Serial EEG Research Articles

Preliminary Study on Rapid Serial Visualization Presentation Multi-Class Target EEG Classification.

Brain-Computer Interface (BCI) provides a direct communication channel between the brain and external devices. After combining with the Rapid Serial Visualization Presentation (RSVP) paradigm, the RSVP-BCI system can be utilized for human vision-based fast information retrieval. Currently only binary classification of single-trial EEG can be achieved, also the research on the multi-class target RSVP is few, which limited information transfer rate and the application scenarios of the system. In this paper, we focus on the RSVP multi-class target image retrieval task that contains two classes of targets for achieving triple classification for RSVP-EEG. Designed two experiments, each containing two tasks with different task difficulties. We recruited 30 subjects to participate in the experiments, collected EEG data, and made the data publicly available. Moreover, we conducted behavioral analysis, ERP analysis, and proposed a model, MDCNet, for EEG classification to study the feasibility of multi-class target RSVP and the impact of task difficulty. The experimental results indicated that (1) RSVP-EEG classification that includes non-target and 2-class target is feasibility; (2) the different targets in the same task will evoke P300 with the same latency and different amplitudes, and the hit rate of the target in EEG classification is positively correlated with its amplitude; (3) the information hidden in the time dimension play an important role in EEG classification; (4) the harder the task is, the latency of P300 is longer. The experimental analysis obtained meaningful results, which provided a theoretical basis for subsequent research.

298 An atypical presentation of Creutzfeldt-Jakob Disease as a stroke mimic—experience from an Irish tertiary centre

Abstract Background Sporadic Creutzfeldt-Jakob Disease (sCJD) is a rare neurodegenerative prion disease that presents with symptoms of rapid neuropsychiatric decline including dementia, behavioural abnormalities and loss of higher cortical function. Patients commonly present with rapidly-progressive neuromotor symptoms such as ataxia and myoclonus. Very few cases of CJD have been reported in which the patient initially presents with stroke symptoms such as hemiparesis as their primary presenting symptom. We present a case of a 56 year old male who initially presented to the stroke unit with waxing and waning left sided weakness and a non-corresponding ipsilateral left-sided acute parietal infarct on diffusion-weighted MRI. Over four weeks, his condition progressively worsened with declining cognitive function, motor dysfunction, sphincter dysfunction and eventual death. Methods Methods used to confirm the suspected diagnosis included: (1) Periodic sharp wave complexes are the most classical EEG finding for CJD and are seen in two third of the patients diagnosed with sporadic CJD, (2) The presence of the 14–3-3 protein in CSF has been found to be a sensitive and specific marker for sporadic CJD, (3) Neuroimaging plays a vital role in the diagnosis of CJD including serial MRIs and EEG sufficient to exclude other differential diagnoses. Results MRI brain revealed an acute cortical infarct within left parietal lobe measuring 3 mm.A n EEG demonstrated periodic sharp wave complexes occurring maximally in the left centro-temporal region. EEG finding was suggestive of a diagnosis of a prion disease. CJD Surveillance Unit confirmed the cerebrospinal fluid analysis was positive for RT-QuIC indicating sporadic CJD. Conclusion In cases of patients presenting with clinical signs and symptoms of a stroke with subsequent ipsilateral neurological findings on MRI, CJD should be included as a differential diagnosis. Additionally, a lumbar puncture, not routinely recommended in stroke patients, should be performed early to aid in early detection of CJD in order to confirm diagnosis and optimise the ceiling of care.

N°10 – Elucidating the fate of ‘doppelgänger’ CSWS patterns in childhood epilepsies by serial EEG and neurocognitive analysis

N°10 – Elucidating the fate of ‘doppelgänger’ CSWS patterns in childhood epilepsies by serial EEG and neurocognitive analysis

Examining the neural mechanisms of rTMS: a naturalistic pilot study of acute and serial effects in pharmacoresistant depression.

