Ictal video-EEG Research Articles

Atonic‐BECTS: An unusual presentation of self‐limited epilepsy in childhood

AbstractObjectivesWe describe an unusual patient with self‐limited epilepsy in childhood to aid in the accurate diagnosis and timely treatment of an atonic variant of self‐limited focal epilepsy of childhood with centrotemporal spikes.MethodsWe reviewed the medical records documenting the clinical presentation, diagnostic evaluation, and treatment. We also reviewed the relevant video electroencephalograms (EEGs).Patient DescriptionThis 3‐year‐old girl with self‐limited focal epilepsy of childhood (formerly called benign rolandic epilepsy) began having recurrent falls. Multiple clinical seizures were recorded on video EEG. The video documented generalized loss of tone resulting in falls, while the ictal EEG revealed one‐second paroxysms of 4 Hz spike‐slow‐wave discharges in the left centrotemporal region, followed by a brief generalized electrodecrement for 400 milliseconds. These findings support the diagnosis of an atypical variant of benign epilepsy with centrotemporal spikes (BECTS), known as atonic‐BECTS. Valproic acid was maximized. On follow‐up, the patient was seizure‐free with a normal EEG and normal development.DiscussionFew prior publications describe atonic‐BECTS. We present a child with atonic‐BECTS whose ictal video EEG confirms atonic seizures. While atonic seizures typically occur with generalized epilepsies, our report highlights that they can present as an atypical manifestation of self‐limited focal epilepsy in childhood.

Epileptic nystagmus as clinical manifestation of self-limited focal epilepsy: Rethinking etiology and prognostic value and literature review.

Epileptic nystagmus (EN) is an uncommon ictal clinical manifestation characterized by rapid, repetitive eyeballs movements. Few cases of EN have been reported and, in most cases, electro-clinical correlation showed a focal EEG activity, mainly in the occipital and temporo-occipital areas. Although EN occurs both in idiopathic and non-idiopathic epilepsy, the most frequent cause appears to be inborn or acquired alteration of brain structures. We report of a 12-year-old girl with EN as ictal manifestation of self-limited focal seizures. We described clinical manifestations, electroencephalographic features, treatment, and follow-up, presenting the ictal video-EEG phenomenon. Alongside, we reviewed the reported clinical features of the few pediatric cases (seven patients) with idiopathic epileptic nystagmus through a systematic literature review. Isolated Epileptic Nystagmus (IEN) is much rarer than EN, as it is more frequently associated with other types of seizures, and can be idiopathic, especially in children. Epilepsy prognosis is usually favorable with appropriate treatment, and ASM discontinuation seems to be successful after few years of treatment.

Long-term follow-up of vagus nerve stimulation in drug-resistant KCNT1-related epilepsy: a case presentation

BackgroundThe KCNT1 gene encodes a Na+-activated K+ channel. Gain-of-function mutations of KCNT1 lead to autosomal dominant sleep-related hypermotor epilepsy, early-onset epileptic encephalopathy, focal epilepsy and other epileptic encephalopathies. In this paper, we report a boy carrying a KCNT1 gene mutation, who presented with drug-resistant focal-onset seizures. He had decreased seizure frequency and improvement of background changes in electroencephalography (EEG) after vagus nerve stimulation (VNS).Case presentationThe case was a nonverbal 9-year-old male who presented with drug-resistant focal-onset seizures since age 3 and had underwent VNS therapy for 2 years. He had hypermotor symptoms, automatism and bilateral asymmetric tonic seizures with cognitive decline and aphasis from age 3. The patient had a variety of seizure types that only occurred at night. The most common seizure type was automatisms, and ictal video EEG showed high-amplitude delta waves, followed by a fast rhythmic sharp activity in the mesial frontal and bitemporal regions. The patient was diagnosed with KCNT1-related epilepsy, epileptic encephalopathy and cognitive disorder. He was refractory to multiple anti-seizure medicines (ASM) and ketogenic diet. After VNS treatment at age 7, the frequency of seizures was reduced significantly and EEG was improved in background slowing.ConclusionsChildren with KCNT1-related epilepsy usually have early onset of disease, are nonverbal, and are refractory to ASM. This boy with drug-resistant KCNT1-related epilepsy showed significantly reduced seizure frequency after VNS. This report may provide reference for management of cases of KCNT1-related epilepsy.

Role of Interictal Arterial Spin Labeling Magnetic Resonance Perfusion in Mesial Temporal Lobe Epilepsy.

