Focal Spikes Research Articles

Utility of Various Activation Procedures in Provoking Ictal and Interictal Patterns, during Routine Electroencephalogram (rEEG) Recording.

Activation procedures (APs) are adopted during routine electroencephalography (rEEG) to provoke interictal epileptiform abnormalities (EAs). This study aimed to observe interictal and ictal (EAs) of different EEG patterns, provoked by various APs. This cross-sectional study was performed in the neurology department of King Fahd hospital of university, Saudi Arabia. The EEGs and medical records of patients who presented for EEG recordings were screened initially, then 146 EEGs provoked EAs due to utilization of APs, were included for analysis. Among all EEGs with provoked EAs, Non-rapid eye movement sleep (NREM) provoked EAs in 93 (63.7%) patients with following patterns, focal spike wave discharges (FSWDs) 45 (P= 0.01), focal spike wave discharges with bilateral synchrony (FSWDBS) 27 (P=0.03) and generalized spike wave discharges (GSWDs) 46 (P=0.01). Intermittent photic stimulation (IPS) most significantly provoked FSWDs in 07 patient (P =0.01) and GSWDs in 30 patients (P=<0.001) 7 patients (P = 0.01) and GSWDs in 30 patients (P < 0.001). Hyperventilation (HV) was associated with a higher occurrence of GSWDs in 37 patients (P =0.01). Female sex 7 (P = 0.02), provoked GSWDs 3 (P = 0.03), NREM sleep 8 (P = 0.04), prolonged EEG record 3 (P = 0.02), clinical events during recording 5 (P ≤ 0.01), diagnosis of genetic 05 (P = 0.03), and immune-mediated epilepsies 2 (P = 0.001) were associated with the provocation of ictal EAs; however, in multiple logistic regression analysis, no statistically significant association of these variables (P ≥ 0.05 each) was noted. The provocation of EAs in rEEG with different APs varies according to circumstances, including seizure types, epilepsy etiology, and the type of AP applied. These clinical and procedural parameters affect the diagnostic yield of rEEG and need careful consideration during rEEG recordings. APs adopted during rEEG recording can induce FSWDs, FSWDBS, and GSWDs in the form of either interictal or ictal EAs in various etiologies of epilepsy. Ictal EAs may appear in the form of GSWDs, during NREM sleep, in prolonged EEG records; however, their independent association needs to be evaluated in larger sample studies. Further, prospective cohort studies with adequate sample sizes are warranted.

Epileptiform Discharges Recorded as Monopoles and Dipoles in the EEG: A Practical Approach to Differentiate Tangential and Radial Generators Using Electrode Position Versus Voltage Graphs.

Differentiating dipoles (tangential generators) from monopoles (radial generators) in routine EEG reading can be difficult. The polarity of sharp waves seen on surface EEG will change depending on the generator being located at the wall of the sulcus versus the crown of a gyrus. In this article, the authors introduce visual rules that may be used to determine polarity and estimate the localization of potentials during analysis of the EEG. They also review a practical approach to differentiate monopoles (radial generators) from dipoles (tangential dipoles) in the surface EEG using "electrode position versus voltage graphs." Finally, the authors illustrate examples of dipoles and monopoles with focal spikes located in the following locations: (1) bipolar spikes located in the anterior bank of the central sulcus, (2) bipolar spikes located in the posterior bank of the central sulcus, (3) monopolar spikes located in the crown of the precentral gyrus, (4) bipolar spikes with a vertically oriented dipole originated within the temporal (inferior) bank of the Sylvian fissure, and (5) monopolar spikes located in the convexity of a temporal gyrus. In summary, this article discusses electrographic features of spikes localized in various fissures and gyri and provides practical rules that permit the identification and location of dipoles and monopoles in standard scalp EEG recordings.