Previous studies have demonstrated the effectiveness of therapeutic repetitive transcranial magnetic stimulation (rTMS) to treat pharmacoresistant depression. Nevertheless, these trials have primarily focused on the therapeutic and neurophysiological effects of rTMS following a long-term treatment course. Identifying brain-based biomarkers of early rTMS therapeutic response remains an important unanswered question. In this pilot study, we examined the effects of rTMS on individuals with pharmacoresistant depression using a graph-based method, called Functional Cortical Networks (FCN), and serial electroencephalography (EEG). We hypothesized that changes in brain activity would occur early in treatment course. A total of 15 patients with pharmacoresistant depression underwent five rTMS sessions (5Hz over the left dorsolateral prefrontal cortex, 120%MT, up to 4,000 pulses/session). Five participants received additional rTMS treatment, up to 40 sessions. Resting EEG activity was measured at baseline and following every five sessions, using 64-channel EEG, for 10 minutes with eyes closed. An FCN model was constructed using time-varying graphs and motif synchronization. The primary outcome was acute changes in weighted-node degree. Secondary outcomes included serial FFT-based power spectral analysis and changes in depressive symptoms measured by the 9-Item Patient Health Questionnaire (PHQ-9) and the 30-item Inventory of Depressive Symptoms-Self Report (IDS-SR). We found a significant acute effect over the left posterior area after five sessions, as evidenced by an increase in weighted-node degree of 37,824.59 (95% CI, 468.20 to 75,180.98) and a marginal enhancement in the left frontal region (t (14) = 2.0820, p = 0.056). One-way repeated measures ANOVA indicated a significant decrease in absolute beta power over the left prefrontal cortex (F (7, 28) = 2.37, p = 0.048) following ten rTMS sessions. Furthermore, a significant clinical improvement was observed following five rTMS sessions on both PHQ-9 (t (14) = 2.7093, p = 0.017) and IDS-SR (t (14) = 2.5278, p = 0.024) and progressed along the treatment course. Our findings suggest that FCN models and serial EEG may contribute to a deeper understanding of mechanisms underlying rTMS treatment. Additional research is required to investigate the acute and serial effects of rTMS in pharmacoresistant depression and assess whether early EEG changes could serve as predictors of therapeutic rTMS response.

Characterizing EEG and Serum Biomarker Trends in Immune Effector Cell-Associated Neurotoxicity: A Case Series (P10-1.001)

<h3>Objective:</h3> To characterize electroencephalographic (EEG) trends over time in immune effector cell-associated neurotoxicity syndrome (ICANS) and identify correlations between neuron-derived serum biomarkers of inflammation, neurotoxicity grade, and EEG findings. <h3>Background:</h3> Chimeric antigen receptor T-cell therapy (CAR-T) can be effective for refractory hematologic malignancies, but may be complicated by ICANS. Previous studies link higher peak neurotoxicity grades with EEG background abnormalities and higher incidences of rhythmic and periodic patterns (RPPs). <h3>Design/Methods:</h3> We performed a retrospective analysis of CAR-T patients who were enrolled into an IRB-approved prospective, longitudinal observational study. Data including demographics, ICE scores, and neurologic symptoms were prospectively entered. Serum biomarkers including Ang-1, Ang-2, IL-6, UCH-L1, and S100b were drawn prior to CAR-T and at defined time points thereafter. EEG data was reviewed, with encephalopathy grade defined by American Clinical Neurophysiology Society (ACNS) criteria. <h3>Results:</h3> Forty-five patients were included in the study. Ten patients (22%) developed ICANS. EEG data was available for five (11%) patients, with a median of 35 hours recorded per patient. Zero patients had confirmed electrographic seizures. Median ICE score prior to EEG was 1. Backgrounds were mildly or moderately encephalopathic, with improving organization and faster frequencies over time. GPDs or LPDs of 0.5–2Hz were rare to abundant and gradually improved with time. Focal slowing and sharp waves were common. S100b consistently increased above (average 45%) and Ang-2 decreased below (average 53%) baseline at the time of becoming symptomatic for two patients (drawn within 48 hours before/after EEG was obtained), whereas Ang-1, IL-6, and UCH-L1 had more variable changes. <h3>Conclusions:</h3> Patients with ICANS who underwent EEGs had up to moderate electrographic (and severe clinical) encephalopathy and RPPs that spontaneously improved with time. Higher encephalopathy grade occurred in those with greater S100b increases. Our observations will need to be confirmed in an independent population with serial samples and EEG data on all patients. <b>Disclosure:</b> Dr. Nguyen has nothing to disclose. Lila Renteria has nothing to disclose. Dr. Motika has nothing to disclose. Dr. Hinson has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Biogen. Dr. Hinson has received personal compensation in the range of $10,000-$49,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for UCSF. Dr. Hinson has received personal compensation in the range of $10,000-$49,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for American Heart Association.

