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Editorial
A new perspective for Epileptic Disorders
Alexis Arzimanoglou
of epileptology in which new diagnostic techniques and approaches to management are continually being developed. A significant amount of new information in all areas of epileptology has accumulated at a brisk pace, leading to a steady increase in the number of articles submitted to the journal. As intended from the outset, the descriptive detailed clinical symptomatology and phenomenology of epileptic seizures and syndromes, in relation to diagnosis and management, published in Epileptic Disorders has served to complement other journals in the field...
Original article
SISCOM and FDG-PET in patients with non-lesional extratemporal epilepsy: correlation with intracranial EEG, histology, and seizure outcome
Martin Kudr, Pavel Krsek, Petr Marusic, Martin Tomasek, Jiri Trnka, Katerina Michalova, Monika Jaruskova, Jan Sanda, Martin Kyncl, Josef Zamecnik, Jan Rybar, Alena Jahodova, Milan Mohapl, Vladimir Komarek, Michal Tichy
positron emission tomography (FDG-PET) in patients with extratemporal epilepsy and normal MRI.
<i>Methods.</i> We retrospectively studied a group of 14 patients who received surgery due to intractable epilepsy and who were shown to have focal cortical dysplasia, undetected by MRI, based on histological investigation. We coregistered preoperative SISCOM and PET images with postoperative MRI and visually determined whether the SISCOM focus, PET hypometabolic area, and cerebral cortex, exhibiting prominent abnormalities on intracranial EEG, were removed completely, incompletely, or not at all. These results and histopathological findings were compared with postoperative seizure outcome.
<i>Results.</i> Two patients underwent one-stage multimodal image-guided surgery and the remaining 12 underwent long-term invasive EEG. SISCOM findings were localised for all but 1 patient. FDG-PET was normal in 3 subjects, 2 of whom had favourable postsurgical outcome (Engel class I and II). Complete resection of the SISCOM focus (
<i>n</i>=3), the area of PET hypometabolism (
<i>n</i>=2), or the cortical regions with intracranial EEG abnormalities (
<i>n</i>=7) were predictive of favourable postsurgical outcome. Favourable outcome was also encountered in: 4 of 8 patients with incomplete resection and 1 of 2 with no resection of the SISCOM focus; 4 of 7 patients with incomplete resection and 1 of 2 with no resection of the PET hypometabolic area; and 2 of 7 patients with incomplete resection of the area corresponding to intracranial EEG abnormality. No correlation between histopathological FCD subtype and seizure outcome was observed.
<i>Conclusion.</i> Complete resection of the dysplastic cortex localised by SISCOM, FDG-PET or intracranial EEG is a reliable predictor of favourable postoperative seizure outcome in patients with non-lesional extratemporal epilepsy.
Original article with supplemental data
Spatiotemporal profiles of focal and generalised spikes in childhood absence epilepsy
Vasileios Kokkinos, Andreas M Koupparis, Dimitrios Tsiptsios, George K Kostopoulos, Michalis Koutroumanidis
has been used to imply fast, secondary, 3-Hz generalisation from occult frontal foci with potential impact on clinical EEG interpretation and syndrome classification. The aim of this study was to investigate the relationship between focal and generalised SWDs. We studied five children with CAE and examined a sufficient number of focal (“interictal”) and generalised SWDs in order to obtain reliable analysis. All generalised SWDs with focal lead-in were “decomposed” into their “pre-generalisation” focal and “generalised” constituents, which were studied separately. Two types of focal SWD (“interictal” and “pre-generalisation”) and generalised SWD were visually clustered into groups, waveform-averaged, and plotted in the 2D-electrode space. Spatiotemporal analysis demonstrated a variety (mean: 4.2 per child; SD: 2.12) of mainly frontal and occipital locations for pre-generalisation focal SWDs with propagation along the longitudinal axis in either direction and across homologous sites. Interictal focal SWDs demonstrated similar spatiotemporal characteristics. In contrast, the topography and propagation patterns of the first generalised spike of the SWD showed less variability (mean: 2.5 per child; SD: 2.07), mainly involved the fronto-temporal/temporal areas, and correlated poorly (<10%) with that of the pre-generalisation focal SWD. Our findings suggest that the process of generalised epileptogenesis in genetic epilepsies with electrographic “frontal absences” is far more complex than that proposed by the model for occult frontal focus with fast secondary generalisation. (