Context:Electrophysiological and hemodynamic data can be integrated to accurately identify the generators of abnormal electrical activity in drug-resistant focal epilepsy. Arterial Spin Labeling (ASL), a magnetic resonance imaging (MRI) technique for quantitative noninvasive measurement of cerebral blood flow (CBF), can provide a direct measure of variations in cerebral perfusion associated with the epileptogenic zone.Aims:1. To evaluate usefulness of ASL for detecting interictal temporal hypoperfusion to localize the epileptogenic zone in patients of drug resistant mesial temporal lobe epilepsy (MTLE). 2. Correlation of localization of epileptogenic zone on ASL MR perfusion with structural MRI and EEG.Methods and Materials:30 patients with MTLE and10 age and gender matched normal controls were studied. All patients underwent ictal video EEG monitoring non-invasively, MR imaging with epilepsy protocol and pseudocontinuous ASL (PCASL) perfusion study. Relative CBF (rCBF) values in bilateral mesial temporal lobes were measured utilizing quantitative analysis of perfusion images. A perfusion asymmetry index (AI) was calculated for each region.Results:In patients, ipsilateral mesial temporal rCBF was significantly decreased compared with contralateral mesial temporal rCBF (p = 0.021). Mesial temporal blood flow was more asymmetric in patients than in normal control participants (p = 0.000). Clear perfusion asymmetry on PCASL-MRI was identified despite normal structural-MRI in 5 cases, agreeing with EEG laterality.Conclusions:Pseudo-continuous ASL offers a promising approach to detect interictal hypoperfusion in TLE and as a clinical alternative to SPECT and PET due to non-invasiveness and easy accessibility. Incorporation of ASL into routine pre-surgical evaluation protocols can help to localize epileptogenic zone in surgical candidates.

Seizures in self-limited epilepsy with centrotemporal spikes: video-EEG documentation

Self-limited epilepsy with centrotemporal spikes, formerly called benign epilepsy with centrotemporal spikes, or rolandic epilepsy, is an age-related and well-defined epileptic syndrome. Since seizures associated with rolandic spikes are infrequent and usually occur during sleep, and repetitive or prolonged EEG recording for diagnostic purposes is not necessary for diagnosis, reports of ictal video-electroencephalographic seizures in this syndrome are rare. We aimed to show ictal video-EEG of typical rolandic seizures. We report the ictal video-EEG recordings of two children with rolandic epilepsy who presented typical rolandic seizures during routine recording. Case 1: A 9-year-old boy, with normal development, had his first seizure at 8years old, characterized by paresthesia in his left face, blocking of speech, and drooling. Carbamazepine was started with seizure control. Case 2: A 10-year-old boy, with normal development, started with focal seizures during sleep, characterized by eye and perioral deviation, and speech arrest at age of 7. He started using oxcarbazepine. Both patients underwent routine electroencephalography for electroclinical diagnosis and presented a seizure. Although self-limited epilepsy with centrotemporal spikes is a very common epileptic syndrome, seizure visualization is very difficult, and these videos may bring didactical information for recognition of this usual presentation of benign childhood focal epilepsy.

A case of dyskinesia after levetiracetam administration

BackgroundAntiepileptic drug (AED) induced dyskinesia is an unusual manifestation in the medical field. In the previous case reports describing first generation-AED related involuntary movements, the authors suggested that a plausible cause is pharmacokinetic interactions between two or more AEDs. To date, development of dyskinesia after levetiracetam (LEV) has not been reported.Case presentationA 28-year-old woman with a history of brain metastasis from spinal cord glioblastoma presented with several generalized tonic-clonic seizures without restored consciousness. LEV was administered intravenously. Thereafter no more clinical or electroencephalographic seizures were noted on video-EEG monitoring, while chorea movement was observed in her face and bilateral upper limbs.Discussion and conclusionsTo our knowledge, there is no case report of dyskinesia after administration of LEV. Considering the temporal relationship and absence of ictal video-EEG findings, we suggest that development of choreoathetosis was closely associated with the undesirable effects of LEV. We propose that dopaminergic system dysregulation and genetic susceptibility might underlie this unusual phenomenon after LEV treatment.

