe epilepsy, who were atypically language dominant by means of language fMRI before surgery, and became seizure free after left selective amygdalo-hippocampectomy. Three out of 53 consecutive atypically dominant patients with chronic epilepsy fulfilled these criteria. Postoperative follow-up language fMRI indicated reversal of right into left dominance in one patient going along with unexpected losses in verbal memory performance. The two other patients experienced unchanged or even enhancement of the pre-existing dominance pattern, going along with consistent postoperative performance changes in cognition. The data thus provide supporting evidence that atypical language dominance can indeed be functionally driven and moreover that in at least some patients, right hemispheric language can shift-back to the left hemisphere when the driving factor, i.e. seizures, becomes successfully controlled. The results have clinical implications for outcome prediction after brain surgery in atypically dominant patients with epilepsy. However, further research in larger groups of atypically dominant patients is required to identify the conditions under which atypical dominance becomes hardwired and when not.
Adult↗
[A new questionnaire for recognizing cognitive problems in children and adolescents].
BACKGROUND: Patients with psychiatric or neurologic disease often suffer from neuropsychologic problems. The purpose of this study was to present a parental questionnaire (KOPKIJ) that assesses neuropsychologic problems in children and adolescents between 6 and 16 years of age. METHODS: We tested theoretical analysis, standardization, and validation of the questionnaire in 352 healthy controls and 116 pediatric patients with epilepsy. RESULTS: The objectivity of this standardized questionnaire can be considered high, and the reliability of the subscales is satisfying. Epileptic patients had higher scores than healthy controls, and the validation displayed reasonable connections with clinical factors and the results of neuropsychological evaluation. CONCLUSION: The KOPKIJ allows an orientating appraisal clear picture of cognitive problems of children and adolescents at home and at school, simplifies anamnesis, and eases the planning of therapy strategies.
Adolescent↗
[NeuroCogFX--a computer-based neuropsychological assessment battery for the follow-up examination of neurological patients].
Many neurological therapeutic trials require a longitudinal assessment of cognitive functions. An ideal instrument for that purpose should be in accordance to the criteria of classical testing theory and, furthermore, it should be repeatable and economic in administration and interpretation. We developed NeuroCogFX, a computerized assessment battery, according to these criteria. NeuroCogFX comprises subtests for short term memory, working memory, psychomotor speed, selective attention, verbal and figural memory and verbal fluency (mean duration: 25 minutes). Age-related normative data was obtained from 244 subjects without history of neurological or psychiatric disease (age range 16 - 75 years). Forty-two subjects were re-tested after an average of 8 weeks (range: 6 - 10 weeks) in order to assess retest reliability and training effects. Retest-reliabilities were middle-sized in all but one subtest, ranging from r (12) = 0.5 to r (12) = 0.7 (2-back Test: r (12) = 0.37). For construct validation NeuroCogFX was administered in addition to a comprehensive neuropsychological assessment battery in a group of 40 healthy subjects and in 42 patients with chronic epilepsy. The test allows a valid assessment of short-term memory, reaction speed, memory and verbal fluency. NeuroCogFX is an economic, sufficiently reliable and valid instrument for the neuropsychological follow-up examination in single patients and study groups which can be administered if a comprehensive neuropsychological assessment is unavailable.
Adolescent↗
CCM3 mutations are uncommon in cerebral cavernous malformations.
Cerebral cavernous malformations (CCMs) are characterized by abnormally enlarged capillary cavities without intervening brain parenchyma. Mutations in the gene PDCD10 have been found in CCM families linked to the CCM3 locus. The authors screened this gene in 15 families that did not have a CCM1 or CCM2 mutation. Only two novel mutations were found, suggesting that mutations in this gene may only account for a small percentage of CCM familial cases.
Apoptosis Regulatory Proteins↗
Greater functional recovery after temporal lobe epilepsy surgery in children.
The purpose of our study is to evaluate whether children recover better than adults from memory deficits as a consequence of temporal lobe surgery. We compared 3 and 12 month outcomes obtained in children and adults with medically refractory epilepsy. Each candidate underwent temporal lobe resection for seizure control and children were matched with regard to pathology, onset of epilepsy, side of surgery and type of surgery with adults (N = 30 for each group, mean age at surgery 13 versus 30 years). Three months after surgery, both left-resected groups displayed a significant decline in verbal learning capacity. During the following 9 months, only the children recovered and were able to reach their preoperative level 1 year after surgery. The left-resected adults remained, for the most part, on their low level and one year after surgery, they were still significantly worse than at the time of their preoperative examination. The right-resected adults experienced a deterioration in visual memory 1 year after surgery relative to the results of the short-term follow-up; the children improved. The children also had a better outcome with regard to attentional functions and, as a trend, a better seizure outcome (Engel Outcome I--1 year after surgery: 63% adults, 80% paediatric patients). Our neuropsychological data provide evidence of greater plasticity and compensational capacity in childhood. The results can be taken as a strong argument for early surgical intervention.
Adolescent↗
Adult-onset epilepsy in focal cortical dysplasia of Taylor type.
Focal cortical dysplasia of Taylor type (FCDT) usually presents with seizures at an early age, whereas adult onset of epilepsy is uncommon. We reviewed the medical records of 213 patients with FCDT. In 21 patients (10%), age at seizure onset ranged from 18 to 55 years (mean 25.3). The outcome of seizures in patients with FCDT and adult-onset epilepsy seems favorable vs childhood-onset seizures.
Adolescent↗
MRI brain volumetry in Rasmussen encephalitis: the fate of affected and "unaffected" hemispheres.