Childhood-onset epilepsy in patients with dyskinetic cerebral palsy caused by basal ganglia and thalamic lesions

ObjectiveTo elucidate the incidence and outcomes of childhood-onset epilepsy and associated factors in term-born patients with basal ganglia and thalamic lesion (BGTL)-induced dyskinetic cerebral palsy (DCP) caused by perinatal hypoxic-ischemic encephalopathy (HIE). MethodsWe studied 104 term-born patients with BGTL-induced DCP (63 males and 41 females, aged 2–22 years) to investigate the incidence of epilepsy and the factors related to its development. We used multivariate analysis to assess perinatal factors, gross motor function, and the extent of brain lesions. We also investigated the seizure onset, clinical course, and electroencephalography (EEG) characteristics. ResultsThe cumulative epilepsy incidence was 36%. Multiple logistic regression analysis revealed that deep white matter lesions were the only independent risk factor for epilepsy. The confirmed seizure types included epileptic spasms (ES, n = 13), myoclonic seizures (MS, n = 6), and focal-onset seizures (FS, n = 24). Only patients with deep white matter lesions exhibited ES or MS. The symptoms of FS resembled those of self-limited epilepsy with centrotemporal spikes; however, only half of the patients reached remission by the time of investigation, and four patients had more than one seizure per month despite appropriate drug therapy. Focal spikes in the peri-rolandic area were detected not only in patients with FS but also in half of the patients without epilepsy. ConclusionsOne-third of term-born patients with BGTL-induced DCP caused by perinatal HIE develop epilepsy, and deep white matter lesions increase the likelihood of epilepsy. Preparation for early-onset ES, MS, and subsequent FS is beneficial.

Focal tonic seizures with asymmetrical posturing could allow voluntary movements: a lesson to not be misled for a non-epileptic event.

This report documents the clinical features of supplementary motor area seizures with voluntary movements in two patients. The first case describes a 13-year-old boy with a 2-year history of nocturnal seizures, characterized by an asymmetrical brief tonic posture followed by bilateral rapid hand shaking, but without impaired awareness. Magnetic resonance imaging revealed no abnormalities. Video electroencephalogram indicated interictal focal spikes and ictal activity 2 s before clinical onset in the frontal midline area. The patient stated that he purposely shook his hands to lessen the seizure-induced upper limb stiffness. The second case describes a 43-year-old man with a 33-year history of nocturnal seizures, characterized by an asymmetric brief tonic posture, with the right hand grabbing to hold this posture, but without impaired awareness. Video electroencephalogram indicated that he voluntarily moved his right hand during the latter part of the seizures; however, no clear ictal electroencephalogram change was noted. Magnetic resonance imaging revealed a mass lesion in the right medial superior frontal gyrus. Fluorodeoxyglucose-positron emission tomography and ictal single-photon emission computed tomography indicated ictal focus in the mesial frontal area, as confirmed by invasive electroencephalogram and seizure freedom after surgery. Both patients had typical supplementary motor area seizures, except they could perform voluntary movements in the body parts. The co-occurrence of supplementary motor area seizures and voluntary movements is clinically useful, as it may help avoid the inaccurate and misleading diagnosis of non-epileptic events such as psychogenic non-epileptic seizures.

Peri-ictal EEG in infants with PRRT2-related self-limited infantile epilepsy.

Pathogenic PRRT2 variants cause self-limited (familial) infantile epilepsy (SeLIE), which is responsive to sodium channel blocking antiseizure medications. The interictal EEG is typically normal. We describe a cohort of infants with PRRT2-related SeLIE with striking peri-ictal EEG abnormalities. We included all infants diagnosed with PRRT2-related SeLIE during July 2020 to November 2021 at the Royal Children's Hospital, Melbourne. Clinical features and results of aetiologic investigations were collected from electronic medical records. All EEGs were reviewed independently by two epileptologists. Ten infants presented with focal seizures at a median age of 5 months (range: 3-6 months). Eight had a family history of epilepsy, paroxysmal kinesigenic dyskinesia (PKD) or hemiplegic migraine. Seven of the eight infants with an EEG performed within 24 h of the most recent seizure had epileptiform discharges. Their EEGs showed focal sharp waves, spikes, polyspikes or fast activity independently over the left and right temporo-occipital regions. Conversely, the two infants with last known seizure greater than 24 h prior to their EEG had no epileptiform discharges. Oxcarbazepine was commenced in two infants and was effective. Eight infants were initially treated with levetiracetam, and all were subsequently switched to oxcarbazepine due to ongoing seizures or side effects. Posterior polymorphic focal epileptiform discharges on a peri-ictal EEG recording are a feature of PRRT2-related SeLIE. This finding, particularly in the presence of a family history of infantile epilepsy, PKD or hemiplegic migraine, suggests a diagnosis of PRRT2-related SeLIE and has important treatment implications.