Cases with parietal and occipital lobe epilepsies

BackgroundFor seizures emerging from the posterior cortex it can be a challenge to differentiate if they belong to temporal, parietal or occipital epilepsies. Sensoric auras like visual phenomena may occur in all of these focal epilepsies. Ictal signs may mimic non- epileptic seizures.Case presentationsCase 1: Patient suffering from a pharmacoresistent focal epilepsy. Focal seizures with sudden visual disturbance, later during the seizure epigastric aura, vertigo-nausea, involvement to bilateral tonic-clonic seizures. MEG detected interictal spikes, source localization indicated focal epileptic activity parietal right.Case 2: Patient with focal pharmacoresistent epilepsy, semiology with focal unaware seizures, feeling that something like a coat is imposed from behind on him, then feeling cold over the whole body, goose bumbs from both arms to head, then block of motoric activity, later focal unaware seizures with stare gaze, blinking of eyes, clouding of consciousness, elevation of arms and legs, sometimes tonic-clonic convulsions. EEG/MEG source localization and MRI detected an epileptogenic lesion parietal left.Case 3: Patient with pharmacoresistent focal epilepsy, focal aware seizures, a dark spot occurring in the left visual field, sometimes anxiety during seizures (leading to the suspicion of non-epileptic psychogenic pseudo seizures). MRI demonstrated an atrophy occipito-temporal right after sinus vein thrombosis. Ictal video-EEG showed a focal seizure onset occipital right.ConclusionContribution of noninvasive and/or invasive confirmation of the localization of the underlying focal epileptic activity in posterior cortex is illustrated. Characteristics of posterior cortex epilepsies are ventilated.

P.052 Benign spasms of infancy - a mimicker of infantile epileptic disorders

Background: Benign spasms of infancy (BSI), previously described as benign non-epileptic infantile spasms or benign myoclonus of early infancy, are non-epileptic movements manifesting during the first year of life and spontaneously resolving in the second year of life. BSI are characterized by spasms typically lasting 1-2 seconds, involving to varying degrees the head, neck, trunk, shoulders and upper extremities. Ictal and interictal EEG recordings are normal. BSI are not associated with developmental retardation and do not require treatment. Distinction between BSI and infantile epileptic disorders, such as epileptic spasms or myoclonic epilepsy of infancy, can be challenging given the clinical similarities. Moreover, interictal EEGs can be normal in all conditions. Epileptic spasms and myoclonic epilepsy require timely treatment to improve neurodevelopmental outcomes. Methods: We describe a 6-month old infant presenting with spasm-like movements. His paroxysms as well as a positive family history for epileptic spasms were in keeping with a likely diagnosis of West syndrome. Results: Surprisingly, ictal video EEG did not reveal epileptiform activity, and suggested a diagnosis of BSI. Conclusions: We emphasize that ictal EEG is the gold standard for classification of infantile paroxysms as either epileptic or non-epileptic, thereby avoiding overtreatment of BSI and facilitating timely targeted treatment of infantile epilepsies.

Establishment of low cost epilepsy surgery centers in resource poor setting

PurposeTo prospectively assess the feasibility of establishing low cost epilepsy surgery programs in resource poor settings. MethodWe started epilepsy surgery centers in Tier 2 and Tier 3 cities in India in private hospitals. This model is based on the identifying and operating ideal epilepsy surgery candidates on the basis of clinical history, interictal and ictal video-EEG data, and 1.5 T MRI without other investigations and without regular involvement of other specialists. Trained epileptologists formed the fulcrum of this program who identified ideal candidates, offered them counseling, and read video-EEG and MRI. We also spread epilepsy awareness among locals and physicians and established focused epilepsy clinics. The expenses were subsidized for deserving patients and policies were devised to keep video-EEG duration and staff requirement to minimum. Difficult epilepsy surgery cases were referred to established centers. Initial surgeries were performed by invited epilepsy surgeons and subsequently by local neurosurgeons. ResultsA total of 125 epilepsy surgeries were performed at three centers since 2012. This included 81(64.8%) temporal lobe resections, 26 (20.8%) extratemporal focal resections, and 13 (10.4%) hemispherotomies. Of the 93 patients with more than 1 year of postoperative followup, 86 (92.5%) had Engel class IA outcome. There were minor complications in 5% patients. Average cost of presurgical evaluation and surgery was Rs. 92,707 (USD 1,324). ConclusionsIt is possible to establish successful epilepsy surgery programs in resource poor setting with reasonable costs. This low cost model can be replicated in other parts of world to reduce the surgical treatment gap.

Ictal nausea and vomiting – Is it left or right?

