Luca Bosisio, Maria Margherita Mancardi, Silvia Boeri, Lino Nobili, Giulia Nobile, Laura Siri, Giulia Prato, Edoardo Canale
{"title":"Subclinical rhythmic EEG discharge of adults (SREDA) in pediatric population: A case series with systematic review of the literature","authors":"Luca Bosisio, Maria Margherita Mancardi, Silvia Boeri, Lino Nobili, Giulia Nobile, Laura Siri, Giulia Prato, Edoardo Canale","doi":"10.1002/epd2.20294","DOIUrl":"10.1002/epd2.20294","url":null,"abstract":"<p>Subclinical rhythmic electrographic discharge of adults (SREDA) is one of the rarest and most challenging non-epileptic electroencephalographic variants. Although the pathogenesis of this activity is unclear, an association with vascular insufficiency and cerebral hypoxia has been proposed. SREDA usually occurs in adulthood, but there are few reports in the pediatric population. We performed a systematic review of the literature, confirming the rarity of this condition in children, and added 5 more subjects. We report on a total of 16 children with SREDA. Sufficient data are available for 15 patients. The mean age at first detection of SREDA was 11.5 years. We observed that 67% (10/15) of the subjects had previous seizures: 80% (8/10) of them had an epilepsy diagnosis and 38% (3/8) had generalized epilepsy. Moreover, 8 of 13 subjects whose medical history was available (61%) had a neurodevelopmental disorder. From an electroencephalographic point of view, we noted a prevalence of bilateral SREDA with atypical localization and abrupt onset and end. Since SREDA can be incorrectly interpreted as an epileptic discharge, with possible therapeutic implications, it is important to consider its possible occurrence also in pediatric patients, perhaps more frequently in those with neurodevelopmental disorders.</p>","PeriodicalId":50508,"journal":{"name":"Epileptic Disorders","volume":"27 1","pages":"71-81"},"PeriodicalIF":1.9,"publicationDate":"2024-10-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/epd2.20294","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142479727","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Audrey Nguyen, Ralf Eberhard, Elisabeth Simard-Tremblay, Kenneth A. Myers
{"title":"Ictal asystole and increased ictal heart rate variability in PCDH19-related epilepsy","authors":"Audrey Nguyen, Ralf Eberhard, Elisabeth Simard-Tremblay, Kenneth A. Myers","doi":"10.1002/epd2.20281","DOIUrl":"10.1002/epd2.20281","url":null,"abstract":"<p>\u0000 <i>PCDH19</i> (OMIM 300460) pathogenic variants are associated with a range of neurodevelopmental abnormalities and epilepsy.<span><sup>1, 2</sup></span> This X-linked condition has been referred to as “girls clustering epilepsy,” with the classical phenotype involving infantile onset of recurrent seizure clusters, sometimes lasting several days, in the context of febrile illnesses.<span><sup>3-5</sup></span> Seizure semiology is focal in the vast majority, with motor onset described in 85%. Non-motor onset seizures are reported in 59%, with features including behavioral arrest, loss of tone, hypopnea, cyanosis, and desaturation. We describe a girl with PCDH19-related epilepsy who had ictal bradycardia and asystole and report the results of an analysis of peri-ictal heart rate variability (HRV).</p><p>At 9 months of age, the proband presented with recurrent focal seizures in the context of a febrile viral illness. While on continuous video EEG monitoring, 27 seizures were recorded over a 49-hour period, 3 focal and 24 focal-to-bilateral. The clinical manifestations usually began after the electrographic onset, and typically involved bilateral arm stiffening and unresponsiveness (Video 1). Based on scalp EEG, 12 seizures had left occipital-temporal onset and 10 right parietal-occipital onset; the region of onset was unclear in three cases. Bradycardia occurred with almost all seizures and ictal asystole (defined as ≥3-second pause between heartbeats) in three instances, lasting as long as 11 s. Seizure duration ranged from 59 to 251 s.</p><p>Her past medical history was significant for being a triplet pregnancy and born extremely premature, at 23 weeks, 6 days gestation. She had sequelae of prematurity, including bronchopulmonary dysplasia, retinopathy of prematurity, and metabolic bone disease. An echocardiogram done early in life showed an atrial septal defect (ASD) and ventricular septal defect (VSD). On family history, her mother had autism and was reported to have “generalized epilepsy,” which had started with febrile seizures at age 10 months; she was still on antiseizure medication, lacosamide and clobazam. The patient's father also had autism, as did two paternal half-brothers.