Role of EEG as a monitoring tool in patients with glucose transporter type I deficiency syndrome (GLUT1-DS) on ketogenic diet.

Glucose transporter type I deficiency syndrome (GLUT1-DS) is the fourth most frequent single-gene epilepsy refractory to standard antiepileptic drugs. Multiple seizure types and variable electrographic findings are reported. Ketogenic diet is expected to result in the complete resolution of the epileptiform activity. A retrospective chart review of patients with GLUT1-DS on ketogenic diet between December 2012 and February 2022 was done. Analysis of the EEGs prior to and during the ketogenic diet was done. 34 patients on ketogenic diet were reviewed. Ten had clinical diagnosis of GLUT1-DS, and seven of them had genetic confirmation. 71% were female. The average age at seizure onset was 13.85 m.o. (range: 3-60, SD ±20.52), at diagnosis was 44.57 m.o (range: 19-79), and at the onset of ketogenic diet was 46.43 m.o. (range: 20-83). 29 months (range: 13-38) delay between symptoms onset until diagnosis was noticed. At the diagnosis 100% reported seizures: 71% myoclonic, 57% generalized motor, 57% absence, 28% atonic, and 14% focal motor. Also, 71% abnormal eye movements, 57% ataxia, and 28% intolerance to fasting. 86% had normal brain MRI. 71% had abnormal EEG. All were on ketogenic diet, and four on classical (1.75:1-2.25:1 ratio). Six were clinically seizure-free after the ketogenic diet. EEG features included notch delta, focal spike and wave, and generalized spike/polyspike and wave. One patient had bilateral independent centrotemporal spikes. Spikes showed high and very high amplitude in all of them (>200 μV). The variation of the spike index decreased in three patients but increased in two. Ketogenic diet is the choice treatment for patients with GLUT1-DS. Electrographic features could show worsening after initiation of the ketogenic diet even with seizure control. EEG did not prove to be a reliable tool for adjusting KD in our cohort. Centrotemporal spikes have not been reported in patients with GLUT-1 DS.

Acute Corticosteroid Responsive Meningoencephalitis with Cerebral Vasculitis after COVID-19 Infection in a Thirteen-Year-Old Case Report.

Various neurologic manifestations have already been described in children during or after severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infections. The central nervous system disorders reported in children are mainly encephalopathies during multisystem inflammatory syndrome. We present here an acute meningoencephalitis with cerebral vasculitis associated to a coronavirus disease 2019 (COVID-19) infection in a 13-year-old girl with a 1-year clinical, electroencephalogram (EEG), and magnetic resonance imaging (MRI) follow-up. A 13-year-old girl presented acute symptoms of consciousness impairment, frontal headache, hyperthermia, and aphasia, with moderate lymphopenia (900/mm3), elevated C-reactive protein (17 mg/L), cerebrospinal fluid (CSF) pleocytosis (15 cells/mm3), slow background with frontal focalization on EEG, a left frontal ischemic lesion, leptomeningeal enhancement, and bilateral limbic fluid-attenuated inversion recovery hyperintensity on cerebral MRI. Reverse transcription-polymerase chain reaction for SARS-CoV-2 was positive in nasopharyngeal swab and COVID serology was positive for immunoglobulin (Ig) M and G, whereas extensive autoimmune antibody investigation was negative except for a positive low titer of anti-myelin oligodendrocyte glycoprotein in CSF and blood. The diagnosis of probable encephalitis associated to cerebral vasculitis after COVID infection was suggested and steroids pulse were started. She recovered within a few days. Six months later, she had moderate clinical sequels including persistent intermittent headaches, an isolated spatial deficit, and focal spikes on the EEG without argument for epilepsia. A teenager without previous medical history presented with acute encephalitis with leptomeningitis and vasculitis after a recent COVID-19 infection. Steroids pulse therapy allowed clinical improvement. Cerebral MRI and EEG helped diagnosis, follow-up of the encephalitis, and evolution after treatment.