<i>Published with Supplemental data</i>)
Sensitivity of scalp 10-20 EEG and magnetoencephalography
Yosuke Kakisaka, Rafeed Alkawadri, Zhong I Wang, Rei Enatsu, John C Mosher, Anne-Sophie Dubarry, Andreas V Alexopoulos, Richard C Burgess
are no published reports that focus on the relationship between spike dipole orientation or sensitivity of scalp EEG/MEG and the “gold standard” of intracranial recording (stereotactic EEG). We evaluated two patients with focal epilepsy; one with lateral temporal focus and the other with insular focus. Two MEG recordings were performed for both patients, each recorded simultaneously with initially scalp EEG, based on international 10-20 electrode placement with additional electrodes for anterior temporal regions, and subsequently stereotactic EEG. Localisation of MEG spike dipoles from both studies was concordant and all MEG spikes were detected by stereotactic EEG. For the patient with lateral temporal epilepsy, spike sensitivity of MEG and scalp EEG (relative to stereotactic EEG) was 55 and 0%, respectively. Of note, in this case, MEG spike dipoles were oriented tangentially to scalp surface in a tight cluster; the angle of the spike dipole to the vertical line was 3.6 degrees. For the patient with insular epilepsy, spike sensitivity of MEG and scalp EEG (relative to stereotactic EEG) was 83 and 44%, respectively; the angle of the spike dipole to the vertical line was 45.3 degrees. For the patient with lateral temporal epilepsy, tangential spikes from the lateral temporal cortex were difficult to detect based on scalp 10-20 EEG and for the patient with insular epilepsy, it was possible to evaluate operculum insular sources using MEG. We believe that these findings may be important for the interpretation of clinical EEG and MEG.
Clinico-pathological investigations of Rasmussen encephalitis suggest multifocal disease progression and associated focal cortical dysplasia
Dandan Wang, Ingmar Blümcke, Qiuping Gui, Wenjing Zhou, Huancong Zuo, Jiuluan Lin, Yang Luo
was to systematically characterise patterns of structural lesions in children with Rasmussen encephalitis, referred for modified anatomical hemispherectomy at the Tsinghua University Epilepsy Center in Beijing. Seven consecutive patients were investigated with a mean age at operation of 4.5 years, who suffered from medically intractable seizures for a mean of 1.6 years.
<b> </b>Foci of abnormally increased T2 signal intensity were observed in all patients. With the exception of one child, all patients presented with progressive unilateral cerebral atrophy. FDG-PET imaging revealed extensive regions of hypometabolism within the affected cerebral hemisphere in 3 of 4 patients. Diagnosis of Rasmussen encephalitis was confirmed histologically, demonstrating CD68 positive microglial nodules, as well as CD3 and CD8 positive T lymphocytes invading the cerebral parenchyma. An intriguing observation was the heterogenous distribution of patterns of lesions throughout the affected hemisphere, suggesting multifocal manifestation and distinct sequences of disease progression, from discrete foci of inflammatory infiltrates (stage 1) to extensive cortical destruction (stage 4). Atypical hippocampal sclerosis (HS), with neuronal cell loss affecting most prominently the CA4 region (HS type 3 or end folium sclerosis), was evident in 5 of 7 cases. Four hippocampi also showed chronic inflammation. In addition, we observed associated focal cortical dysplasia (FCD; ILAE type IIId) in 4 of 7 children, supporting the concept of acquired and postmigratory FCD pathomechanisms. Postsurgical seizure freedom was achieved in all children with a mean follow-up period of 2.7 years and continuous antiepileptic medication.