570. Effects of Repetitive Transcranial Magnetic Stimulation on Functional Cortical Networks and Serial EEG in Pharmacoresistant Depression

570. Effects of Repetitive Transcranial Magnetic Stimulation on Functional Cortical Networks and Serial EEG in Pharmacoresistant Depression

The state of pediatric tuberous sclerosis complex epilepsy care: Results from a national survey.

Epilepsy associated with tuberous sclerosis complex (TSC) can be challenging to treat and is associated with significant disease burden. Our objective was to better understand the state of epilepsy care of TSC amongst pediatric neurologists in Canada, identify gaps in care and determine whether access to a dedicated TSC clinic has an impact on epilepsy management. A survey was developed after a literature review and discussion amongst two pediatric epileptologists and one nurse practitioner with expertise in TSC about the state of epilepsy care of TSC patients in Canada. Canadian pediatric neurologists were asked to participate in sharing their experiences via an anonymous web-based survey through the Canadian League Against Epilepsy (CLAE) and the Canadian Neurological Sciences Federation (CNSF). Fifty-seven responses were received. Access to a dedicated TSC clinic was reported by 25% (n=14). Sixty percent (n=34) reported performing serial EEG monitoring in infants with TSC and 57% (n=33) started prophylactic antiseizure therapy when EEG abnormalities were detected, regardless of whether there was access to a TSC clinic (P=.06 and P=.29, respectively). While 52% (n=29) did not feel comfortable prescribing mTORi for epilepsy, 65% (n=36) indicated they would consider it with additional training. Epilepsy surgery was offered in 93% (n=13) of centers with a dedicated TSC clinic but only 45% of centers without a TSC clinic (n=19) (P=.002). Our findings demonstrate the variability in neurological care of pediatric patients with TSC as it pertains to epilepsy management. There is a need for the establishment of epilepsy practice guidelines and a national network to support clinical practice, research, and education.

The Role of Serial Follow-up and Sleep Deprivation Methods in Improving Electroencephalography Diagnostic Yield in a Cohort of Omanis Aged 13 Years and Above: A Clinical Audit Study

ABSTRACT The aim of this audit study was to establish the utility of follow-up and sleep-deprived electroencephalography testing to improve the detection of interictal abnormalities in a tertiary referral center in Oman. As part of our ongoing auditing process, a total of 3010 EEGs were included in this study. All EEGs were routinely performed for Omanis aged 13 years and above, who were referred for possible diagnosis of seizure disorders. Each EEG was performed over an average period of 20–30 minutes. Of the 3010 EEGs, there were 553 follow-up and sleep-deprived EEGs, including initial baseline EEG studies which were analyzed for this study. The total progressive yield of serial follow-up EEGs to detect overall EEG changes was 53.5%, distributed as 8.8%, 11.4%, 0%, and 33.3% for the second, third, fourth, and fifth serial EEG studies, respectively. For the sleep deprivation EEG group, the yield was 6.5% for detecting overall EEG changes compared to the initial EEG studies. A limitation in this study was the small sample size in the subsequent follow-up and sleep deprivation EEGs. In conclusion, we found a minimal contribution of serial follow-up and sleep deprivation methods in improving the EEG abnormality detection in our study. National guidelines and an increase in awareness among physicians are required to increase the benefit of these well-established, yet not optimally utilized EEG methods.