Clinical and electrophysiological pattern of startle epilepsy: a comprehensive literature review

Startle epilepsy is a rare form of epilepsy characterized by recurrent seizures that are triggered by a surprising stimulus. It affects children between 10 months and 14 years of age, with a history of brain damage due to ante- or neonatal hypoxia, and hemiparesis. The most common triggers for these seizures are auditory stimulation, followed by somesthetic stimulation and visual stimulation. Typical startle seizures are rare, and they have a large variety of clinical manifestations. The most frequent type is short hemitonic flexion seizures, followed by myoclonic seizures, and bilateral tonic seizures.&#x0D; Few studies have focused on the electrophysiological features of this condition and the literature is controversial regarding the EEG pattern. Interictal EEG is often normal. The most frequent interictal abnormalities are a slowing of the background rhythm, associated with focal spikes in the frontal, central, and parietal regions. Several ictal EEG patterns have been reported: diffuse flattening of the background rhythm, focal fast activity, an isolated focal spike followed by a discharge of spikes. Intracerebral EEG recording shows primary and secondary motor cortex involvement as initial discharge areas. The most efficient antiepileptic drugs are Lamotrigine, Oxcarbazepine, and benzodiazepines. But most patients are often drug-resistant. Surgery is a possible albeit rare therapeutic option.&#x0D; We describe the case of a 13-year-old female patient with hemitonic seizures triggered by surprising auditory stimuli. The video EEG allowed us to record and define an EEG pattern of her seizures. Interictal EEG showed right fronto-central abundant spikes. The ictal EEG showed right fronto-central and medial spikes followed by a fronto-central fast rhythm discharge. The clinical and electrographic data permitted the diagnosis of startle epilepsy. Our patient was treated with Levetiracetam during 6 months followed by Oxcarbazepine and Clobazam with a partial improvement (reduction of the frequency of focal to bilateral tonic-clonic seizures). Considering the usual pharmaco-resistance of this type of epilepsy, we proposed our patient a presurgical evaluation. This case shows that the diagnosis should be considered in paroxysmal dystonic movements with a stereotyped triggering factor, and underlines the importance of repeating the EEG recordings in this type of epilepsy. In this article we will review the literature, discuss the underlying pathophysiology, and identify the common electrophysiological characteristics associated with startle epilepsy.

Focal low amplitude periodic spikes on interictal scalp EEG and intracranial EEG for localizing epileptogenic foci

Focal low amplitude periodic spikes on interictal scalp EEG and intracranial EEG for localizing epileptogenic foci

Clinical and electroencephalography characteristics of 41 children with epileptic spasms onset after 1 year of age