</p><p>The patient's brain MRI showed periventricular leukomalacia. Genetic testing revealed a maternally-inherited <i>PCDH19</i> pathogenic variant (NM_001184880.2, c.1201_1202dup, p.(Ser401Argfs*169)).</p><p>The proband is now 21 months old and has had 5 more admissions with seizure clusters over the past 12 months; however, ictal bradycardia/asystole has not again been reported. She is currently taking topiramate, carbamazepine, clobazam, and levetiracetam. From a developmental perspective, she can pull to stand and walk one or two steps with support. She babbles and says “dada” and “mama.” There has been no regression.</p><p>Using the ECG derivation from the scalp EEG recording, we extracted interbeat intervals during all seizures, as well as for ","PeriodicalId":50508,"journal":{"name":"Epileptic Disorders","volume":"26 6","pages":"882-885"},"PeriodicalIF":1.9,"publicationDate":"2024-10-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/epd2.20281","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142394929","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Identifying the infraslow activity associated with faciobrachial dystonic seizures in EEG","authors":"Richard Wennberg, Julien Hébert, David Tang-Wai","doi":"10.1002/epd2.20296","DOIUrl":"10.1002/epd2.20296","url":null,"abstract":"<p>The tonic-dystonic motor spasms or faciobrachial dystonic seizures (FBDS) pathognomonic of anti-LGI1 encephalitis have been shown to be associated with a preceding cortical change visible in EEG as a phasic DC (direct current) shift, or infraslow activity (ISA).<span><sup>1, 2</sup></span> At usual clinical filter settings, the ISA may be difficult to appreciate and is often unrecognized. Best seen in common average or linked ears reference montages, ISA precedes the spasms by ~1 s over the contralateral frontal lobe,<span><sup>2</sup></span> in some patients maximal over the mid frontal area and in others the frontocentral region. When reviewed using a longer time constant (e.g., the 0.07 Hz analog filter of the clinical amplifier, with no added digital high-pass filter, as shown in Video 1, Figure 1), the ISA is more readily apparent. In some patients, FBDS may be very subtle (see later examples in Video 1) and thus identifying the characteristic EEG features can have diagnostic importance.</p>","PeriodicalId":50508,"journal":{"name":"Epileptic Disorders","volume":"27 1","pages":"121-122"},"PeriodicalIF":1.9,"publicationDate":"2024-10-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/epd2.20296","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142394930","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Nothing about us all, without us all—Time for a second look at our exclusion criteria?","authors":"Claire Behan","doi":"10.1002/epd2.20297","DOIUrl":"10.1002/epd2.20297","url":null,"abstract":"","PeriodicalId":50508,"journal":{"name":"Epileptic Disorders","volume":"27 1","pages":"135-136"},"PeriodicalIF":1.9,"publicationDate":"2024-10-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142378497","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Reactivity of EEG patterns is a crucial indicator to determine the EEG is not ictal: A case of topiramate overdose","authors":"Philippe Gélisse, Arielle Crespel","doi":"10.1002/epd2.20298","DOIUrl":"10.1002/epd2.20298","url":null,"abstract":"<p>Hleuhel et al. reported the case of baclofen intoxication with generalized periodic discharges at 2.6 Hz, fulfilling the Salzburg criteria for definite nonconvulsive status epilepticus (NCSE).<span><sup>1</sup></span> However, auditory stimulation led transiently to the termination of the epileptiform activity and the authors concluded that the EEG pattern was the consequence of a toxic encephalopathy and that the strict application of the Salzburg criteria for NCSE may lead to an overestimation of NCSE in baclofen intoxication.</p><p>Overdose of topiramate may result in a coma, seizures, hemodynamic instability, and severe metabolic acidosis.<span><sup>2</sup></span> We report a 10.5-year-old boy who attempted suicide with 700 mg of topiramate (his mother's antiseizure medication) resulting in a confusional state and low-serum bicarbonate on a blood test (16 mmol/L). His EEG showed when his eyes were closed, a bilateral rhythmic activity at 4.5 Hz with a sinusoidal aspect (Figure 1A; Supporting Information). The anterior rhythmic theta waves disappeared upon eye-opening but were still present on the posterior regions, especially on the left side (Figure 1B; Supporting Information). The reactivity to eye-opening eliminates an absence status epilepticus (ASE). The patient recovered spontaneously and the control EEG performed 3 days later was normal.