P.087 The landscape of paediatric tuberous sclerosis complex (TSC) neurological care in canada: results from a national survey

Background: Tuberous Sclerosis Complex (TSC) is a genetic disease that affects multiple body systems with the neurological manifestations causing the greatest disease burden. The objective of this study was to understand the scope of neurological TSC care delivery across Canada. Methods: A survey was developed after literature review and discussion amongst two Paediatric Epileptologists and one Nurse Practitioner with expertise in TSC. Canadian Paediatric Neurologists participated via an anonymous web-based survey through the Canadian League Against Epilepsy (CLAE) and Canadian Neurological Sciences Federation (CNSF). Results: Fifty-eight responses were received. A dedicated TSC clinic was reported by 24% (n=14). Sixty percent (n= 35) reported preforming serial screening EEG monitoring in infants and 58% (n= 34) started prophylactic therapy when EEG abnormalities occurred. Vigabatrin was used in 37% (n=21). For management of drug-resistant epilepsy, surgery was reported as the preferred therapeutic option in 57% (n=32) of respondents. Barriers to treatment identified were a lack of multi-disciplinary care, unfamiliarity with new therapies and insufficient resources. Conclusions: Our findings demonstrate the variability in neurological care delivery of patients with TSC. With few dedicated TSC clinics, there is a need for the establishment of a national network to support clinical practice, research and education.

Clinical and prognostic features of Heidenhain variant of Creutzfeldt−Jakob disease: A retrospective case series study

Heidenhain variant of Creutzfeldt-Jakob disease (CJD) remains a diagnostic challenge in clinical practice. We aimed to describe the clinical and prognostic features of Heidenhain cases, through a case series study. We retrospectively reviewed the definite or probable CJD cases admitted to two tertiary referral university hospitals over a decade to identify Heidenhain cases and investigated their survival status by telephone follow-up. Their clinical characteristics, neuroimaging features, electroencephalography (EEG) results, cerebrospinal fluid profiles, and PRNP gene mutations were also analyzed. Of a total of 85 CJD cases, 20 (24%) Heidenhain cases (11 women [55%]; median age, 64years [range, 44-72years]) were identified. The median survival time was 22weeks (range, 5-155weeks). The median duration of isolated visual symptoms was 3weeks (range, 1-12weeks). The most common early visual symptom was blurred vision (16/20, 80%), followed by diplopia (6/20, 30%). The prevalence significantly increased for complex visual hallucination (p=0.005) and cortical blindness (p=0.046) as the disease progressed. The positive rate of serial magnetic resonance images (20/20, 100%) was higher than that of serial EEGs (16/20, 80%). Two patients (2/10, 20%) had pathogenic PRNP mutations, E196A and T188K, respectively. Heidenhain cases with PRNP mutations had significantly longer survival time than those without PRNP mutations (p=0.047). Besides blurred vision (80%), diplopia (30%) was also a frequent early visual symptom among Heidenhain cases. Heidenhain phenotype can occur in genetic CJD cases. PRNP mutation status might be an important prognostic factor for Heidenhain cases.

Association of Early MRI Characteristics With Subsequent Epilepsy and Neurodevelopmental Outcomes in Children With Tuberous Sclerosis Complex.

Multiple factors have been found to contribute to the high risk of epilepsy in infants with tuberous sclerosis complex (TSC), including evolution of EEG abnormalities, TSC gene variant, and MRI characteristics. The aim of this prospective multicenter study was to identify early MRI biomarkers of epilepsy in infants with TSC aged <6 months and before seizure onset, and associate these MRI biomarkers with neurodevelopmental outcomes at 2 years of age. The study was part of the EPISTOP project. We evaluated brain MRIs performed in infants younger than 6 months with TSC. We used harmonized MRI protocols across centers and children were monitored closely with neuropsychological evaluation and serial video EEG. MRI characteristics, defined as tubers, radial migration lines, white matter abnormalities, cysts, calcifications, subependymal nodules (SEN), and subependymal giant cell astrocytoma (SEGA), were visually evaluated and lesions were detected semiautomatically. Lesion to brain volume ratios were calculated and associated with epilepsy and neurodevelopmental outcomes at 2 years. Lesions were assessed on MRIs from 77 infants with TSC; 62 MRIs were sufficient for volume analysis. The presence of tubers and higher tuber-brain ratios were associated with the development of clinical seizures, independently of TSC gene variation and preventive treatment. Furthermore, higher tuber-brain ratios were associated with lower cognitive and motor development quotients at 2 years, independently of TSC gene variation and presence of epilepsy. In infants with TSC, there is a significant association between characteristic TSC lesions detected on early brain MRI and development of clinical seizures, as well as neurodevelopmental outcomes in the first 2 years of life. According to our results, early brain MRI findings may guide clinical care for young children with TSC. This study provides Class I evidence that in infants with TSC, there is a significant association between characteristic TSC lesions on early brain MRI and the development of clinical seizures and neurodevelopmental outcomes in the first 2 years of life.