The incidence of epileptic spasms (ES) that begin after the first year of life is much lower than that before 1 year of age. The aim of this study was to identify clinical and electroencephalography (EEG) characteristics, etiologies, treatments, and prognoses in pediatric patients with ES onset after 1 year of age. Forty-one children were retrospectively identified in Children's Hospital of Chongqing Medical University between January 1, 2020 and December 1, 2021. ES onset after 1 year of age have diverse presentations. Although most occur in clusters, are symmetrical and flexional, and occur frequently during awakening, some are characterized as isolated and asymmetrical, have a tonic component, and can also occur during sleep. The hypsarrhythmia variants and focal or multifocal discharges occur alternately in the interictal period, and the focal spikes and slow waves predominated in the unilateral temporal or frontotemporal areas. These patients had diverse etiologies, including structural (51.2 % of patients) and genetic (22.0 %) ones, and 11 patients (26.8 %) had an unknown etiology. No patients in our study had an infectious or immune-mediated etiology. Forty-eight percent of patients responded to hydrocortisone and/or adrenocorticotropic hormone. The efficacy of antiepileptic drug therapy was lower in patients who did not receive concurrent steroid therapy. However, ES onset after 1 year of age caused by a tumor, brain malformation, or other focal lesions, may be cured by focal cortical resection despite a lack of clearly localized EEG surface anomalies. Delays in motor, language, and cognitive development, or behavioral problems were observed in all but three patients.

Electroencephalographic findings after convulsive seizures due to cerebral arterial air embolism secondary to lung cancer: a case report

BackgroundCerebral arterial air embolism is often associated with an invasive iatrogenic etiology and a high rate of convulsive seizures. There are only a few descriptions of electroencephalogram findings in convulsive seizures due to cerebral arterial air embolism of noniatrogenic etiology. Herein, we describe the case of a patient with lung cancer and convulsive seizures with abnormalities detected on electroencephalogram caused by cerebral arterial air embolism of noniatrogenic etiology.Case presentationA 55-year-old Japanese man underwent radiotherapy and chemotherapy for cancer in the hilum of the left lung that was diagnosed after hemoptysis. One year after the diagnosis, he developed fever and chest pain that required hospitalization. At admission, he was in shock, and chest computed tomography revealed invasion of the left atrium and left main bronchus by the hilar cancer. Chest and abdominal computed tomography revealed small low-density areas within the tumor and around the intestinal membrane, which were interpreted as the presence of air due to invasion of the lung cancer. He was diagnosed with septic shock due to necrotic infection secondary to cancer invasion into the left atrium. The following day, he complained of difficulty in speaking and weakness in the left side of his body. A head computed tomography scan revealed multiple small low-density areas in the right cortex and bilateral subcortex, which were interpreted as air emboli. On day 3, he experienced generalized tonic–clonic seizures for approximately 1 minute, followed by myoclonus-like convulsions in the left lower limb and a right-sided gaze. The electroencephalogram findings after the convulsive seizures revealed partial epilepsy-like waves with intermittent spikes in the bilateral cerebral hemispheres and a diffuse slow wave in the left frontal lobe. He recovered from sepsis without recurrence of convulsive seizures; however, he died of hemoptysis on day 50 after discharge.ConclusionsElectroencephalogram findings of focal spike activities and diffuse slow waves were detected in early seizures due to cerebral arterial air embolism of noniatrogenic etiology associated with lung cancer. Additional case descriptions are warranted to establish patterns in electroencephalogram findings specific to cerebral arterial air embolism.

Morphological Audit of glomerular diseases in a tertiary care Hospital.

Background: Glomerular diseases are a universal health issue, and Pakistan ranks eighth in renal disease, causing 20,000 deaths every year. Renal biopsy is a valuable diagnostic tool and provides prognostic insight and therapeutic plans. The study's objective is to determine the frequency and pattern of various glomerulopathies concerning age and gender. Methodology: A cross-sectional study was performed at the Department of Pathology, Ziauddin Medical University, and Hospital, Karachi, from 19th December 2018 to 15th April 2019. A total of 91 patients were included of either gender with a clinical history of glomerulonephritis. All biopsy samples were analyzed by using light and immunofluorescence microscopy techniques. Patient data were compiled and analyzed through SPSS version 20.0. Results: A total of 91 cases of the renal biopsy were evaluated; 51.6% of these cases were male patients. Forty-nine (53.8%) cases were reported as focal segmental glomerulosclerosis, 35(38.5%) were diagnosed with membranous glomerulonephritis, and 7(7.7%) were reported as mesangiocapillary glomerulonephritis. In membranous glomerulonephritis, 7.7% were pre-spike membranous glomerulonephritis. In the current study, 35.2% of cases showed diffuse glomerular basement membrane (GBM) thickening with spikes, and 4.4% showed GBM thickening with focal spikes on silver stain. Conclusion: Glomerular diseases are slightly more prevalent in males than females. The most commonly diagnosed primary glomerulonephritis was focal segmental glomerulosclerosis, followed by membranous glomerulonephritis.

