Sarah Lippé, Marie-Sylvie Roy, Claudine Arcand, Morris H Scantlebury, Lionel Carmant, Maryse Lassonde
predictive value for the outcome of FSs infants.Pattern visual evoked potentials (pVEPs) were recorded in 18 FS infants (mean age of seizure 15.97 months). Spectral density and coherence analyses were performed in infants evaluated at 1.1 month (n = 4), 5.75 months (n = 4) or 30.33 months (n = 6) following a prolonged FS and compared to age-matched healthy controls. The impact of severity of seizures was assessed by comparing the children who had prolonged FSs to 4 infants that had experienced a simple FS. Cognitive tests (Bayley, Stanford-Binet) were administered at the time of testing in FS and control children. Behavioural measures (Achenbach Child Behavior Check List) were administered two years after the FS. pVEP responses and coherence measures failed to yield significant differences between the FS groups and healthy controls. However, spectral density measures showed a significant increase in delta band activity in both FS groups and a reduced high frequency density only in the prolonged FS groups that was seen up to 39 months post-seizure. Behavioural and cognitive measures revealed cognitive development within average, but lower attentional capacities in the FS infants. The persistent changes in spectral density patterns seen in children with prolonged FS may reflect seizure induced alterations in the developing brain or a result of a complex mode of inheritance. Further studies are needed to determine whether these observations can be used as a marker to predict the vulnerability of the child in developing behavioral deficits or epilepsy.
Michail Koutroumanidis, Katerina Tsatsou, Sue Sanders, Michael Michael, Stella V Tan, Alexandros Agathonikou, Chrysostomos P Panayiotopoulos
sensitivity (FOS), delineate the electrographic types of FOS abnormalities and identify the patterns that can be associated with clinical seizures, and examine whether there may be a pure form of fixation-off sensitive epilepsy. Methods. We reviewed the clinical and video EEG data of all our patients with FOS over the last 12 years. Children with idiopathic focal epilepsies and occipital EEG paroxysms were excluded. Results. From January 1995 to December 2006, 19 of about 8,500 patients had had one or more video-EEGs with FOS, yielding an approximate incidence of 0.2%. From the 14 patients with full clinical and EEG data available, 12 had various epilepsies that included IGE phenotypes (7), symptomatic or probably symptomatic focal (3), cryptogenic generalised (1), and adult onset idiopathic photosensitive occipital (1), and two had no seizures. Seven patients (50%) were photosensitive. FOS EEG abnormalities were occipital in six patients, generalised in eight, and generalised with posterior emphasis in two patients. Seven of these patterns were associated with habitual seizures in seven patients, but actual FOS-induced seizures (absences) were documented with video EEG in only one patient; three others had some historical evidence suggesting that, under some circumstances, their FOS EEG abnormalities might generate clinical seizures. Conclusions. Despite the association of FOS with generalised and focal, symptomatic and cryptogenic and mild or pharmaco-resistant epilepsies, closer analysis of our data, and supportive evidence from functional imaging and physiological observations on alpha rhythm generation, disclose a prominent role of the occipital areas, even when FOS EEG abnormalities and seizures are ostensibly generalised. Although FOS appears to be of relatively low epileptogenicity, an electroclinical profile of pure FOS epilepsy may exist [Published with video sequences].
Yoshiko Hirano, Hirokazu Oguni, Makiko Osawa
Subject and methods. The patient was a six-year two-month-old girl, who had started to have focal motor seizures, later combined with daily epileptic negative myoclonus (ENM) and drop attacks, causing multiple injuries. We studied episodes of ENM and drop attacks using video-polygraphic and computer-assisted back-averaging analysis. Results. A total of 12 ENM episodes, seven involving the left arm (ENMlt) and five involving both arms (ENMbil), and five drop attacks were captured for analysis. All episodes were time-locked to spike-and-wave complexes (SWC) arising from both centro-temporo-parietal (CTP) areas. The latency between the onset of SWC and ENMlt, ENMbil, and drop attacks reached 68 ms, 42 ms, and 8 ms, respectively. The height of the spike as well as the slow-wave component of SWC for drop attacks were significantly larger than that for both ENMlt and ENMbil (p < 0.05). Conclusion. Drop attacks were considered to be epileptic negative myoclonus involving not only upper proximal but also axial muscles, causing the body to fall. Thus, drop attacks in ABPE are considered to be epileptic negative drop attacks arising from bilateral CTP foci and differ from drop attacks of a generalized origin seen in Lennox-Gastaut syndrome and myoclonic-astatic epilepsy.