P-EP022. Clinical diagnosis, EEG evaluation and treatment for NMDAR autoimmune encephalitis

Introduction. NMDAR autoimmune encephalitis is a very rare disease. Autoimmune encephalitis (AE) is an increasingly recognized etiology of acute neuropsychiatric decline in adults and children. The clinical manifestations of AE are broad and include seizures, movement disorders, behavior and mood changes, psychosis, memory/ cognitive impairment, autonomic dysfunction, and altered level of consciousness. We described one case of NMDAR autoimmune encephalitis. Diagnosis was made base on clinical sign, serial electroencephalogram (EEG) patterns and NMDAR CSF evaluation. The clinical course of the disease, specific treatment and outcome were follow up until 3 months. Results. An 11-year-old female presented with focal seizure, psychiatric features, behavioral changes followed by autonomic dysfunction, orofacialbrachial dyskinesias, speech reduction and gradual altered consciousness. Serial EEG examination between August until November 2020 showed progressive changes. Initial EEG was normal (August 2020). Second EEG showed continuous slow activity 5–6 Hz generally and excessive beta activity (September 21, 2020). Last EEG (September 26, 2020) before treatment showed excessive beta activity range 14–20 Hz, Extreme Delta Brush (EDB) and Generalized Rhythmic Delta Activity (GRDA) 2–3 Hz. Based on clinical manifestation and EEG examination we start immunotherapy treatment with IVIG and Methylprednisolone IV to avoid fatal complication of the disease. Evaluation of anti-NMDAR from cerebrospinal fluid tapping was done simultaneously. Patient showed significant clinical improvement and Modified Ranking Scale improved after immunotherapy. Conclusion. Early identification of NMDAR autoimmune encephalitis manifestation is very important. Combination of symptom consist of psychiatric manifestations (behavioral changes), clinical symptom (autonomic dysfunction, orofacialbrachial dyskinesias, speech reduction and altered consciousness), should be followed by serial EEG evaluation. Immediate immunotherapy treatment can be started based on clinical diagnosis to avoid fatal complication.

Prognostic value of electroencephalography in hypoxic-ischemic encephalopathy

Background: Hypoxic-ischemic encephalopathy (HIE) is one of the main causes of neurodevelopmental disorders. We developed a model that has diagnostic and prognostic value in predicting the neurodevelopmental outcomes in newborns with HIE. HIE staging allows us to start therapeutic interventions early in newborns with suspected encephalopathy.&#x0D; Methods: This was a retrospective study in a cohort of 58 full-term neonates with clinical suspicion of HIE. We assessed electroclinical variables at birth [etiology of hypoxia, neonatal seizures, HIE stages based on Sarnat criteria, use of therapeutic hypothermia, neuroimaging tests and electroencephalography (EEG) findings] and two years of follow up (EEG findings, development of epilepsy, the presence of cognitive deficits, behavioral issues, language problems, visual or hearing disturbances, and cerebral palsy).&#x0D; Results: There was a high electro-clinical correlation to severe HIE (88.8%) and moderate HIE (50%). There was a considerable proportion of patients affected by mild HIE, based on clinical examination, who presented with an abnormal EEG (32.3%). There is a relationship between the onset of neonatal seizures, epilepsy, and severe HIE diagnosed with EEG (88.9%). A higher percentage of patients with moderate and severe HIE, based on EEG findings, present abnormal results in cranial ultrasound and cerebral magnetic resonance imaging (62.5%). At two years of age, functional neurodevelopment disturbances were observed most frequently in patients affected with severe and moderate HIE based on EEG.&#x0D; Conclusions: This study shows a model with diagnostic and prognostic value in predicting newborns' neurodevelopmental outcomes with suspected HIE. This knowledge allows us to assess the role of performing serial EEG in patients with suspected HIE and the relevance of EEG findings in the prognosis of neurodevelopmental disorders.