EEG abnormalities indicating the genetic determination of epilepsies

Genetic and acquired factors contribute to epileptogenesis in all epilepsy syndromes and patients. A comprehensive evaluation of both components of etiology is essential in every patient. In this review, the authors enumerate the EEG abnormalities indicating the genetic determination of epilepsy. The authors briefly describe generalized spike-and-wave paroxysms, focal spikes and sharp waves, photosensitivity, 4-7 Hz theta rhythm, 2-4 Hz occipital intermittent rhythmic delta activity, phi rhythm, and generalized monomorphic alpha EEG background activity. For each abnormality, the authors review prior knowledge and add recent research results. The neurophysiological meaning of the abnormalities, age distribution, the relationship to epilepsy, the role of the EEG patterns in epileptogenesis, clinical presentation, and prognosis in the individual patient are the main aspects of description and discussion. These EEG abnormalities may shape the typical, syndrome-specific phenotype, forecast severity of the disease and difficulty of treatment or pharmacoresistance, and modify the syndrome-specific general prognosis of the syndrome. Relationship to epilepsy and heritability data of the EEG abnormalities can help to estimate the risk of epilepsy in the offspring. Epilepsy syndromes are taxonomic diagnostic categories that might be enriched with neurobiological meaning and heritability relations.

Epilepsy and Electroencephalogram Characteristics in Children with Neurofibromatosis Type 1, What We Have Learned from a Tertiary Center Five Years' Experience.

Introduction: Neurofibromatosis type 1(NF-1) is the commonest neurocutaneous phacomatosis in children. Epilepsy is an infrequent comorbidity. Reports of seizure and Electroencephalogram (EEG) characteristics in children are sparse. Methods: A retrospective review was performed on patients with NF-1 seen between 2016–2020. Patients with co-existing epilepsy were identified. Demographic, clinical, radiological and neurophysiological data were reviewed and analyzed. Results: Out of 118 children with NF1, 16 had epilepsy. 11 patients had focal onset seizures, whereas 5 had generalized onset seizures. Most patients had easy seizure control. Focal epileptiform discharges were the most prevalent EEG abnormality. There was no significant correlation between seizure patterns and presence of intracranial tumors. Conclusion: Epilepsy is a relatively uncommon in pediatric NF-1. Seizures are often of focal semiology and likely to be easily controlled. Focal and multifocal spike epileptiform discharges are the typical interictal EEG findings. Correlation of clinical and EEG findings with intracranial lesions is poor.

Epilepsy with myoclonic-atonic seizures, also known as Doose syndrome: Modification of the diagnostic criteria.

The aim of this review is to propose the updated diagnostic criteria of epilepsy with myoclonic-atonic seizures (EMAS), which is a recent subject of genetic studies. Although EMAS has been well known as Doose syndrome, it is often difficult to diagnose due to a lack of consensus regarding some of the inclusion criteria. Along with progress in molecular genetic study on the syndrome, it becomes important to recruit electroclinical homogeneous EMAS patients, hence the validity of the clinical criteria should be verified based on recent clinical researches. At present, the most updated ILAE diagnostic manual of EMAS includes: (1) normal development and cognition before the onset of epilepsy; (2) onset of epilepsy between 6 months and 6 years of age (peak: 2-4 years); (3) myoclonic-atonic seizures (MAS) are mandatory (4) presence of generalized spike-wave discharges at 2-3Hz without persistent focal spike discharges; and (5) exclusion of other myoclonic epilepsy syndromes. In the criteria, we should emphasize that the age at onset of MAS is between 2-5 years in (2), presence of myoclonic-atonic, atonic or myoclonic-flexor seizures (MASs) causing drop attacks associated with generalized spike-wave discharges is mandatory in (3), and epileptic spasms causing drop attacks must be excluded in (5). In the modified criteria, I propose that EMAS is redesignated as genetic generalized epilepsy with MASs, consistent with the familial genetic study conducted by Doose and the recent identification of candidate genes. It should also be noted that EMASs evolves to transient or long-lasting epileptic encephalopathy.