Sheila Koutsogiannopoulos, Francine Adelson, Virginia Lee, Frederick Andermann
precipitating factors for the occurrence of the first seizure have been considered but not documented. Recognition of such triggers may lead to a better understanding of the cause and mechanism of the epilepsy. Using a phenomenological approach, 19 participants were interviewed and recalled the occurrence of significant life events in the year prior to a diagnosis of generalized or focal epilepsy. There were gender and age-related differences in the types of triggering events, e.g. men tended to specify work related stressors while women generally cited relationship issues. None of the participants reported constraining beliefs about the cause of their epilepsy. Most respondents incorporated their knowledge of seizure triggers into strategies to achieve control of their epilepsy. This study highlights the potential value of questioning possible life stressors as triggers for the onset of epilepsy. Early awareness of high risk factors for seizures may lead to strategies of seizure self control by avoiding situations associated with high risks, improving lives disrupted by the uncertainty of epilepsy.
José Manuel Pardal-Fernández, María Carmen Carrascosa-Romero, Carlos de Cabo-de la Vega, Ivan Iniesta-López, Esther Gil-Pons, Andrés Martínez-Gutiérrez
jerks, apnea and encephalopathy three months after birth without satisfactory therapeutic response. During the first days of clinical symptoms the patient presented a paroxystic burst-attenuation EEG pattern which progressively evolved into an established typical burst-suppression pattern within a few days. West syndrome occurred four weeks later and the patient died at seven months of extra-uterine life due to a serious respiratory infection with cardio-respiratory arrest. Genetic analysis showed a non-previously described mutation affecting a consensus splice site (IVS2-1G > C 3) in the AMT gene encoding the T protein of the glycine cleavage system.
Judith Kröll-Seger, Christian G Bien, Hans-Jürgen Huppertz
clinical data in children are rare and only comprise paraneoplastic forms of LE. We describe a 13-year-old pre-pubertal girl in whom non-paraneoplastic LE was diagnosed according to diagnostic criteria proposed by Bien and Elger (2007). The girl presented with a subacute syndrome comprising memory impairment, affective disturbances, and refractory temporal lobe seizures. Serial MRI scans demonstrated an initial temporo-medial swelling with T2/FLAIR signal increase progressing to bilateral hippocampal atrophy within seven months. Two years after onset of symptoms, antibodies to potassium channels were found to be slightly elevated. Immunosuppressive therapy with steroid-pulses was followed by a transient reduction of seizure frequency, even though this was started more than two years after onset of first symptoms. However, extended immunotherapy was refused by the patient’s parents, so no full assessment of the treatment response was possible. In conclusion, this case shows that non-paraneoplastic LE leading to mesial temporal lobe epilepsy is not restricted to adult patients. The proposed diagnostic criteria therefore should be adapted for paediatric patients. Patients may profit from immunosuppressive therapy even when it is started at a late stage with already overt hippocampal sclerosis.
Ainhoa Marinas, Vicente Villanueva, Beatriz G Giráldez, Albert Molins, Javier Salas-Puig, Jose M Serratosa
with different types of IGEs who were treated with ZNS between the years 2006 and 2008 were identified. Efficacy and tolerability were assessed at months 6 and 12 post-treatment. Response was defined as a 50% or greater reduction in seizure frequency. Results. Twelve patients (92.3%) continued with ZNS at month 6, and 11 (84.6%) at month 12. Mean daily dose was 319 mg (range 100-500 mg/d). Response was achieved at month 6 in eight of the 12 patients that continued with ZNS (66.6%), of which 7 were seizure-free (58.3%). At month 12, eight of the 11 patients that continued with ZNS were responders (72.7%) and 6 were seizure-free (63.6%). For different types of seizures, better responses were observed for absences and generalized tonic-clonic seizures. Four out of 13 patients (30.7%) experienced adverse events and in two (15.3%), these led to withdrawal. Conclusion. In this retrospective study, ZNS showed efficacy and tolerability for the treatment of different types of IGEs. Limitations include a small sample size and a relatively short period of follow-up. Our results are promising and justify the need for prospective controlled trials in IGE.