Early epileptiform EEG activity in infants with tuberous sclerosis complex predicts epilepsy and neurodevelopmental outcomes

To study the association between timing and characteristics of the first electroencephalography (EEG) with epileptiform discharges (ED-EEG) and epilepsy and neurodevelopment at 24months in infants with tuberous sclerosis complex (TSC). Patients enrolled in the prospective Epileptogenesis in a genetic model of epilepsy - Tuberous sclerosis complex (EPISTOP) trial, had serial EEG monitoring until the age of 24months. The timing and characteristics of the first ED-EEG were studied in relation to clinical outcome. Epilepsy-related outcomes were analyzed separately in a conventionally followed group (initiation of vigabatrin after seizure onset) and a preventive group (initiation of vigabatrin before seizures, but after appearance of interictal epileptiform discharges [IEDs]). Eighty-three infants with TSC were enrolled at a median age of 28days (interquartile range [IQR] 14-54). Seventy-nine of 83 patients (95%) developed epileptiform discharges at a median age of 77days (IQR 23-111). Patients with a pathogenic TSC2 variant were significantly younger (P-value .009) at first ED-EEG and more frequently had multifocal IED (P-value .042) than patients with a pathogenic TSC1 variant. A younger age at first ED-EEG was significantly associated with lower cognitive (P-value .010), language (P-value .001), and motor (P-value .013) developmental quotients at 24months. In the conventional group, 48 of 60 developed seizures. In this group, the presence of focal slowing on the first ED-EEG was predictive of earlier seizure onset (P-value .030). Earlier recording of epileptiform discharges (P-value .019), especially when multifocal (P-value .026) was associated with higher risk of drug-resistant epilepsy. In the preventive group, timing, distribution of IED, or focal slowing, was not associated with the epilepsy outcomes. However, when multifocal IEDs were present on the first ED-EEG, preventive treatment delayed the onset of seizures significantly (P-value <.001). Early EEG findings help to identify TSC infants at risk of severe epilepsy and neurodevelopmental delay and those who may benefit from preventive treatment with vigabatrin.

Remote and Long-Term Self-Monitoring of Electroencephalographic and Noninvasive Measurable Variables at Home in Patients With Epilepsy (EEG@HOME): Protocol for an Observational Study.

BackgroundEpileptic seizures are spontaneous events that severely affect the lives of patients due to their recurrence and unpredictability. The integration of new wearable and mobile technologies to collect electroencephalographic (EEG) and extracerebral signals in a portable system might be the solution to prospectively identify times of seizure occurrence or propensity. The performances of several seizure detection devices have been assessed by validated studies, and patient perspectives on wearables have been explored to better match their needs. Despite this, there is a major gap in the literature on long-term, real-life acceptability and performance of mobile technology essential to managing chronic disorders such as epilepsy.ObjectiveEEG@HOME is an observational, nonrandomized, noninterventional study that aims to develop a new feasible procedure that allows people with epilepsy to independently, continuously, and safely acquire noninvasive variables at home. The data collected will be analyzed to develop a general model to predict periods of increased seizure risk.MethodsA total of 12 adults with a diagnosis of pharmaco-resistant epilepsy and at least 20 seizures per year will be recruited at King’s College Hospital, London. Participants will be asked to self-apply an easy and portable EEG recording system (ANT Neuro) to record scalp EEG at home twice daily. From each serial EEG recording, brain network ictogenicity (BNI), a new biomarker of the propensity of the brain to develop seizures, will be extracted. A noninvasive wrist-worn device (Fitbit Charge 3; Fitbit Inc) will be used to collect non-EEG biosignals (heart rate, sleep quality index, and steps), and a smartphone app (Seer app; Seer Medical) will be used to collect data related to seizure occurrence, medication taken, sleep quality, stress, and mood. All data will be collected continuously for 6 months. Standardized questionnaires (the Post-Study System Usability Questionnaire and System Usability Scale) will be completed to assess the acceptability and feasibility of the procedure. BNI, continuous wrist-worn sensor biosignals, and electronic survey data will be correlated with seizure occurrence as reported in the diary to investigate their potential values as biomarkers of seizure risk.ResultsThe EEG@HOME project received funding from Epilepsy Research UK in 2018 and was approved by the Bromley Research Ethics Committee in March 2020. The first participants were enrolled in October 2020, and we expect to publish the first results by the end of 2022.ConclusionsWith the EEG@HOME study, we aim to take advantage of new advances in remote monitoring technology, including self-applied EEG, to investigate the feasibility of long-term disease self-monitoring. Further, we hope our study will bring new insights into noninvasively collected personalized risk factors of seizure occurrence and seizure propensity that may help to mitigate one of the most difficult aspects of refractory epilepsy: the unpredictability of seizure occurrence.International Registered Report Identifier (IRRID)PRR1-10.2196/25309