Auditory stimulation during sleep suppresses spike activity in benign epilepsy with centrotemporal spikes

SummaryBenign epilepsy with centrotemporal spikes (BECTS) is a common form of childhood epilepsy linked to diverse cognitive abnormalities. The electroencephalogram of patients shows focal interictal epileptic spikes, particularly during non-rapid eye movement (NonREM) sleep. Spike formation involves thalamocortical networks, which also contribute to the generation of sleep slow oscillations (SOs) and spindles. Motivated by evidence that SO-spindle activity can be controlled through closed-loop auditory stimulation, here, we show in seven patients that auditory stimulation also reduces spike rates in BECTS. Stimulation during NonREM sleep decreases spike rates, with most robust reductions when tones are presented 1.5 to 3.5 s after spikes. Stimulation further reduces the amplitude of spikes closely following tones. Sleep spindles are negatively correlated with spike rates, suggesting that tone-evoked spindle activity mediates the spike suppression. We hypothesize spindle-related refractoriness in thalamocortical circuits as a potential mechanism. Our results open an avenue for the non-pharmacological treatment of BECTS.

Temporal Onset Focal Seizures Induced by Intermittent Photic Stimulation.

The study aimed to review the clinical, radiological, and pathological findings and electroencephalogram (EEG) of pediatric epilepsy patients with temporal onset focal seizures induced by intermittent photic stimulation (IPS). Four patients with temporal onset photosensitivity focal seizures were analyzed. Three (75%) of the four patients were female. The average age of seizure-onset was 4.4 years. The interictal EEG showed both generalized and focal spike and waves in one patient and focal or multifocal spike and waves alone in three patients. Photoparoxysmal response (PPR) was evoked in all patients and showed generalized discharges (patients 2–4), both generalized and posterior discharges (patient 1). Both generalized and focal discharges could coexist in interictal discharges and PPR. The sensitive frequencies of PPR and photoconvulsive response (PCR) were 12–30 and 10–16 Hz, respectively, which were close to the occipital rhythm. In all patients, the onset of PCR was recorded, namely, the left anterior and mesial temporal lobe (TL), the left posterior TL, and the whole left TL, which showed two forms: the seizure of two patients was the onset of slow waves in the temporal area without spreading generalized discharges (patients 1 and 4), and the other one was fast rhythmic activities in the temporal area, spreading to the occipital area or gradually evolving into the generalized discharges (patients 2 and 3). During follow-up, except for patient 3 who had occasional seizures, the seizures of the remaining patients were under control. Temporal onset focal seizures could be induced by IPS. Temporal onset photosensitivity seizures were mostly easy to control with antiseizure drugs.

Neonates with the KCNQ2 Y755C Variants: Not Associated with Neonatal Epileptic Encephalopathy

Background: Pediatric epilepsy caused by a KCNQ2 gene mutation usually manifests the phenotype of a neonatal seizure. KCNQ2 encephalopathy in newborns continues to be reported on. Objectives: The exact mechanism and phenotype of the KCNQ2 mutation still require investigation. Methods: One hundred twenty-one patients with childhood epilepsy without an identified cause underwent KCNQ2 sequencing. KCNQ2 mutation variants were transfected into human embryonic kidney 293 (HEK293) cells to investigate functional changes. Results: Two patients with the c.2264G&gt;G/A (p.Y755C) variant had neonatal epileptic encephalopathy: one had electroencephalography (EEG) burst suppression and the other had multiple focal spikes. However, the mutation was not found in the 80 healthy adult claiming without ever seizures before. A functional study showed that p.Y755C currents were not different from those in the wild-type and from those in the benign (p.N780T) polymorphism in homomeric and heteromeric (wild-type KCNQ2: mutant = 1:1) transfected HEK293 cells. Electrical current differences between HEK293 cells with wildtype mutations and cells transfected with the wild-type KCNQ2, KCNQ3, and p.Y755C mutations in a 1:2:1 ratio were not significant. Their seizures remitted after they turned 1 year old. Conclusion: We suggest that patients with the KCNQ2 p.Y755C mutations are not associated with neonatal epileptic encephalopathy

