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The objective of this study was to determine seizure control in women with epilepsy (WWE) undergoing assisted reproductive technology (ART). Through retrospective chart review, WWE undergoing ART were identified. Demographics and details regarding epilepsy type, seizure control, and ART procedures were extracted. Seizure frequency prior to and during ART were compared. We identified 12 WWE, who underwent 29 embryo transfers, resulting in 16 pregnancies and 10 live births. Nine women were seizure-free at least 2 years before fertility treatment, including three with resolved epilepsy. Seven were on antiseizure medications throughout fertility treatment and pregnancy, with only one on polytherapy. Eleven (all with controlled epilepsy or epilepsy in remission) remained seizure-free throughout fertility treatment. One woman with drug-resistant epilepsy continued to have seizures throughout fertility treatment and pregnancy without an exacerbation of seizure frequency. There was no increased seizure frequency associated with fertility treatment and subsequent pregnancy in this small series of WWE. Although this study was statistically underpowered, our results provide some preliminary evidence that ART might not pose a threat to seizure control, but larger, confirmatory studies are necessary., (© 2023 International League Against Epilepsy.)

Background and Objectives: To determine risk factors associated with clinical relapses and development of chronic epilepsy in patients with anti-leucine-rich glioma-inactivated 1 (LGI1) immunoglobulin G encephalitis., Methods: Patients with seizures related to LGI1-antibody encephalitis with ≥24 months of follow-up from disease onset were identified in the Mayo Clinic electronic medical record and neuroimmunology laboratory records. Charts were reviewed to determine clinical factors, seizure types, imaging, treatment, occurrence of relapse, and outcome. Binary logistic regression analysis was performed to identify predictors of the development of chronic epilepsy. Univariate Cox proportional hazards regression was used to examine the influence of baseline characteristics on relapse risk., Results: Forty-nine patients with LGI1-antibody encephalitis and acute symptomatic seizures were identified. Almost all patients (n = 48, 98%) were treated with immunotherapy. Eight had definite and 2 had possible chronic epilepsy at the last follow-up (10 of 49, 20.4%). Female sex ( p = 0.048) and younger age at disease onset ( p = 0.02) were associated with development of chronic epilepsy. Relapses occurred in 20 (40.8%), with a median time to first relapse of 7.5 months (range 3-94 months). Initial treatment with long-term steroid-sparing immunotherapy was associated with reduced risk of relapse (hazard ratio 0.28, 95% confidence interval 0.11-0.73, p = 0.009)., Discussion: Chronic epilepsy occurred in 20.4% of our patients with LGI1-antibody encephalitis despite aggressive immunotherapy. Risk factors for chronic epilepsy were female sex and earlier age at onset. Relapses occurred in 40.8% of patients with prolonged follow-up, and long-term steroid-sparing immunotherapy was associated with a lower relapse rate., (© 2021 American Academy of Neurology.)

Recognition of autoimmunity as a cause of encephalopathy has increased. Recent studies have validated the use of Antibody-Prevalence-in-Epilepsy (APE) and Responsive-to-immunotherapy-in-Epilepsy (RITE) scores in the evaluation and management of autoimmune-epilepsy. We aim to assess the utility of these models for patients with cognitive dysfunction. Among the evaluated patients, 17% had antibodies universally associated with autoimmune-encephalopathy. NMDA-R-IgG and LGI1-IgG were the most common antibody specificities. Antibody-Prevalence-in-Epilepsy-and-Encephalopathy (APE 2 ) score ≥ 4 was 99% sensitive and 93% specific for neural-specific-antibodies. Responsive-to-immunotherapy-in-Epilepsy-and-Encephalopathy (RITE 2 ) score ≥ 7 had 96% sensitivity and 86% specificity for favorable initial immunotherapy response. Application of these models may optimize autoantibody evaluations and immunotherapeutic trials., (Copyright © 2018. Published by Elsevier B.V.)