Spike wave characteristics and temporal spike evolution on serial EEG in childhood epilepsy with centrotemporal spikes

PurposeTo assess the spike characteristics and temporal spike evolution on serial EEG of children with childhood epilepsy with centrotemporal spikes (CECTS) treated with anti-seizure medication. MethodsThe study cohort consisted of 127 children with CECTS divided into three groups based on anti-seizure medication responsiveness: group I: seizure-free with monotherapy (n: 61, 48%), group II: seizure-controlled with monotherapy (n: 52, 41%) and group III: seizure-controlled with dual therapy (n: 14, 11%). The clinical profiles and sequential four-year follow-up visual EEG recordings of the children were evaluated. Each EEG was reanalyzed with three spike characteristics on the epochs: (1) spike-wave rate, (2) spike topography, and (3) spike localization. We calculated the spike clearance velocity, which is defined as a decrease in the spike-wave rate over time in four-year sequential follow-up EEGs. ResultsThere was no statistical significance across the study groups with respect to initial EEG spike characteristics (spike-wave rate, spike localization, and spike topography). Seizure recurrence occurred in 15 patients (12.8%) who discontinued anti-seizure medication. There was no statistically significant difference between the spike characteristics on EEG just before the discontinuation of anti-seizure medication and seizure recurrence. However, the spike clearance velocity was significantly slower in group III than in group I in four-year sequential follow-up EEGs (p = 0.002). A statistically significant decrease in the mean spike-wave rate was observed in group I in the first year of anti-seizure medication (p<0.001). The spike clearance velocity was also more prominent during the second year of treatment in group II and the third year of treatment in group III. However, the spike clearance velocity was not different across the anti-seizure medication groups (oxcarbazepine, valproic acid, and levetiracetam). ConclusionSpike clearance velocity might be a valuable EEG marker to guide anti-seizure medication in children with CECTS.

Two Concomitant Cases with Febrile Infection-Related Epilepsy Syndrome (FIRES) treated with andldquo;3-Dimensional Combination Treatment Protocolandrdquo;

Objective: Febrile Infection-Related Epilepsy Syndrome (FIRES) is an immun-mediated epileptic encephalopathy presents as a febrile infection related super refractory status epilepticus with mortality rate of up to 30% in intensive care unit in spite of pharmacologically induced coma and immunotherapies. Early interventional ketogenic diet and cannabinoids are suggested for treatment with unknown mechanisms. Case presentation: Here we report two concomitant FIRES cases treated with a “3 dimensional combined treatment protocol“ for FIRES including (1); super refractory status epilepticus treatment protocol with intravenous infusion of antiepileptic and anesthetic drugs, (2) immunomodulatory therapies and (3) early interventional anti-epileptogenic therapy (ketogenic diet plus cannabinoids). The therapeutic intensity of the treatment protocol was monitored with serial EEG monitoring and Functional Independence Scores-WeeFIM. Two previously healthy school children manifesting super refractory status epilepticus were treated with moderate success. Both patients recovered with drug refractory epilepsy and neurocognitive impairments (patient 1; WeeFIM Scores were: self-care: 17, motor: 24, cognition: 10 and patient 2; WeeFIM score remained totally dependent: self-care: 8, motor: 5, cognition: 5) at the 12 months of follow-up. Conclusion: Febrile infection-related epilepsy syndrome is a devasting catastrophic epileptic encephalopathy. Early interventional treatment protocols should be applied in the early period of super refractory status epilepticus.