</p><p>EEG reactivity refers to a change in the EEG background activity in response to stimulation (change in amplitude and/or frequency, including attenuation of activity),<span><sup>3</sup></span> and is considered a marker of good prognosis in comatose patients, especially after a cardiac arrest.<span><sup>3</sup></span> With the strict application of the Salzburg criteria for NCSE,<span><sup>4</sup></span> the EEG of our patient with continuous bilateral epileptiform discharges >2.5 Hz in association with a confusional state corresponded to NCSE. There was also a fluctuation of the morphology of the pattern with a sinusoidal aspect, but unusual for an epileptic activity. This EEG pattern with a sinusoidal aspect differentiates it from an epileptic seizure, but more importantly, the reactivity to eye-opening allowed us definitively to retain a toxic encephalopathy and no antiseizure medication has been given. Due to self-perpetuating processes and the failure of self-terminating mechanisms, NCSE epilepticus is unlikely to cease transitory when patients open their eyes, except in patients with epilepsy with eyelid myoclonia (Jeavons syndrome) where the discharges in the case of ASE may be fragmented by eyes opening.<span><sup>5</sup></span> Testing of the reactivity is an easy and safe test that must always be carried out when there is any doubt regarding an NCSE.<span><sup>6</sup></span></p><p>We confirm that we have read the Journal's position on issues involved in ethical publication and affirm that this report is consistent with those guidelines.</p><p>Philippe Gélisse: Conceptualization, Wr","PeriodicalId":50508,"journal":{"name":"Epileptic Disorders","volume":"27 1","pages":"137-138"},"PeriodicalIF":1.9,"publicationDate":"2024-10-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/epd2.20298","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142378498","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Eivind Aanestad, Sándor Beniczky, Henning Olberg, Jan Brogger
{"title":"Unveiling variability: A systematic review of reproducibility in visual EEG analysis, with focus on seizures","authors":"Eivind Aanestad, Sándor Beniczky, Henning Olberg, Jan Brogger","doi":"10.1002/epd2.20291","DOIUrl":"10.1002/epd2.20291","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Objective</h3>\u0000 \u0000 <p>Reproducibility is key for diagnostic tests involving subjective evaluation by experts. Our aim was to systematically review the reproducibility of visual analysis in clinical electroencephalogram (EEG). In this paper, we give data on the scope of EEG features found, and detailed reproducibility data for the most studied feature.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We searched four databases for articles reporting reproducibility in clinical EEG, until June 2023. Two raters screened 24 553 citations, and then 2736 full texts. Quality was assessed according to the GRRAS guidelines.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>We found 275 studies (268 interrater and 20 intrarater), addressing 606 different EEG features. Only 38 EEG features had been studied in >2 studies. Most studies had <50 patients and EEGs. The most often addressed feature was seizure detection (62 papers). Interrater reproducibility of seizure detection was substantial-to-almost-perfect with experienced raters and raw EEG (kappa .62–.88). With experienced raters and transformed EEG, reproducibility was substantial (kappa .63–.70). Inexperienced raters had lower reproducibility. Seizure lateralization reproducibility was moderate to substantial (kappa .58–.77) but lower than for seizure detection.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Significance</h3>\u0000 \u0000 <p>Most EEG reproducibility studies are done only once. Intrarater studies are rare. The reproducibility of visual EEG analysis is variable. Interrater reproducibility for seizure detection is substantial-to-perfect with experienced raters and raw EEG, less with inexperienced raters or transformed EEG.</p>\u0000 \u0000 <p>The results of visual EEG analysis vary within the same rater, and between raters. There is a need for larger collaborative studies, using improved methodology, as well as more intrarater studies of EEG interpretation.</p>\u0000 </section>\u0000 </div>","PeriodicalId":50508,"journal":{"name":"Epileptic Disorders","volume":"26 6","pages":"827-839"},"PeriodicalIF":1.9,"publicationDate":"2024-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/epd2.20291","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142331669","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Melanie Nana, Surabhi Nanda, Jennifer Nightingale, Maryann Butchers, Anita Banerjee, Sonji Clarke, Catherine Williamson, William Stern, Catherine Nelson-Piercy