Objective: We aimed to determine the frequency of probable obstructive sleep apnea (pOSA) in refractory epilepsy monitoring unit inpatients and clinical features associated with pOSA, including risk for sudden unexpected death in epilepsy (SUDEP)., Methods: We prospectively recruited 49 consecutive adult patients admitted to the Mayo Clinic Epilepsy Monitoring Unit with focal, generalized, or unclassified epilepsy syndromes. pOSA was identified using oximetric oxyhemoglobin desaturation index (ODI) and the Sleep Apnea-Sleep Disorders Questionnaire (SA-SDQ) and STOP-BAG screening tools. Revised SUDEP Risk Inventory (rSUDEP-7) scores were calculated, and epilepsy patients with and without pOSA were compared with Wilcoxon signed-rank tests. Correlation and regression analyses were utilized to determine relationships between pOSA and rSUDEP-7 scores., Results: Thirty-five percent of patients had pOSA, with a mean ODI of 11.3 ± 5.1/h (range = 5.1-22.8). Patients with pOSA were older and heavier, and more frequently had a focal epilepsy syndrome and longer epilepsy duration, with higher SA-SDQ and STOP-BAG scores (all P < 0.05). Median rSUDEP-7 score was 3 ± 1.4 (range = 0-6). Higher rSUDEP-7 scores were positively correlated with higher ODI (P = 0.036). rSUDEP-7 score ≥ 5 was associated with pOSA by ODI, SA-SDQ, and STOP-BAG questionnaire criteria (P < 0.05)., Significance: Our pilot study identified a high frequency of pOSA in refractory epilepsy monitoring patients, finding that pOSA patients were older and heavier, with higher screening symptoms for sleep apnea and more frequent focal seizures with a longer epilepsy duration. We also found a possible association between OSA and SUDEP risk. Identification and treatment of OSA in patients with epilepsy could conceivably provide a novel approach toward preventing the risk of SUDEP. Future studies with polysomnography are needed to confirm predictive features for OSA in epilepsy populations, and to determine whether OSA is associated with SUDEP risk., (Wiley Periodicals, Inc. © 2018 International League Against Epilepsy.)

Objective: To determine the occurrence of chronic traumatic encephalopathy (CTE) in young adult patients undergoing epilepsy surgery., Methods: Ten patients who underwent epilepsy surgery were randomly selected for this retrospective study. The patients were 18-45 years of age, had preoperative neuropsychological evaluation, and had 1 year postoperative follow-up. Microscopic sections from resections were evaluated for the presence of CTE with standard stains and antibodies to tau (clone AT8)., Results: The median age at resection was 32.5 years (range 23-43) and the median duration of seizures was 23.5 years (range 3-28). Eight had a history of head injury. Preoperative neuropsychological testing showed mild to moderate cognitive impairment in 8 patients (80%). Pathologic examination in one patient showed focal sparse tau-immunoreactive lesions along descending rami and cortical gyral depths of the resected frontal lobe. Nine patients had no evidence of CTE. All focal cortical resections showed variable subpial and subcortical gliosis commonly identified in patients with chronic seizure disorders., Conclusions: The present small retrospective observational study suggests that CTE may occur, but appears uncommon, in young adult patients undergoing surgical treatment for drug-resistant focal epilepsy. The significance of these findings requires further investigation to define the relative importance of tau accumulation in younger adult patients with drug-resistant focal epilepsy and cognitive decline., (© 2018 American Academy of Neurology.)

Objective: To validate predictive models for neural antibody positivity and immunotherapy response in epilepsy., Methods: We conducted a retrospective study of epilepsy cases at Mayo Clinic (Rochester-MN; Scottsdale-AZ, and Jacksonville-FL) in whom autoimmune encephalopathy/epilepsy/dementia autoantibody testing profiles were requested (06/30/2014-06/30/2016). An Antibody Prevalence in Epilepsy (APE) score, based on clinical characteristics, was assigned to each patient. Among patients who received immunotherapy, a Response to Immunotherapy in Epilepsy (RITE) score was assigned. Favorable seizure outcome was defined as >50% reduction of seizure frequency at the first follow-up., Results: Serum and cerebrospinal fluid (CSF) from 1,736 patients were sent to the Mayo Clinic Neuroimmunology Laboratory for neural autoantibody evaluation. Three hundred eighty-seven of these patients met the diagnostic criteria for epilepsy. Central nervous system (CNS)-specific antibodies were detected in 44 patients. Certain clinical features such as new-onset epilepsy, autonomic dysfunction, viral prodrome, faciobrachial dystonic seizures/oral dyskinesia, inflammatory CSF profile, and mesial temporal magnetic resonance imaging (MRI) abnormalities had a significant association with positive antibody results. A significantly higher proportion of antibody-positive patients had an APE score ≥4 (97.7% vs. 21.6%, p < 0.01). Sensitivity and specificity of an APE score ≥4 to predict presence of specific neural auto-antibody were 97.7% and 77.9%, respectively. In the subset of patients who received immunotherapy (77), autonomic dysfunction, faciobrachial dystonic seizures/oral dyskinesia, early initiation of immunotherapy, and presence of antibodies targeting plasma membrane proteins (cell-surface antigens) were associated with favorable seizure outcome. Sensitivity and specificity of a RITE score ≥7 to predict favorable seizure outcome were 87.5% and 83.8%, respectively., Significance: APE and RITE scores can aid diagnosis, treatment, and prognostication of autoimmune epilepsy. A PowerPoint slide summarizing this article is available for download in the Supporting Information section here., (Wiley Periodicals, Inc. © 2017 International League Against Epilepsy.)