Quantitative burst suppression on serial intermittent EEG in refractory status epilepticus

ObjectivesIn refractory status epilepticus (RSE), the optimal degree of suppression (EEG burst suppression or merely suppressing seizures) remains unknown. Many centers lacking continuous EEG must default to serial intermittent recordings where uncertainty from lack of data may prompt more aggressive suppression. In this study, we sought to determine whether the quantitative burst suppression ratio (QBSR) from serial intermittent EEG recording is associated with RSE patient outcome. MethodsWe screened the EEG database to identify non-anoxic adult RSE patients for EEG and chart review. QBSR was calculated per 10-second EEG epoch as the percentage of time during which EEG amplitude was <3 µV. Patients who survived 1–3 months after discharge from ICU and hospital comprised the favorable group. Further to initial unadjusted univariate analysis of all pooled QBSR, we conducted multivariate analyses to account for individual patient confounders (“per-capita analysis”), uneven number of EEG recordings (“per-session analysis”), and uneven number of epochs (“per-epoch analysis”). We analyzed gender, anesthetic number, and adjusted status epilepticus severity score (aSTESS) as confounders. ResultsIn 135,765 QBSR values over 160 EEG recordings (median 2.17 h every ≥24 h) from 17 patients on Propofol, Midazolam, and/or Ketamine, QBSR was deeper in the favorable group (p < 0.001) on initial unadjusted analysis. However, on adjusted multivariate analysis, there was consistently no association between QBSR and outcome. Higher aSTESS consistently associated with unfavorable outcome on per-capita (p = 0.033), per-session (p = 0.048) and per-epoch (p < 0.001) analyses. Greater maximal number of non-barbiturate anesthetic associated with favorable outcome on per-epoch analysis (p < 0.001). ConclusionsThere was no association between depth of EEG suppression using non-barbiturate anesthetic and RSE patient outcome based on QBSR from serial intermittent EEG. A per-epoch association between non-barbiturate anesthetic and favorable outcome suggests an effect from non-suppressive time-varying EEG content. SignificanceTargeting and following deeper burst suppression through non-barbiturate anesthetics on serial intermittent EEG monitoring of RSE is of limited utility.

When should we obtain a routine EEG while managing people with epilepsy?

More than eight decades after its discovery, routine electroencephalogram (EEG) remains a safe, noninvasive, inexpensive, bedside test of neurological function. Knowing when a routine EEG should be obtained while managing people with epilepsy is a critical aspect of optimal care. Despite advances in neuroimaging techniques that aid diagnosis of structural lesions in the central nervous system, EEG continues to provide critical diagnostic evidence with implications on treatment. A routine EEG performed after a first unprovoked seizure can support a clinical diagnosis of epilepsy and differentiate those without epilepsy, classify an epilepsy syndrome to impart prognosis, and characterize seizures for antiseizure management. Despite a current viral pandemic, EEG services continue, and the value of routine EEG is unchanged.

Creutzfeldt-Jacob Disease, As a Rare Cause of Dementia Should Not be Forgotten: Single-Center Experience

Introduction: Dementia, which is an important public health problem, is divided into two groups as primary and secondary dementia. Creutzfeldt–Jakob disease (CJD), which is rarely seen, is a secondary cause of dementia with a rapidly progressive course. It is a fatal neurodegenerative disorder of infectious spongiform encephalopathy that can affect humans and animals. Sporadic CJD is the most common type that can present in four forms and has typical clinical presentations of progressive cognitive impairment, myoclonus, and ataxia. We aimed to discuss the demographic, clinical, and laboratory findings of CJD cases diagnosed in the neurointensive care unit. Methods: Sixteen patients were included in this retrospective study. Age, sex, complaints on admission, duration from symptom onset to mortality, neurologic examination, brain magnetic resonance imaging (MRI), electroencephalography (EEG), and the protein 14.3.3 status of the cerebrospinal fluid (CSF) were recorded. Results: The mean age was 61.18 ± 9.1 years (range, 37–73 years), and the most common complaints on admission were cognitive impairment, disturbance in consciousness, ataxia, and gait disturbance. CSF protein 14-3-3 was positive in 100% of patients. The most common findings were periodic sharp and wave complexes on EEG, cortical or putamen and caudate nucleus hyperintensity, cortical and cerebellar atrophy on MRI. One of the patients was diagnosed as variant CJD, and the others were diagnosed as the sporadic form. Conclusions: CJD should be kept in mind in patients with myoclonus or ataxia accompanied by progressive cognitive impairment. Neuroimaging, serial EEG recordings, CSF analysis, and histopathologic examination should be performed for diagnosis.