Rasmussen encephalitis (RE) is regarded as a unihemispheric disease. Serial three-dimensional MRIs of 18 patients under immunotherapy were analyzed volumetrically and planimetrically. Median volume loss was significantly higher in the affected than in the unaffected hemispheres (29.9 cm3/y vs 6.8 cm3/y). Correlation of the planimetrically and volumetrically assessed hemispheric ratios (HRs) was significant. The results support the concept of RE as a basically unilateral disease. Planimetric HR assessment is valid and time efficient.
Adolescent↗
Pathogenesis, diagnosis and treatment of Rasmussen encephalitis: a European consensus statement.
Rasmussen encephalitis (RE) is a rare but severe immune-mediated brain disorder leading to unilateral hemispheric atrophy, associated progressive neurological dysfunction and intractable seizures. Recent data on the pathogenesis of the disease, its clinical and paraclinical presentation, and therapeutic approaches are summarized. Based on these data, we propose formal diagnostic criteria and a therapeutic pathway for the management of RE patients.
Adolescent↗
Processing of famous faces and medial temporal lobe event-related potentials: a depth electrode study.
The present study aims at analyzing the modulation of two types of event-related potentials originating from the human medial temporal lobe, the rhinal AMTL-N400 and the hippocampal P600 by the processing of famous faces. Therefore, we used a face recognition paradigm in which subjects had to discriminate the faces of famous persons from the faces of non-famous persons. Eleven patients with unilateral medial temporal lobe epilepsy undergoing intrahippocampal depth electrode recording for presurgical evaluation participated in this study. Event-related potentials (ERP) were recorded while a sequence of famous and non-famous faces was presented to the patients. The presentation of each face was repeated. The faces evoked N400-like potentials (anterior medial temporal lobe N400, AMTL-N400) in the rhinal cortex and P600-like potentials in the hippocampus. ERPs elicited by famous faces were contrasted with ERPs elicited by non-famous faces. The first presentation of famous faces elicited an enhanced AMTL-N400 and an enhanced hippocampal P600 in comparison to the second presentations of the famous faces or the (first and second presentation of the) non-famous faces. This findings are discussed in terms of associative semantic memory processes and the retrieval of person-specific information from long-term memory stores triggered by the processing of famous faces.
Adult↗
[Familial cavernous malformations of the central nervous system. A clinical and genetic study of 15 German families].
In 1928, Hugo Friedrich Kufs reported on a family with cerebral, retinal, and cutaneous cavernous malformations. Since then, more than 300 families with inherited cavernous malformations have been reported. Genetic studies showed three loci, on chromosomes 7q21-q22 (with the gene CCM1), 7p15-p13 (CCM2), and 3q25.2-q27 (CCM3). The gene product of CCM1 is Krit 1 (Krev interaction trapped 1), a protein interacting with angiogenesis by various mechanisms. Recently, CCM2 has also been identified; its product is a protein which might have a function similar to that of Krit 1. However, the CCM3 gene has still not been found. In this study, we present clinical and genetic findings on 15 German families.
Adult↗
[VGKC antibodies associated with limbic encephalitis].
Since the initial description of limbic encephalitis (LE) in 1960/1968, several subforms of this clinico-neuropathological syndrome have been identified. The best known is paraneoplastic LE. However, non-paraneoplastic forms have been reported, too. Very recently, autoantibodies against voltage-gated potassium channels have been described in association with LE. The diagnostic workup of such a case and the apparently typical good response to long-term immunotherapy of this LE subform are described.
Antibodies↗
[Recent insights into Rasmussen encephalitis].
Rasmussen encephalitis (RE) is a severe and at the same time pathophysiologically fascinating condition. The chronic inflammation affects one of the two cerebral hemispheres and destroys it during the disease process that lasts from months to years. The patients -- mostly children -- suffer from frequent pharmacoresistant seizures, often in the form of epilepsia partialis continua. In parallel to the atrophy of the affected hemisphere, the neurological functions associated with it decline continuously. This results in a final stage with a usually high-grade sensorimotor hemisyndrome, hemianopia, cognitive impairment and -- if the language-dominant hemisphere is affected -- aphasia. Research results in the last 5 years have contributed to a deeper understanding of the pathogenesis of this condition. Formal diagnostic criteria have been proposed, and new therapeutic options have emerged by which the disease progression can be slowed or stopped. This article summarizes the current research results on the background of older data and gives recommendations regarding diagnostic and therapeutic procedures in RE patients.
Clinical Trials as Topic↗
Sensory gating in the human hippocampal and rhinal regions.
OBJECTIVE: The objective of this work was to ascertain if sensory gating can be demonstrated within the human medial temporal lobe. METHODS: Eight patients with intractable epilepsy with depth electrodes implanted in the medial temporal lobe for pre-surgery evaluation underwent evoked response recording to auditory paired-stimuli (S1-S2). Each of the eight subjects had a diagnosis of left medial temporal lobe epilepsy (MTLE). RESULTS: Data from the non-focal right hippocampi revealed a large negative response on S1 (starting at about 190 ms and lasting for approximately 300 ms from stimulus onset). Rhinal region recordings revealed a positive response (starting at about 240 ms with a rapid incline, followed by a long-lasting decline). A significant attenuation of both responses to S2 stimuli was observed. CONCLUSIONS: Data are suggestive of an involvement of the human medial temporal lobe in the processing of simple auditory information which occurs in a time frame later than the neocortical auditory evoked components. The exact role of these anatomical structures in the sensory gating process remains to be defined. SIGNIFICANCE: This study provides the first evidence of an activation of the rhinal cortex after simple auditory stimulation and provides new evidence that the activation of the medial temporal lobe structures occurs at a later stage than that of the neocortex.
Acoustic Stimulation↗