Importance: The implications of stimulus-induced rhythmic, periodic, or ictal discharges (SIRPIDs) sometimes found on prolonged electroencephalographic (EEG) recordings are uncertain., Objective: To evaluate the incidence of SIRPIDs and their clinical implications in critically ill patients., Design, Setting, and Participants: A multicenter, international retrospective study was performed from October 1, 2012, through September 30, 2014, of consecutive adult patients hospitalized in intensive care units with alteration of consciousness who underwent EEG recordings at 3 separate centers. Demographic data, including admission diagnosis, age, sex, history of epilepsy, and EEG findings, were noted. Characteristics of SIRPIDs were documented. Data were evaluated for predictors of SIRPIDs and in-hospital mortality. Data analysis was performed from January 16, 2015, to June 15, 2015., Main Outcomes and Measures: Incidence of SIRPIDs, association of SIRPIDs with mortality and other EEG characteristics, and EEG and clinical predictors of mortality., Results: A total of 416 patients were studied. The median age of patients was 60 years (interquartile range, 46-71 years), and 252 (60.6%) were male. A total of 104 patients (25.0%) did not survive to hospital discharge. SIRPIDs were identified in 43 patients (10.3%). The proportion of patients with SIRPIDs was not significantly different across the 3 sites (P = .34). Anoxic brain injury (odds ratio [OR], 3.80; 95% CI, 1.73-8.33; P < .001), the use of antiepileptic medications (OR, 3.24; 95% CI, 1.31-8.00; P = .01), electrographic seizures (OR, 2.85; 95% CI, 1.13-7.19; P = .03), generalized periodic discharges with triphasic morphologic features (OR, 3.66; 95% CI, 1.67-8.02; P = .001), and sporadic sharp waves and periodic discharges (OR, 2.59; 95% CI, 1.13-5.92; P = .02) were independently associated with the presence of SIRPIDs. Older age (OR, 1.02; 95% CI, 1.01-1.04; P = .005), anoxic brain injury (OR, 3.49; 95% CI, 1.96-6.21; P ≤ .001), and absence of EEG reactivity (OR, 8.14; 95% CI, 4.20-15.79; P < .001) but not SIRPIDs (OR, 1.73; 95% CI, 0.79-3.78; P = .17) were independently associated with in-hospital mortality., Conclusions and Relevance: In critically ill patients undergoing EEG recordings, SIRPIDs occurred in 43 (10.3%) and were associated with other electrographic abnormalities previously reported to indicate poor prognosis. However, SIRPIDs were not independently associated with in-hospital mortality.

Objective: To compare the yield of epileptiform abnormalities on 30-minute recordings with those greater than 45 minutes., Methods: We performed a prospective observational cross-sectional study of all outpatient routine EEGs comparing the rate of interictal epileptiform discharges (IEDs) and clinical events during the initial 30 minutes (routine) with those occurring in the remaining 30-60 minutes (extended). A relative increase of 10% was considered clinically significant., Results: EEGs from 1,803 patients were included; overall EEG duration was 59.4 minutes (SD ±6.5). Of 426 patients with IEDs at any time during the EEG, 81 (19.1%, 95% confidence interval 15.6-23) occurred only after the initial 30 minutes. The rate of late IEDs was not associated with age, indication, IED type, or sleep deprivation. Longer recording times also increased event capture rate by approximately 30%., Conclusions: The yield of IED and event detection is increased in extended outpatient EEGs compared to 30-minute studies., (© 2016 American Academy of Neurology.)