PurposeIctal nausea/vomiting has been linked to the non-dominant hemisphere and has been considered a lateralizing sign. However, small case series and single cases have reported seizure localisation in the dominant hemisphere. Here we aimed to determine the seizure localisation and lateralization in cases with ictal nausea/retching/vomiting to test the hypothesis that these signs are of lateralizing value. MethodsWe searched two large tertiary epilepsy-center video-EEG databases (period: 1980–2017) for reports on ictal nausea/retching/vomiting and retrieved ictal EEG seizure location, lateralization and clinical symptoms. ResultsWe identified 13 patients with focal epilepsy and video-EEG-documented ictal nausea (n = 2), nausea and retching (n = 3), retching followed by vomiting (n = 5) or only vomiting (n = 3). Aetiology was genetic (n = 1), structural/metabolic (n = 7) or of unclear origin (n = 5). While in 12/13 patients epileptic discharges were temporal, a single case was parietal. A left-sided seizure origin was more frequent than a right-sided origin (62 vs. 38%). Assuming a left-sided language dominance in the single left-handed patient and in those two patients with unclear handedness (based on the known distribution of hemispheric speech-dominance), the distribution of ictal nausea/retching/vomiting to the dominant vs. non-dominant hemisphere was not significantly different from a random distribution (8 vs. 5, p = 0.581). ConclusionIctal nausea/retching/vomiting are most frequently of temporal origin. In contrast to previous studies, our ictal video-EEG data suggests that these signs have no lateralizing value. Thus, video-EEG should be performed and while this clinical sign points to a temporal seizure origin, it does not determine its lateralization.

Understanding Lennox-Gastaut syndrome: insights from focal epilepsy patients with Lennox-Gastaut features.

To delineate the clinical and EEG features of adults with focal epilepsy associated with a generalized paroxysmal fast activity (GPFA) pattern on EEG who developed refractory seizures, notably drop attacks, but do not fulfill the classical triad for the diagnosis of Lennox-Gastaut syndrome (LGS) and provide further insight into LGS mechanisms. Among 957 patients admitted to video-EEG monitoring between 2002 and 2015, we retrospectively research adult patients with refractory focal epilepsy, drop attacks and GPFA on EEG. We collected demographic, anamnestic, and clinical data from medical records. We reviewed for all patients the interictal and ictal video-EEG recordings. We identified ten patients with focal epilepsy and electro-clinical features of LGS. As compared to classical LGS patients, our patients: (1) began epilepsy later (15.4±8years); (2) exhibited exclusively focal onset seizures, including drop attacks seizures linked to focal asymmetrical tonic posturing seizures; (3) had a stable cognition over time and (4) evolved favourably with a good secondary response to treatments in 80% of cases. Interestingly, all patients exhibited apparent diffuse interictal and ictal EEG abnormalities but a detailed analysis revealed that 50% had asymmetrical GPFA and 70% secondary bilateral synchrony processes. We may hypothesize here that a process of "secondary LGS" occurred which produced a worsening of seizures with the apparition of drop attacks and GPFA on EEG. This study brings arguments to consider that some cases of LGS could be linked to the development of a "secondary epileptic network" driven by a primary focal epileptic zone.

Can developmental venous anomalies cause seizures?

Developmental venous anomalies (DVAs) are congenital anatomical variants of normal venous drainage of normal brain. Although DVAs are often discovered on the occasion of a seizure, their involvement in epilepsy is poorly studied. Our objective was to determine whether DVA can cause seizures, in the cases where there is no associated lesion, including no cavernoma or dysplasia. Based on clinical history, cerebral MRI, EEG recording, and 18F-FDG PET, we report 4 patients with DVA revealed by seizures. The first patient had a convulsive seizure caused by a hemorrhagic infarction due to thrombosis of her DVA. The second patient had a left temporo-parietal DVA next to a nonspecific lesion, possibly a sequelae of a venous infarction. The last two patients disclosed an isolated and uncomplicated DVA with a concordant epileptic focus confirmed on ictal video EEG recording. We reviewed literature and identified 21 other published cases of seizures caused by complications of a DVA and 9 patients that may have a direct link between epilepsy and an isolated and uncomplicated DVA. Seizures are linked to a DVA in two main situations: presence of an associated epileptogenic lesion, such as cavernoma or dysplasia, and occurrence of a complication of the DVA. Before concluding that a seizure is caused by a DVA, it is essential to perform full MRI protocols to search them. It remains rare and uncertain that isolated and uncomplicated DVA can cause seizures. In this last situation, physiopathological processes are probably different in each patient.