Vascular Epilepsy

Seizures can be a presenting feature of acute stroke. They may complicate its early clinical course or be a late complication or sequel. The incidence of combined “early” and “late” seizures after stroke has been reported to be approximately 10% and the prevalence is of 2-4%. Mechanisms leading to post stroke seizures and chronic epilepsy, in both ischemic and hemorrhagic strokes, are diverse. Focal slowing or diffuse slowing activities, rhythmic slow waves are associated with a low risk of early onset seizures whereas focal spikes, sharp waves, periodic lateralized, or periodic bilateral discharges are associated with a higher risk. There are no randomised controlled trials performed in stroke patients evaluating management strategies for poststroke seizures and epilepsy. Treatment options should be extrapolated from those of epilepsy in general, taking into consideration hepatic and renal function, tolerability, costs, interactions between antiepileptic drugs (AED), interactions with antithrombotic and other treatments. However, randomised controlled trials of AED prophylaxis for acute and remote seizures are essential to improve the evidence level of current guidelines and recommendations for the management of post-stroke seizures

Treatment for the Benign Childhood Epilepsy With Centrotemporal Spikes: A Monocentric Study

Background and Purpose: To date, there is no specific treatment guideline for the benign childhood epilepsy with centrotemporal spikes (BECTS). Several countries recommend levetiracetam, carbamazepine, sodium valproate, oxcarbazepine, and lamotrigine as first-line drugs. Nevertheless, some of these drugs are associated with cognitive decline. Available studies that investigated the efficacy of levetiracetam and sodium valproate on BECTS involved small sample sizes. This study aimed to evaluate the efficacy of levetiracetam and sodium valproate on cognition, and to investigate the prognostic factors for BECTS as whole.Methods: Clinical data and treatment status of all patients with BECTS at Xiangya Hospital, Central South University followed from 2008 to 2013 were analyzed retrospectively. Since electrical status epilepticus in sleep (ESES) has been confirmed to play a role in cognitive deterioration, in order to evaluate the response to drugs and their cognitive effects, we created two groups of patients according to the levels of spike wave index (SWI): group 1; 0–50% SWI and group 2; >50% SWI at the last follow up.Results: A total of 195 cases were enrolled: 49.7% received monotherapies, 24.1% duotherapies and 27.2% polytherapies. Medications included; levetiracetam plus other drug (s) (75.9%), levetiracetam alone (32.8%), sodium valproate plus other drug (s) (31.3%), and sodium valproate alone (5.1%). After 2 years of treatment and follow up, 71% of the cases had a good seizure outcome, 15.9% had an improvement of SWI, and 91.7% had a normal DQ/IQ. Sodium valproate combined with levetiracetam, and sodium valproate alone correlated with good improvement of SWI, whereas, focal spikes were linked with poor improvement. For both groups (group 1 and group 2): monotherapy, levetiracetam alone, and a normal DQ/IQ at seizure onset correlated with good cognitive outcomes, in contrast, polytherapy, sodium valproate plus other drug (s), levetiracetam plus sodium valproate, an initial SWI of ≥85%, and multifocal spikes were linked to cognitive deterioration.Conclusions: Monotherapy, particularly levetiracetam seems to be a good first-line therapy which can help in normalizing the electroencephalograph and preventing cognitive decline. Polytherapy, mostly the administration of sodium valproate seems to relate with poor cognition, therefore, it is recommended to avoid it.