Objective: To evaluate the benefit of statistical SPECT processing over traditional subtraction methods, we compared ictal-interictal SPECT analyzed by statistical parametric mapping (SPM) (ISAS), statistical ictal SPECT coregistered to MRI (STATISCOM), and subtraction ictal-interictal SPECT coregistered with MRI (SISCOM) in patients with MRI-negative focal temporal lobe epilepsy (nTLE) and extratemporal lobe epilepsy (nETLE)., Methods: We retrospectively identified 49 consecutive cases of drug-resistant focal epilepsy that had a negative preoperative MRI and underwent interictal and ictal SPECT prior to resective epilepsy surgery. Interictal and ictal SPECT scans were analyzed using SISCOM, ISAS, and STATISCOM to create hyperperfusion and hypoperfusion maps for each patient. Reviewers blinded to clinical data and the SPECT analysis method marked the site of probable seizure origin and indicated their confidence in the localization., Results: In nTLE and nETLE, the hyperperfusions detected by STATISCOM (71% nTLE, 57% nETLE) and ISAS (67% nTLE, 53% nETLE) were more often colocalized with surgery resection site compared to SISCOM (38% nTLE, 36% nETLE). In nTLE, localization of the hyperperfusion to the region of surgery was associated with an excellent outcome for STATISCOM (p = 0.005) and ISAS (p = 0.027), but not in SISCOM (p = 0.071). This association was not present in nETLE for any method., Conclusion: In an unselected group of patients with normal MRI and focal epilepsy, SPM-based methods of SPECT processing showed better localization of SPECT hyperperfusion to surgical resection site and higher interobserver agreement compared to SISCOM. These results show the benefit of statistical SPECT processing methods and further highlight the challenge of nETLE.

Cerebral oximetry has not been explored in patients experiencing seizures in the epilepsy monitoring unit (EMU). The purpose of our study was to evaluate the feasibility of periictal measurement of cerebral oxygenation using noninvasive cerebral tissue oximetry and to determine whether there was evidence of cerebral hypoxemia during generalized seizures. Cerebral oxygen saturation findings were subsequently correlated with sudden unexpected death in epilepsy (SUDEP) risk factors. We prospectively evaluated six patients admitted to our EMU with histories of generalized tonic-clonic seizures (GTCS) with prolonged scalp electroencephalography (EEG) and two regional cerebral oxygen saturation (rSO(2)) sensors. Minimum rSO(2) values were recorded in the 5 min preceding seizure onset, during the seizure, and in the 5 min following seizure offset. SUDEP risk was assessed using the SUDEP-7 Inventory. Cerebral oximetry was well tolerated, with a mean duration of rSO(2) monitoring of 81.1 h. Cerebral oxygen saturation data were available from at least one sensor in 9 (90%) of 10 seizures; only 6 (60%) of 10 seizures had useable periictal digital pulse oximetry data. GTCS were associated with significantly lower minimum ictal (p = 0.003) and postictal (p = 0.004) %rSO(2) values than the minimum preictal value. Patients with at least one seizure with a %rSO(2) decrease of ≥20% tended to have higher SUDEP-7 Inventory scores (mean SUDEP-7 Inventory score 7 ± 2.8) versus patients without recorded desaturations (4.3 ± 0.5, p = 0.08). Larger studies are needed to determine the value of cerebral oximetry in the identification of patients at risk of SUDEP., (Wiley Periodicals, Inc. © 2012 International League Against Epilepsy.)