{"title":"Peripartum seizures in women with epilepsy and multidisciplinary considerations as to how they can be prevented","authors":"Melanie Nana, Surabhi Nanda, Jennifer Nightingale, Maryann Butchers, Anita Banerjee, Sonji Clarke, Catherine Williamson, William Stern, Catherine Nelson-Piercy","doi":"10.1002/epd2.20283","DOIUrl":"10.1002/epd2.20283","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Objective</h3>\u0000 \u0000 <p>Seizures during labor are reported in 3.5% of women with epilepsy (WWE) and can result in both maternal and fetal morbidity. In response to an anecdotal increase in WWE developing seizures in labor or peripartum (up to 24 h post-partum), a review of patients managed in our service was undertaken to define the incidence of peripartum seizures and determine learning points.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>A retrospective review of all cases of WWE having peripartum seizures from 2017 to 2022 in our institution was undertaken. Each case was reviewed by the multidisciplinary team (MDT) and common themes identified.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>In total, 106 WWE received pregnancy care in the study period, of whom 8/106 (7.5%) had a seizure in the peripartum period. The MDT-agreed learning points included importance of pre-pregnancy counseling, prompt up-titration of antiepileptic drugs where indicated and recommendations for the management in the peripartum period.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Significance</h3>\u0000 \u0000 <p>We concluded that the peripartum period remains a high-risk time for seizures in WWE. There is little evidence to support guidelines for the management of WWE in the peripartum period. In the absence of this evidence, sharing experience may help those managing such women to improve care and reduce risk during this vulnerable time.</p>\u0000 </section>\u0000 </div>","PeriodicalId":50508,"journal":{"name":"Epileptic Disorders","volume":"26 6","pages":"797-803"},"PeriodicalIF":1.9,"publicationDate":"2024-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142331667","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Daniel Filipe Borges, Giulia Primicerio, Radu-Ștefan Perjoc, Lars Ølgaard Bloch, Melita Cacic Hribljan
{"title":"The Dianalund experience: A review of the 6th ILAE School on Advanced EEG and Epilepsy","authors":"Daniel Filipe Borges, Giulia Primicerio, Radu-Ștefan Perjoc, Lars Ølgaard Bloch, Melita Cacic Hribljan","doi":"10.1002/epd2.20289","DOIUrl":"10.1002/epd2.20289","url":null,"abstract":"","PeriodicalId":50508,"journal":{"name":"Epileptic Disorders","volume":"26 6","pages":"898-901"},"PeriodicalIF":1.9,"publicationDate":"2024-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142331668","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Sleep-related hypermotor seizures originating from the occipital lobe","authors":"Lara Wadi, Mays Khweileh, Shruti Agashe, Derek Southwell, Prachi Parikh, Birgit Frauscher","doi":"10.1002/epd2.20285","DOIUrl":"10.1002/epd2.20285","url":null,"abstract":"<p>We present two unique cases of sleep-related hypermotor epilepsy (SHE) originating from the occipital lobe. Patients with sleep-related seizures and drug-resistant occipital lobe epilepsy were identified from the ANPHY lab stereo-electroencephalography (SEEG) research database at the Duke Comprehensive Epilepsy Center. We identified two young females with frequent sleep-related focal seizures and occasional focal to bilateral tonic clonic seizures characterized by hypermotor movements. During wakefulness, the semiology also involved an elementary visual aura. They meet the 2016 diagnostic criteria for SHE, and SEEG monitoring with cortical stimulation mapping identified an epileptogenic zone (EZ) within the occipital lobe, with most seizures occurring out of NREM 2 sleep. Responsive neurostimulation devices were implanted, which indicated a trend for event detections in nocturnal periods. Extrafrontal SHE has characteristically been described in the temporal, insular-opercular, and parietal lobes. Here, we demonstrate using SEEG-confirmed EZ identification, that SHE can also originate in the occipital lobe. In patients with sleep-related seizures and hypermotor behavior, occipital lobe seizures thus should not be excluded from the differential diagnosis. Key in identifying this rare localization is non-frontal aura semiology and delay to motor symptoms, which may be supported by a visual field deficit and structural MRI abnormality.</p>","PeriodicalId":50508,"journal":{"name":"Epileptic Disorders","volume":"26 6","pages":"868-874"},"PeriodicalIF":1.9,"publicationDate":"2024-09-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142300030","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}