Preoperative prediction of temporal lobe epilepsy surgery outcome

PurposeThere is controversy about relative contributions of ictal scalp video EEG recording (vEEG), routine scalp outpatient interictal EEG (rEEG), intracranial EEG (iEEG) and MRI for predicting seizure-free outcomes after temporal lobectomy. We reviewed NIH experience to determine contributions at specific time points as well as long-term predictive value of standard pre-surgical investigations. MethodsRaw data was obtained via retrospective chart review of 151 patients. After exclusions, 118 remained (median 5 years follow-up). MRI-proven mesial temporal sclerosis (MTSr) was considered a separate category for analysis. Logistic regression estimated odds ratios at 6-months, 1-year, and 2 years; proportional hazard models estimated long-term comparisons. Subset analysis of the proportional hazard model was performed including only patients with commonly encountered situations in each of the modalities, to maximize statistical inference. ResultsAny MRI finding, MRI proven MTS, rEEG, vEEG and iEEG did not predict two-year seizure-free outcome. MTSr was predictive at six months (OR=2.894, p=0. 0466), as were MRI and MTSr at one year (OR=10.4231, p=0. 0144 and OR=3.576, p=0. 0091). Correcting for rEEG and MRI, vEEG failed to predict outcome at 6 months, 1year and 2 years. Proportional hazard analysis including all available follow-up failed to achieve significance for any modality. In the subset analysis of 83 patients with commonly encountered results, vEEG modestly predicted long-term seizure-free outcomes with a proportional hazard ratio of 1.936 (p=0.0304). ConclusionsIn this study, presurgical tools did not provide unambiguous long-term outcome predictions. Multicenter prospective studies are needed to determine optimal presurgical epilepsy evaluation.

Characteristic phasic evolution of convulsive seizure in PCDH19-related epilepsy.

PCDH19-related epilepsy is a genetic disorder that was first described in 1971, then referred to as "epilepsy and mental retardation limited to females". PCDH19 has recently been identified as the responsible gene, but a detailed characterization of the seizure manifestation based on video-EEG recording is still limited. The purpose of this study was to elucidate features of the seizure semiology in children with PCDH19-related epilepsy. To do this, ictal video-EEG recordings of 26 convulsive seizures in three girls with PCDH19-related epilepsy were analysed. All seizures occurred in clusters, mainly during sleep accompanied by fever. The motor manifestations consisted of six sequential phases: "jerk", "reactive", "mild tonic", "fluttering", "mild clonic", and "postictal". Some phases were brief or lacking in some seizures, whereas others were long or pronounced. In the reactive phase, the patients looked fearful or startled with sudden jerks and turned over reactively. The tonic and clonic components were less intense compared with those of typical tonic-clonic seizures in other types of epilepsy. The fluttering phase was characterised initially by asymmetric, less rhythmic, and less synchronous tremulous movement and was then followed by the subtle clonic phase. Subtle oral automatism was observed in the postictal phase. The reactive, mild tonic, fluttering and mild clonic phases were most characteristic of seizures of PCDH19-related epilepsy. Ictal EEG started bilaterally and was symmetric in some patients but asymmetric in others. It showed asymmetric rhythmic discharges in some seizures at later phases. The electroclinical pattern of the phasic evolution of convulsive seizure suggests a focal onset seizure with secondary generalisation. Based on our findings, we propose that the six unique sequential phases in convulsive seizures suggest the diagnosis of PCDH19-related epilepsy when occurring in clusters with or without high fever in girls. [Published with video sequences online].

The early electroclinical manifestations of infantile spasms: A video EEG study.