Original article
Head atonic attacks: a new type of benign non-epileptic attack in infancy strongly mimicking epilepsy
Giuseppe Capovilla, Alessandra Montagnini, Cinzia Peruzzi, Francesca Beccaria
limited to a specific period of life. If motor manifestations are strongly suggestive of an epileptic phenomenon, the risk of misdiagnosis is greater. Here, we describe a novel NEA with infantile onset, characterised by repeated head drops, mimicking epileptic negative myoclonus of the neck. The frequency of the episodes was very high, at hundreds or thousands per day. The episodes appeared in the second semester of the first year of life and spontaneously disappeared within a few months. [
<i>Published with video sequences</i>]
Short-term efficacy and tolerability of rufinamide adjunctive therapy in children with refractory generalised epilepsy
Se Hee Kim, Jeong Ho Lee, Hye Won Ryu, Byung Chan Lim, Jong Hee Chae, Ji Eun Choi, Yong Seoung Hwang, Ki Joong Kim
syndrome, 5 with Dravet syndrome, and 28 with unclassified refractory generalised epilepsy. Patients with more than 50% seizure reduction at three and six months were defined as responders. The overall response rate was 37.7% at three months and 34.0% at six months. At three months, patients with Lennox-Gastaut syndrome (40.0%) and epilepsy with spasms/tonic seizures (38.5%) showed higher response rates than those with Dravet syndrome (20.0%) and epilepsy with myoclonic seizures (20.0%). High response rates in patients with Lennox-Gastaut syndrome (30.0%) and epilepsy with spasms/tonic seizures (38.5%) were sustained throughout the six-month study. The accuracy of, and differences between, responder rates should, however, be interpreted with caution due to the small number of patients. Overall, rufinamide appeared to be effective and reasonably well tolerated in this group of children with refractory generalised epilepsies, although a subgroup of patients with Dravet syndrome and epilepsy with myoclonic seizures were less responsive to rufinamide treatment.
Clinical commentary
Neonatal suppression-burst without epileptic seizures: expanding the electroclinical phenotype of STXBP1-related, early-onset encephalopathy
Massimo Mastrangelo, Angela Peron, Luigina Spaccini, Francesca Novara, Barbara Scelsa, Paola Introvini, Federico Raviglione, Stefano Faiola, Orsetta Zuffardi
severe developmental delay and poor prognosis. Mutations and deletions in the
<i>STXBP1</i> gene are associated with Ohtahara syndrome, also known as “
<i>early infantile epileptic encephalopathy</i>”. We report an infant affected by EOEE with a 9q34.11 deletion that encompassed the genes
<i>STXBP1</i> and
<i>SPTAN1</i>. The infant presented with neonatal encephalopathy without epileptic seizures and an EEG pattern varying from highly discontinuous to suppression-burst. This was followed by West syndrome at 2 months with atypical hypsarrhythmia and spasms, easily controlled by therapy. Our findings suggest that molecular analysis of
<i>STXBP1</i> should be considered for newborns affected by neonatal encephalopathy associated with a peculiar EEG pattern, even in the absence of neonatal epileptic seizures.
Clinical commentary
Intracranial video-EEG and surgery for focal atonic seizures
Martín Donadío, Gabriela Ugarnes, Mónica Segalovich, Naomi Arakaki, Federico Sanchez Gonzalez, César Petre, Hugo Pomata, Carlos D’Giano
motor area or the supplementary negative motor area, could be responsible. We present findings observed in a patient with atonic seizures due to focal epilepsy, who underwent intracranial video-EEG monitoring and epilepsy surgery, and discuss possible underlying mechanisms. [
<i>Published with video sequences</i>]
Post-traumatic epilepsy with isolated memory flashbacks
Chusak Limotai, Seyed M. Mirsattari
to the development of paroxysmal episodes of isolated memory flashbacks related to the injury. Detailed analysis of her symptoms along with video-EEG telemetry recordings was helpful to distinguish between these two conditions. [
<i>Published with video sequences</i>]
Crying with sorrow evoked by electrocortical stimulation
Tyson Burghardt, Maysaa M Basha, Darren Fuerst, Sandeep Mittal
first reported case of cortical stimulation of the left posterior orbito-frontal gyrus, generating consistent and reproducible crying with affective content in a 41-year-old woman with medically intractable left temporal lobe epilepsy, who underwent extraoperative intracranial video-EEG monitoring for resective non-lesional epilepsy surgery.
