Christoph Kellinghaus, Gabriel Möddel, Hiroshi Shigeto, Zhong Ying, Berit Jacobsson, Jorge Gonzalez-Martinez, Candice Burrier, Damir Janigro, Imad M Najm
to those found in human polymicrogyria. Previous studies reported in vitro hyperexcitability in this model, but in vivo epileptogenicity has not been confirmed.
<b>Methods.</b> Neocortical freeze lesions were induced in Sprague-Dawley rat pups (n = 10) on postnatal day 0 or 1 (P0/P1). Sham-operated animals served as controls (n = 10). On P60, animals were implanted with epidural electrodes for long-term video-EEG monitoring (4 weeks). The threshold for pentylenetetrazol-induced seizures was determined. Animals were sacrificed and brain sections processed for histological staining and in vitro electrophysiological recordings. Epileptiform field potential repetition rate, amplitude and integral were compared between slices containing a cortical freeze lesion, and slices from sham-operated rats.
<b>Results.</b> No interictal spikes and no electrographic or clinical seizures occurred in either group. The median threshold for pentylenetetrazol-induced seizures was 60 mg/kg for lesioned, and 45 mg/kg for control animals (difference not significant). No spontaneous epileptiform field potentials were recorded from either freeze-lesion or control slices bathed in normal, artificial cerebrospinal fluid (ACSF). Upon omission of Mg
<sup>2+</sup> from the bath, epileptiform field potentials were elicited that showed a significantly higher burst integral in the freeze lesion slices compared to control slices.
<b>Conclusion.</b> Neocortical freeze lesions induced in newborn rat pups show histological characteristics reminiscent of human cortical dysplasia. Brain slices containing neocortical freeze lesions display hyperexcitability in vitro, but the same lesion does not appear to show spontaneous epileptogenicity in vivo.
M Kurian, L Spinelli, J Delavelle, JP Willi, M Velazquez, V Chaves, W Habre, K Meagher-Villemure, E Roulet, JG Villemure, M Seeck
with the maximum yield should be considered in order to reduce the complexity of the workup. Objective. To determine the extent to which PET, ictal/interictal SPECT and its co-registration with the patient’s MRI contributes to correct localization of the epileptogenic focus, surgical intervention and to the post surgical outcome in paediatric patients. Methods. The study population included children and adolescents with pharmacoresistant epilepsy (n = 50) who underwent preoperative evaluation, surgery and had postoperative follow-up for at least 12 months. Outcome was measured by postoperative seizure frequency using Engel’s classification. Results. Thirty-nine patients (78%) became completely seizure free after surgical intervention. The likelihood to benefit from surgical treatment was significantly higher if localization with more imaging modalities (MRI, PET, SPECT) were concordant with respect to the resected brain area (p < 0.01). Preoperative PET examination provided better localizing information in patients with extratemporal epilepsy and/or dysplastic lesions, whereas SPECT was found to be superior to PET in patients with temporal lobe epilepsy and/or tumors (p < 0.05). No significant difference was noted in the surgical outcome in younger or older age group, in children with or without special education needs. Conclusion. In paediatric epilepsy pre-surgical evaluation, the combined use of multiple functional imaging modalities for a precise localisation of the epileptogenic focus is worthwhile for both extratemporal and temporal lobe epilepsy, also when EEG and MRI alone are non-contributive, given the potential benefit of complete postoperative seizure control.
Laetitia Deltour, Marion Barathon, Véronique Quaglino, Marie-Pierre Vernier, Pascal Despretz, Muriel Boucart, Patrick Berquin
between focal and generalized epilepsy. In this study, an attentional capture paradigm was used to study and compare one aspect of attentional control, the resistance to interference from distractors, in 18 children with benign epilepsy with centrotemporal spikes (BECTS), 18 children with idiopathic generalized epilepsy and 18 controls aged 7-12 years. The results showed longer response times (RT) and more omissions in the two groups with epilepsy compared to controls. Attentional capture with longer response times in trials with a moving distractor compared to baseline condition with stationary distractors was found in both controls and children with epilepsy. The magnitude of interference from moving distractors was greater in the BECTS group than in the idiopathic generalized epilepsy group and in the controls group. These results suggest an impact of epilepsy on resistance to interference from distractors in children with BECTS.
Gerhard Bauer, Richard Bauer, Judith Dobesberger, Thomas Benke, Gerald Walser, Eugen Trinka
event. We report four cases of absence status with presentation after the age of 60 years. At admission, no history of epilepsy had been mentioned. The preliminary diagnosis of absence status occurring de novo in later life had to be changed on completion of case histories. All patients had suffered from idiopathic generalized epilepsy with absence seizures, which had resolved after puberty. A second peak of IGE, with repeated episodes of absence status after the menopause and without identifiable triggering factors is assumed.