Purpose:Infantile spasms are described as flexor extensor and mixed; but more features of their semiology and ictal electroencephalography (EEG) changes are sparse in the literature. The purpose of the study was to describe the clinical and ictal video-EEG characteristics of consecutive cases with infantile spasms and to try to find an association with the etiology.Materials and Methods:The clinical phenomenology and EEG characteristics on video-EEG were analyzed in 16 babies with infantile spasms.Results:A total of 869 spasms were reviewed. Nine (56.3%) showed focal seizures at least once during the recording and 1 (6.3%) had multifocal myoclonus in addition to the spasms. The duration of the cluster and interval between spasms was totally variable in all patients. Lateralizing phenomena were present in at least some of the spasms in all patients. Unilateral manual automatism in the form of holding the pinna was noted in three patients following the spasm. The ictal EEG activity in the majority (75%) was the slow wave. Four (25%) showed fast generalized spindle-like ictal discharges. Spikes, spike and wave activity, or electrodecremental pattern alone during the ictus was seen in none. On bivariate analysis, no factor noted on the video EEG had association with the etiology.Conclusion:Infantile spasms could be associated with focal and other seizures, has unique, non-uniform and variable semiology from patient to patient. The ictal EEG manifestation in the majority (75%) of our patients was the slow wave transient with 25% showing generalized fast spindle-like activity.

Seizure characteristics of epilepsy in childhood after acute encephalopathy with biphasic seizures and late reduced diffusion.

The aim of this study was to clarify characteristics of post-encephalopathic epilepsy (PEE) in children after acute encephalopathy with biphasic seizures and late reduced diffusion (AESD), paying particular attention to precise diagnosis of seizure types. Among 262 children with acute encephalopathy/encephalitis registered in a database of the Tokai Pediatric Neurology Society between 2005 and 2012, 44 were diagnosed with AESD according to the clinical course and magnetic resonance imaging (MRI) findings and were included in this study. Medical records were reviewed to investigate clinical data, MRI findings, neurologic outcomes, and presence or absence of PEE. Seizure types of PEE were determined by both clinical observation by pediatric neurologists and ictal video-electroencephalography (EEG) recordings. Of the 44 patients after AESD, 10 (23%) had PEE. The period between the onset of encephalopathy and PEE ranged from 2 to 39months (median 8.5months). Cognitive impairment was more severe in patients with PEE than in those without. Biphasic seizures and status epilepticus during the acute phase of encephalopathy did not influence the risk of PEE. The most common seizure type of PEE on clinical observation was focal seizures (n=5), followed by epileptic spasms (n=4), myoclonic seizures (n=3), and tonic seizures (n=2). In six patients with PEE, seizures were induced by sudden unexpected sounds. Seizure types confirmed by ictal video-EEG recordings were epileptic spasms and focal seizures with frontal onset, and all focal seizures were startle seizures induced by sudden acoustic stimulation. Intractable daily seizures remain in six patients with PEE. We demonstrate seizure characteristics of PEE in children after AESD. Epileptic spasms and startle focal seizures are common seizure types. The specific seizure types may be determined by the pattern of diffuse subcortical white matter injury in AESD and age-dependent reorganization of the brain network.

P160 – 2423: Idiopathic ocular neuromyotonia in a child: Case report

Objective To describe clinical features and course of a child with ocular neuromyotonia (ONM), a rare ocular syndrome that causes ocular paroxysms because of an abnormal delay in extra ocular muscle relaxation. Methods A case report is presented to demonstrate clinical characteristics and treatment of ONM. Results A 4.5 year-old old girl was admitted with transient painless, unilateral spontaneous abnormal ocular deviation which started one week ago. The episodic spell duration was 30 to 40 seconds and occurred nearly 10 to15 times a day. She had preserved awareness during the paroxysms. She did not have headache, fever or any systemic or neurological symptoms. Her neurological examination, in between the ocular paroxysms, was normal. The MRI and MR angiography of brain and cervical cord was normal. Since the ictal video EEG was normal, epileptic seizure was excluded. Her spells characterized by spontaneous inward shift of left eye together with ptosis during 30 to 40 seconds without any nystagmoid movement, pupillary defect and change in consciousness. She was diagnosed as ONM. Her blood investigations, including haematological, biochemical, endocrine function tests and paraneoplastic as well as detailed vasculitic workup, were normal. Cerebrospinal fluid analysis for immunological markers, immunoglobulin (Ig) G index and oligoclonal band was all negative. The voltage-gated potassium channel (VGKC) antibody could not be detected in serum. Therefore we could not find an underlying abnormality to account for ONM. Treatment with carbamazepine 10 mg/kg twice a day was started and the attacks were completely resolved. Conclusion After conducting a search of the medical literature using the terms “ONM” and “child” we identify only 5 previously published reports of ONM in children. To the best of our knowledge this patient is the youngest patient reported with a diagnosis of ONM.