Corpus callosotomy in a patient with startle epilepsy
Nicolás Garófalo Gómez, Ana Paula Hamad, Murilo Marinho, Igor M Tavares, Henrique Carrete, Luís Otávio Caboclo, Elza Márcia Yacubian, Ricardo Centeno
suffering with startle-induced seizures since 2 years of age. She had focal, tonic and tonic-clonic seizures, refractory to antiepileptic treatment. Daily tonic seizures led to very frequent falls and morbidity. Neurologically, she had no deficit. Interictal EEG showed slow waves and epileptiform discharges in central and fronto-central regions. Video-polygraphic recordings of seizures, triggered by stimuli, showed generalised symmetric tonic posturing with ictal EEG, characterised by an abrupt and diffuse electrodecremental pattern of fast activity, followed by alpha-theta rhythm superimposed by epileptic discharges predominantly over the vertex and anterior regions. Magnetic resonance imaging showed no abnormalities. Corpus callosotomy was performed when the patient was 17. Since surgery, the patient (one year follow-up) has remained seizure-free. Corpus callosotomy may be considered in patients with startle epilepsy and tonic seizures, in the absence of focal lesions amenable to surgery. [
<i>Published with video sequences</i>]
Gastaut type idiopathic childhood occipital epilepsy
Taissa Ferrari-Marinho, Eugenia Fialho Macedo, Rafael Scarpa Costa Neves, Lívia Vianez Costa, Ivanda SS Tudesco, Kelly C Carvalho, Henrique Carrete, Luis Otavio Caboclo, Elza Marcia Yacubian, Ana Paula Hamad
or blindness. Other occipital (non-visual) symptoms may also occur. Interictal EEG typically shows occipital paroxysms, often with fixation-off sensitivity. Ictal EEG is usually characterised by interruption by paroxysms and sudden appearance of low-voltage, occipital, fast rhythm and/or spikes. Despite well described clinical and EEG patterns, to our knowledge, there are very few reports in the literature with video-EEG recording of either seizure semiology or fixation-off phenomena. We present a video-EEG recording of a 12-year-old girl with Gastaut type epilepsy, illustrating the interictal and ictal aspects of this syndrome. Our aim was to demonstrate the clinical and neurophysiological pattern of a typical seizure of Gastaut type epilepsy, as well as the fixation-off phenomena, in order to further clarify the typical presentation of this syndrome. [
<i>Published with video sequences</i>]
Pure epileptic headache and related manifestations: a video-EEG report and discussion of terminology
Carlo Cianchetti, Dario Pruna, Lucia Porcu, Maria Teresa Peltz, Maria Giuseppina Ledda
of severe frontal headache, which corresponded to the presence of concurrent spikes and slow waves, starting in the right temporal area. A dysplastic lesion of the right temporal lobe was observed by MRI and the patient received surgery, with subsequent disappearance of headaches. This case highlights ictal EEG as the main diagnostic tool for epileptic headache. We discuss the terminology regarding this type of manifestation and believe that cases without subsequent epileptic manifestations, as in the present case, should be more appropriately referred to as “
<i>pure ictal epileptic headache</i>” or simply “
<i>pure epileptic headache</i>”. [
<i>Published with video sequences</i>]
Misleading features of neuroimaging and electroencephalography: insulinoma misdiagnosed as temporal lobe epilepsy
Jérôme Aupy, Aurélien Benoilid, Maysa Sarhan, Constanza Dalvit, Maria-Paola Valenti, Edouard Hirsch
abnormalities not related to phenomenology. Consequences of erroneous diagnosis of epilepsy may lead to aggressive and escalating pharmacotherapy with potentially serious side effects. Metabolic disorders, which may mimic epilepsy, should always be considered as they are potentially curable and may be fatal if untreated. We report a case of an insulinoma, misdiagnosed as temporal lobe epilepsy. We highlight the risks associated with misinterpretation of neuroimaging and EEG and outline an approach to differentiate between symptoms of insulinoma or neuroglycopenia and temporal epileptic seizures.