Taim Muayqil, Brian H Rowe, S Nizam Ahmed
sought to review the process of acute management of SE at the University of Alberta Hospital and correlate outcome with adherence to a recommended treatment protocol. Methods. We identified 86 patients 18 years of age or older who presented with convulsive SE to our emergency room between 2000 and 2004. We defined SE as continuous convulsive activity for 30 or more minutes or ≥ 2 convulsions with incomplete recovery in the interim. Information was collected pertaining to etiology, epidemiology, and management. We then reviewed the relationship of the treatment protocol in terms of mortality and morbidity. Results. Forty five patients were included. There were 18 males and 27 females with a mean age of 45 years; 80% were known to have epilepsy. Sub-therapeutic drug levels were found in the majority 60%; benzodiazepines (diazepam 81% and lorazepam 19%) were the first line agent in 93.3% mostly initiated by paramedics (EMS); 48.9% of patients required intubation and 26.7% required admission to intensive care. Four patients died. Control of convulsive SE was obtained sooner for patients in whom therapy was administered according to the recommended time frame (p ≤ 0.02). Conclusion. The presence of strict treatment protocols for SE made readily available for the treating staff could potentially improve the outcome of patients. Despite the lack of standardized treatment protocols among various physicians, most patients are treated according to generally recommended sequence and time frames. Analysis of this data will help devise prospective treatment protocols.
Candan Gürses, Murat Kürtüncü, Jeffrey Jirsch, Nilüfer Yeşilot, Haşmet Hanağası, Nerses Bebek, Betül Baykan, Murat Emre, Ayşen Gökyiğit, Frederick Andermann
administration. In all cerebrospinal fluid and the serum Venereal Disease Research Laboratory Test (VDRL) and Treponema Pallidum hemagglutination (TPHA) or fluorescent treponemal antibody absorption test (FTA-ABS) were positive, but HIV serology was negative. Their EEGs showed periodic, lateralized, epileptiform discharges (PLEDs) just after SE. The first and third patients had no history of epilepsy. Seizures started as focal motor attacks but then secondarily generalized. The first patient’s cranial MRI showed cerebral atrophy and hyperintensity involving bilateral medial and anterior temporal regions, more prominent on the left and which disappeared after penicillin treatment. The second case, after receiving penicillin, had nonconvulsive SE, a clinical presentation suggesting a Jarisch-Herxheimer reaction (JHR). Her cranial MR revealed moderate cortical atrophy and widespread confluent hyperintense foci mainly in both periventricular areas, corona radiata and centrum semiovale. MRI of the third case showed a large, left sylvian, arachnoid cyst without mass effect. Executive dysfunction was observed in follow-up neuropsychological tests in all patients. When investigating status epilepticus, neurosyphilis as a cause must not be forgotten.
Katia Bruhn, Saskia Kronisch, Stephan Waltz, Ulrich Stephani
hypersynchronous activity is induced less often by viewing a PC monitor than a television screen and 2) whether certain images are more likely to cause hypersynchronous activity than others. All subjects were tested for sensitivity to intermittent photic stimulation (IPS) and to a black and white striped pattern on cards. Additionally, all were subjected to stimuli from four different images (vertical black and white striped pattern, geometric figures, text, and a painting by Max Pechstein – 1913, Italian church), presented on a television screen (with an image regeneration frequency of 50 Hz) and on PC screens (with regeneration frequencies of 48 and 100 Hz). A total of 21 non-photosensitive, healthy children and adolescents served as controls. Of the 48 photosensitive subjects 13% were also pattern sensitive (cards), and 33% exhibited screen sensitivity. No differences were found between the three monitor types. However, the hypersynchronous reactivity to the four images presented was significantly different, with high contrast vertical striped pattern being the most provocative. Non-photosensitive subjects did not react to any of the stimuli. The results of the present study show that screen-dependant factors are less important than image-dependant factors.
Lorena Benavente Fernández, Javier Salas-Puig
up for at least ten years. Patients younger than 18 years of age were excluded. The primary endpoint was the occurrence of seizures while awake, after a period of 10 years or longer suffering from pure sleep seizures. Results. The duration of the pure sleep seizures ranged from 10 to 67 years (median 22). The patients had been followed in our Department for a mean of 12 years. Patients’ ages ranged from 18 to 88 years (median 50); 44% of patients suffered from apparently generalized seizures. Epilepsy was considered undetermined in 38.2%, focal cryptogenic in 38.2%, and focal symptomatic in 21.8%. There was a single case of idiopathic generalized epilepsy. In the last evaluation, 35 patients were on monotherapy and two were not receiving treatment. Seizure frequency was < 1/year in 65.5%; 1-10/year in 14.5%; > 1/month in 9.1%. Seventeen patients (30.9%) had suffered one or more seizures while awake. Multivariate analysis showed that sudden withdrawal of treatment (p < 0.032) and polytherapy (p < 0.18) were associated with an increased risk of seizures while awake. Conclusions. In spite of a small number of seizures and good response to monotherapy, a third of the patients studied suffered seizures while awake. The significant risk factors were sudden withdrawal of treatment and polytherapy.