Objective: To describe clinical characteristics and immunotherapy responses in patients with autoimmune epilepsy., Design: Observational, retrospective case series., Setting: Mayo Clinic Health System., Patients: Thirty-two patients with an exclusive (n=11) or predominant (n=21) seizure presentation in whom an autoimmune etiology was suspected (on the basis of neural autoantibody [91%], inflammatory cerebrospinal fluid [31%], or magnetic resonance imaging suggesting inflammation [63%]) were studied. All had partial seizures: 81% had failed treatment with 2 or more antiepileptic drugs and had daily seizures and 38% had seizure semiologies that were multifocal or changed with time. Head magnetic resonance imaging was normal in 15 (47%) at onset. Electroencephalogram abnormalities included interictal epileptiform discharges in 20; electrographic seizures in 15; and focal slowing in 13. Neural autoantibodies included voltage-gated potassium channel complex in 56% (leucine-rich, glioma-inactivated 1 specific, 14; contactin-associated proteinlike 2 specific, 1); glutamic acid decarboxylase 65 in 22%; collapsin response- mediator protein 5 in 6%; and Ma2, N-methyl-D-aspartate receptor, and ganglionic acetylcholine receptor in 1 patient each., Intervention: Immunotherapy with intravenous methylprednisolone; intravenous immune globulin; and combinations of intravenous methylprednisolone, intravenous immune globulin, plasmapheresis, or cyclophosphamide., Main Outcome Measure: Seizure frequency., Results: After a median interval of 17 months (range, 3-72 months), 22 of 27 (81%) reported improvement postimmunotherapy; 18 were seizure free. The median time from seizure onset to initiating immunotherapy was 4 months for responders and 22 months for nonresponders (P<.05). All voltage-gated potassium channel complex antibody-positive patients reported initial or lasting benefit (P<.05). One voltage-gated potassium channel complex antibody-positive patient was seizure free after thyroid cancer resection; another responded to antiepileptic drug change alone., Conclusion: When clinical and serological clues suggest an autoimmune basis for medically intractable epilepsy, early-initiated immunotherapy may improve seizure outcome.

To evaluate the association between cerebral hypoperfusion and seizure termination, we compared seizure duration in seven patients with syncopal ictal asystole (IA), seven with non-syncopal ictal bradycardia, and ten with non-bradycardic seizures. Mean seizure duration was 34.4±13 s in IA, 67±28.9 s in ictal bradycardia, and 82.1±31.1 in non-bradycardic seizures. These were significantly different (ANOVA, p<0.02). This suggests cerebral hypoxia-ischemia favors seizure termination., (Copyright © 2011 Elsevier B.V. All rights reserved.)

Ictal asystole may contribute to seizure-related injury and mortality. The purpose of this study was to evaluate the effect of cardiac pacing on seizure-related injury rates in ictal asystole patients. A survey was conducted to determine seizure-related fall rate and other morbidity in all seven patients with ictal asystole who underwent cardiac pacing at our institution between 1990 and 2004. The rate of seizure-related falls and other morbidities before and after pacing were compared using the Wilcoxon rank-sum test. The mean fall rate was 3.28 falls/month pre-pacemaker implantation. Following pacemaker implantation, this was reduced to 0.005 falls/month (p = 0.001). Seizure-related fractures and motor vehicle accidents were also reduced following cardiac pacing. These findings may have implications in mitigating the potential morbidity associated with ictal asystole., (Wiley Periodicals, Inc. © 2011 International League Against Epilepsy.)

The question of the advisability of antiepileptic drug (AED) discontinuance is common in epilepsy care. With currently available therapies, prolonged periods of seizure remission are not uncommon. After a prolonged period of seizure freedom, patients and clinicians often begin to wonder whether therapy can be discontinued. The AED discontinuance literature shows relapse rates ranging from 20% to 60%. Factors with prognostic significance have been recognized, including several findings identified on the routine EEG. This article reviews the role of EEG in the setting of AED discontinuance and summarizes the literature describing the prognostic value of EEG.

Purpose: To evaluate the diagnostic yield and clinical impact of chloral hydrate (CH) in the routine EEG., Methods: The EEG results and records of all patients receiving CH during routine EEG (CH-EEG) at Mayo Clinic Rochester between 4/1/07 and 9/30/07 (n=216 total; 148 adults) were reviewed for clinical indication, presence of epileptiform abnormalities and EEG duration. The results were compared to an equivalent number of consecutive EEGs performed without CH over the same time period (non-CH-EEGs). The clinical impact of the CH-EEG findings was evaluated by evaluating resulting clinical decisions made in the medical record. Chi-squared analysis was performed for discontinuous data, Student's t-test for continuous data., Results: The proportion of EEGs with sleep-specific epileptiform abnormalities (SSEAs) was not statistically different (7.8% CH-EEG vs. 10.5% non-CH-EEG, p=0.34). CH was ordered more often by non-epileptologists than epileptologists (57% vs. 35%, p=0.0004). The mean acquisition time was greater for CH-EEGs (66.9min vs. 55.1min; p<<0.001). CH-EEGs resulted in a change in clinical management in 5/216 (2.3%)., Discussion: Compared to non-CH-EEGs, CH-EEGs were no more likely to show SSEAs, prolonged the acquisition time, and were associated with changes in clinical care in <3%. Routine use of CH in outpatient EEG is not strongly supported by these data.