Mohamad A Mikati, Nour F Ataya, Jessica C El-Ferezli, Tarafa S Baghdadi, Ali H Turkmani, Youssef G Comair, Sujay Kansagra, Marwan W Najjar
5 adults) who had vagal nerve stimulation at our center were studied. Quality-of-life was assessed pre- and post-vagal nerve stimulation using the Quality-of-Life in Childhood Epilepsy questionnaire for children and the Epilepsy Surgery Inventory-55 for adults. Results. Sixteen patients who did not qualify for resective surgery were included; seven (43.75%) were males and 9 (56.25%) were females. Mean age at onset of seizures was 3.96 ± 4.00 years and at surgery was 15.78 ± 10.78. Follow-up time was 1.26 ± 0.92 years. Fourteen patients (87.5%) were mentally retarded. Ten (62.5%) had cryptogenic etiology and 6 patients (37.5%) symptomatic etiology. Fifty percent had localization-related epilepsy. Six of 7 patients with generalized cryptogenic etiology (85.71%) had Lennox-Gastaut syndrome. Seizures dropped from 122.31 ± 159.49 to 67.84 ± 88.22 seizures/month. Seizure reduction (> 50%) correlated with improvement in total quality-of-life (p = 0.034). Post-vagal nerve stimulation, the total group scored significantly higher in the social domain (p = 0.039). In patients with localization-related epilepsy, significant improvements were detected in the social domain (p = 0.049) and in total quality-of-life (p = 0.042). Conclusion. Despite a diverse and small population size, we observed significant improvements in the social domain 1.26 years post-vagal nerve stimulation. In addition, there was an improvement in total quality-of-life amongst patients with partial seizures. Finally, seizure reduction was associated with quality-of-life improvement. Our results support previous studies from the West reporting improvement in quality-of-life following vagal nerve stimulation, contradict those studies that did not show such differences, and are the first coming from a developing country.
Iris Unterberger, Giorgi Kuchukhidze, Gerald Walser, Judith Dobesberger, Florian Koppelstaetter, Martin Ortler, Richard Bauer, Eugen Trinka
phenotypic expression, caused by mutations in one of the two tumor suppressor genes, TSC1 or TSC2. Epilepsy is the most common neurological presentation and seizures are often medically intractable. Definition of the epileptogenic zone during presurgical evaluation is challenging given the multiple potentially epileptogenic lesions visible on MRI. However, TSC patients may nevertheless achieve seizure freedom, when preoperative evaluation yields concordant results. The strategies used in these patients vary substantially among different epilepsy surgery centres. We present a 21-year-old right-handed, intellectually not impaired woman with TSC and medically intractable seizures since the age of 15 years. Careful multi-stage presurgical evaluation, including prolonged video-EEG-monitoring, cerebral high resolution MRI, ictal and interictal [99m Tc]HMPAO-SPECT, [18 F]FDG-PET and further invasive recordings with subdural and depth electrodes led to the identification of an epileptogenic tuber with concordant seizure onset zone in the right neocortical temporal lobe. A tailored resection was performed leading to excellent surgical outcome (follow-up 12 months, Engel class I).
Bernhard Oehl, Silke Biethahn, Andreas Schulze-Bonhage
a motor component. It has been suggested that the affective component may result from an involvement of temporobasal structures, whereas the motor part is related to an involvement of the mesial frontal cortex. So far, evidence is based on a limited number of cases with spontaneously recorded seizures or in whom electrical stimulation of invasive intracranial EEG recordings has been performed. We report a patient who suffered from epigastric psychic auras, complex partial seizures with a gelastic component and secondarily generalized seizures. To evaluate a possible epileptogenic role of the hippocampus and dysplastic region in the right mid-temporal gyrus, intracranial monitoring with subdural electrodes over the temporobasal and temporolateral regions, as well as a deep brain electrode in the hippocampus, were performed. During the intial part of the seizure, consisting of an intense retrosternal ascending feeling with sexual connotation, rhythmic spikes in temporolateral contacts were detected. Concomitant with the development of smiling and laughter, a rhythmic activity over the temporobasal regions evolved. The patient became seizure-free following right temporal lobe resection. This case supports the assumption that ictal involvement of temporobasal structures is crucial for gelastic seizure components in patients with temporal lobe epilepsy. Progression to temporobasal regions was associated with the feeling of happiness whereas motor components of laughter occurred later. These findings are in accordance with the interpretation of surface recordings by Dericioglu and co-workers in a similar previous case. [Published with video sequences].
Romy Hoque, Edouardo Gonzalez-Toledo, Elena Korniychuk, John DellaBadia, Stephen L Jaffe
motor-sensory, lingual-jaw cortical area. The clinical seizure phenomena were time-locked with the EEG epileptic activity. [Published with video sequences]
Pascal Vrielynck, Pierre Defresne
published polygraphic recordings are rare. We report the case of a 22 month-old boy suffering from a symptomatic CSWS syndrome associated with a perinatal stroke involving the right middle cerebral artery territory. He presented with psychomotor regression and daily multiple falls related to myoclonic-atonic seizures. Neurophysiological examination showed secondary generalized myoclonus systematically correlated with a bilateral spike spreading from the right central area. This confirms that positive myoclonus, in addition to negative myoclonus, may be responsible for epileptic falls in CSWS syndrome. [Published with video sequences]