PP14.1 – 2490: Ictal video-EEG studies in Caucasian children with hot water reflex epilepsy

Objective Hot water is an exceptional stimulus for reflex epilepsies. Our objective is to present two cases of children with clinical features/seizures compatible with hot water epilepsy. Methods Cases description providing ictal video-EEG. Results Two Caucasian 13 month-old children, with uneventful birth and normal development, presented bouts of paleness, perioral cyanosis, hypotonia and loss of consciousness for a few minutes during bathing. Cardiac examination and cerebral MRI were normal in both. Interictal EEG showed normal background activity during awakening and sleep with no abnormal discharges in both of them. Seizures were provoked in the EEG laboratory with a small tub. Different stimulus (auditory, blowing, rubbing, cold water) did not provoke changes. Trial with lukewarm water and pouring it over his head and neck triggered the seizures: in the first case, and after a face tactile stimulus with a sponge, the seizure started with perioral subtle colour changes, followed by head drop, with an abrupt change in the EEG at the same time (3–4 Hz pattern of slow-voltage waves started above the temporal region, with fast spreading in the next 30 seconds). Slowing of the background during loss of axial tone and unconsciousness with prolonged staring for 90 seconds followed it. In the second case, seizure manifestation were staring and immobility, correlated with an abrupt change in the EEG (4Hz pattern of high-voltage waves above bitemporal region for 10 seconds, with abrupt normalization subsequently). They remained asymptomatic with valproate and changes in the method of bathing. Conclusions Bathing epilepsy is a rare form of reflex epilepsy with complex triggering stimulus. A combination of tactile and thermal stimulus is revealed in the first case. The exact physiopathology remains unknown. The prognosis is favorable, because of the good response to pharmacological treatment and the tendency towards spontaneous remission.

Complete seizure freedom is possible in patients with MTLE-HS after surgery in spite of extratemporal electro-clinical features

The aim was to identify the common typical and atypical ictal semiologic and EEG features of patients with mesial temporal lobe epilepsy and hippocampal sclerosis who became seizure-free postoperatively. The semiologic and EEG findings of 126 ictal video-EEG recordings of 50 patients who remained seizure-free for at least 2 years (mean=9.46±3.20; range=3-14.5 years) after surgery were reviewed. Statistical analysis was used to determine the frequencies of the most common auras, semiologic features and EEG patterns and to define the symptom clusters. Aura was reported in 82% (n:41) of patients and the most common type was epigastric sensation. Early symptoms were contralateral upper extremity dystonic posturing (66%), oro-alimentary automatisms (50%) and ipsilateral hand automatisms (40%). Ictal EEG findings revealed localized and lateralized activity in 62% of patients. Of the 17 most common early symptoms, there was a tendency toward two clusters. Cluster 2 consisted of findings that were typical for patients with mesial temporal lobe epilepsy whereas Cluster 1 mostly consisted of atypical findings. This study demonstrated two clusters of the semiologic repertoire, and the most frequent EEG finding was lateralized and localized ictal activity, while other types of EEG patterns were also recorded. Although the presence of atypical findings may suggest a more extended or even extratemporal zone, the seizure-free outcome demonstrated that if the critical amount of tissue can be resected within the epileptogenic network, this may be sufficient for termination of seizures.

Lateralization of epileptic foci through causal analysis of scalp-EEG interictal spike activity.

The purpose of this work was to find an objective and quantitative method for epileptic foci lateralization that may aid and support the decision made by the usually chosen lateralizing criteria. A frequency-derived causal analysis method, full frequency adaptive directed transfer function, was applied to the interictal scalp EEG data collected from 2 groups of total 30 epileptic patients. Group A included 15 refractory epileptic patients who had undergone an epileptic surgery; group B consisted of 15 outpatient epileptic patients who had a seizure type of generalized tonic-clonic seizure. All patients had no abnormalities detected in their routine brain MRI examinations. The methodology that leads to the lateralization is based on several processing steps: interictal spikes selection, full frequency adaptive directed transfer function analysis, significant causal connections extraction, and then lateralization. Ninety-six of 104 spikes' sources lateralized by full frequency adaptive directed transfer function were consistent with their corresponding surgery side in group A, with the accuracy of 92.31%. Ninety-seven of 101 spikes' sources were lateralized correctly in group B, according to technicians' judgments from ictal scalp video-EEG recordings, with the accuracy of 96.04%. The results demonstrated the feasibility of correctly lateralizing the epileptic foci by using full frequency adaptive directed transfer function to scalp EEG interictal spike activity.