Object: Patients with normal MR imaging (nonlesional) findings and medically refractory extratemporal epilepsy make up a disproportionate number of nonexcellent outcomes after epilepsy surgery. In this paper, the authors investigated the usefulness of intracranial electroencephalography (iEEG) in the identification of surgical candidates., Methods: Between 1992 and 2002, 51 consecutive patients with normal MR imaging findings and extratemporal epilepsy underwent intracranial electrode monitoring. The implantation of intracranial electrodes was determined by seizure semiology, interictal and ictal scalp EEG, SPECT, and in some patients PET studies. The demographics of patients at the time of surgery, lobar localization of electrode implantation, duration of follow-up, and Engel outcome score were abstracted from the Mayo Rochester Epilepsy Surgery Database. A blinded independent review of the iEEG records was conducted for this study., Results: Thirty-one (61%) of the 51 patients who underwent iEEG ultimately underwent resection for their epilepsy. For 28 (90.3%) of the 31 patients who had epilepsy surgery, adequate information regarding follow-up (> 1 year), seizure frequency, and iEEG recordings was available. Twenty-six (92.9%) of 28 patients had frontal lobe resections, and 2 had parietal lobe resections. The most common iEEG pattern at seizure onset in the surgically treated group was a focal high-frequency discharge (in 15 [53.6%] of 28 patients). Ten (35.7%) of the 28 surgically treated patients were seizure free. Fourteen (50%) had Engel Class I outcomes, and overall, 17 (60.7%) had significant improvement (Engel Class I and IIAB with > or =80% seizure reduction). Focal high-frequency oscillation at seizure onset was associated with Engel Class I surgical outcome (12 [85.7%] of 14 patients, p = 0.02), and it was uncommon in the nonexcellent outcome group (3 [21.4%] of 14 patients)., Conclusions: A focal high-frequency oscillation (> 20 Hz) at seizure onset on iEEG may identify patients with nonlesional extratemporal epilepsy who are likely to have an Engel Class I outcome after epilepsy surgery. The prospect of excellent outcome in nonlesional extratemporal lobe epilepsy prior to intracranial monitoring is poor (14 [27.5%] of 51 patients). However, iEEG can further stratify patients and help identify those with a greater likelihood of Engel Class I outcome after surgery.

Objective: The occurrence of asystole during an epileptic seizure is the most dramatic manifestation of ictal bradycardia. Recognition of ictal asystole is important as treatment with both antiepileptic drugs and cardiac pacing may be necessary. The purpose of this study was to identify clinical cues to aid in the detection of ictal asystole., Methods: We analyzed the clinical and electrophysiologic features of all recorded seizures in consecutive patients diagnosed with ictal bradycardia and asystole on prolonged video-EEG/ECG monitoring over a 14 year period., Results: Twenty-nine seizures with ictal bradycardia were identified in 13 patients. Of these, 11 seizures in seven patients were associated with asystole. Bilateral transient limb movements consisting of asymmetric posturing or jerking occurred during eight episodes of ictal asystole, and loss of muscle tone occurred during four. In contrast, neither bilateral limb movement nor loss of muscle tone was observed in any of the non-asystolic seizures, whether ictal bradycardia was present or not. All patients with ictal asystole reported a history of seizure-related falls and brief body and limb jerking., Conclusions: The presence of loss of muscle tone or bilateral asymmetric jerky limb movements during a seizure suggests the possibility of ictal asystole. Video-EEG/ECG monitoring should be considered in patients with epilepsy demonstrating these clinical features to determine if ictal asystole is present.