G Kuchukhidze, I Unterberger, J Dobesberger, N Embacher, G Walser, G Luef, F Koppelstaetter, Th Gotwald, G Bauer, ST Felber, E Trinka
The mechanisms which cause this condition are not well understood. The aim of our study was to delineate further, clinical and neuroimaging features of this malformation of cortical development and to explore its possible etiological background.Patients and methods. Four patients (two males and two females), aged from 23 to 31 years (mean age range 27.5 years) were evaluated. Subjects underwent clinical, electrophysiological, neuropsychological and high resolution magnetic resonance imaging assessment.Results. No significant perinatal event or exposure to intrauterine infection was noted. None suffered from birth asphyxia or ischemic injury. The parents of two patients were first cousins. Every subject had delayed developmental milestones, mental disability and congenital, non-progressive, spastic hemiparesis. They had epilepsy with seizure-onset ranging from three months to 17 years (mean 6.8 years); two had intractable seizures. In all patients, unilateral, right-sided polymicrogyria was associated with ipsilateral cerebral hemiatrophy. Polymicrogyria involved mainly anterior perisylvian areas; occipital regions were relatively spared.Conclusion. The evaluated patients showed homogenous clinical and neuroimaging characteristics. We support the idea that the disorder could constitute a clinical entity with an underlying genetic cause.
Clinical commentary with video
Howan Leung, Kasper Schindler, Patrick Kwan, Christian Elger
death in epilepsy (SUDEP). We report an 18-year-old patient with frontal lobe epilepsy who had intracranial electrode placement showing bifrontal seizure-onset. This patient received electrical stimulation to the left cingulate gyrus and developed cardiac asystole within 3 seconds of electrical stimulation. Intracranial monitoring showed epileptiform discharges in the left frontal polar, frontal lateral and interhemispheric electrodes. We suggest that the left cingulate gyrus, as part of the central autonomic network, may mediate bradyarrhythmia through the vagal pathway. There remains the possibility that other brain regions were also involved due to the time lag between asystole and epileptiform discharges, and the lack of intracranial exploration in the mesial temporal and insular regions. [Published with video sequences]
Pavel Krsek, Michal Tichy, Milan Hajek, Monika Dezortova, Josef Zamecnik, Milan Zedka, Radana Stibitzova, Vladimir Komarek
clear-cut lateralization. MRI showed a lesion in the right cingulate gyrus, initially regarded as a hamartoma. Ictal SPECT did not have a localization value. MR spectroscopy revealed two metabolic abnormalities: one in the area of the MRI lesion and a second contra-laterally. Invasive monitoring using subdural electrodes covering the convexity and mesial part of the right frontal lobe including mesial strips with bilateral contacts was used. The invasive monitoring failed to localize ictal onset in the right hemisphere; however, electric stimulation induced seizures from electrodes facing the left supplementary sensorimotor area (“through” the falx cerebri). We re-implanted the electrodes over the left frontal lobe and the second invasive monitoring clearly localized the ictal onset zone in the left supplementary sensorimotor area, which was subsequently resected. Histopathology found MRI-negative focal cortical dysplasia. The contralateral lesion was reassessed as nonspecific enlargement of perivascular spaces. The patient has been seizure-free for more than two years.[Published with video sequences]
Eduardo López-Laso, M Elena Mateos González, Rafael Camino León, M Dolores Jiménez González, Javier Esparza Rodríguez
infarction. We describe the case of an infant with gelastic, dacrystic and other types of seizures associated with a giant hypothalamic hamartoma, and present a video sequence of dacrystic seizures. Dacrystic episodes presented in clusters at sleep onset, initially in the form of moaning followed by face-flushing that rapidly evolved to crying, associated with a lateral and upper deviation of both eyeballs, along with clonic aspects of the eyelids. After a few seconds, the crying became less intense, she stared, and oro-alimentary automatisms became prominent along with some slow horizontal movements of the eyes and the head. Following surgery, at the age of nine months, the gelastic seizures stopped, but dacrystic seizures persisted. [Published with video sequences]
Andreas Hahn, Martina Heckel, Bernd A Neubauer
infants in 1995, only 16 additional patients have been reported. We add a further case and present a video that shows a typical generalizing tonic seizure arising from the right temporo-occipital area and slowly spreading about both hemispheres. In addition to other symptoms previously described, almost complete arrest of brain growth with onset of seizures and evolution of distinctive secondary microcephaly were striking features in this patient. [Published with video sequences]