Cardiac troponin concentrations are important sensitive and specific markers for myocardial injury in clinical medicine. Troponin (TnT) elevations have been noted in some series in the setting of acute neurologic disease. We have previously reported that solitary seizures do not evoke elevations in TnT. The importance of this negative finding is exemplified by a patient who arrived at our clinic following a new onset seizure and in whom the cardiac TnT level was observed to rise. Triggered by this observation and by the knowledge that seizures alone would not do this, a subsequent targeted cardiologic workup documented what was believed to be an extension of a previously unrecognized myocardial infarction, with a seizure as its clinical presentation. Elevations of troponin should not be considered to be due to isolated seizures. This case illustrates the importance of having data concerning the response of troponin in various emergency and clinical situations.

Although pathophysiologically distinct, syncope and seizures share clinical characteristics which may make diagnosis difficult. Adding to diagnostic complexity are the facts that seizures and syncope may coexist in the same patient, syncope may be associated with seizure-like motor manifestations, and seizures may be complicated by cardiac arrhythmia and syncope. Combined EEG/ECG telemetry is sometimes necessary to establish the correct diagnosis. These techniques also provide an opportunity to study the role of certain cortical regions in the modulation of cardiac function. There is an increasing understanding of the central autonomic pathways involved in the genesis of the cardiovascular changes that occur during epileptic seizures. This article reviews the use of EEG/ECG telemetry in the evaluation of syncope and seizures, and the neuroanatomic circuitry involved in the production of the cardiovascular manifestations of seizures.

Neurostimulation is a recent development in the treatment of epilepsy. Vagus nerve stimulation (VNS), the only approved neurostimulation therapy for epilepsy to date, has proved to be a viable adjunctive treatment option. The exact mechanism of action of VNS is not fully understood. In 2 randomized double-blind trials, seizure frequency declined approximately 30% after 3 months of treatment. Long-term follow-up studies suggest that response improves over time, with approximately 35% of patients experiencing a 50% reduction and 20% experiencing a 75% reduction in seizure frequency after 18 months of treatment. Unfortunately, the number of patients rendered medication-free and seizure-free with VNS is low. Vagus nerve stimulation is best viewed as an option for patients who are not surgical candidates or who hesitate to take the risk of surgery yet continue to have seizures despite maximal medical therapy. Stimulation of other regions of the central nervous system for treating epilepsy, including the anterior and centromedian nuclei of the thalamus, the hippocampus, the subthalamic nucleus, and the cerebral neocortex, is currently under investigation. We review the history, proposed mechanisms of action, clinical trials, adverse effects, and future direction of VNS and other modalities of neurostimulation therapy for epilepsy.

Of patients with epilepsy, 60% to 70% achieve control with antiepileptic medication. Antiepileptic drugs may be associated with unwanted adverse effects, inconvenience, and cost. Remission may occur in some patients, raising the issue of whether continued treatment is necessary. Identifying patients from whom treatment can be withdrawn successfully would be beneficial on many levels, but selecting patients may be difficult. Several published antiepileptic drug withdrawal studies show variable rates of success, with relapse rates ranging from 12% to 63%. Several prognostic factors help identify patients who may be amenable to antiepileptic drug withdrawal. The results and limitations described in the antiepileptic drug withdrawal literature, prognostic factors, and general guidelines for antiepileptic drug withdrawal are presented in this article.

Objective: Seizures are a common manifestation of paraneoplastic neurologic syndromes. The objective of this study was to describe the seizure characteristics and outcomes in patients with high‐risk paraneoplastic autoantibodies (>70% cancer association) and to determine factors associated with ongoing seizures. Methods: Patients from 2000 to 2020 with seizures and high‐risk paraneoplastic autoantibodies were retrospectively identified. Factors associated with ongoing seizures at last follow‐up were evaluated. Results: Sixty patients were identified (34 males, median age at presentation = 52 years). ANNA1‐IgG (Hu; n = 24, 39%), Ma2‐IgG (n = 14, 23%), and CRMP5‐IgG (CV2; n = 11, 18%) were the most common underlying antibodies. Seizures were the initial presenting symptom in 26 (43%), and malignancy was present in 38 (63%). Seizures persisted for >1 month in 83%, and 60% had ongoing seizures, with almost all patients (55/60, 92%) still being on antiseizure medications at last follow‐up a median of 25 months after seizure onset. Ongoing seizures at last follow‐up were associated with Ma2‐IgG or ANNA1‐IgG compared to other antibodies (p =.04), highest seizure frequency being at least daily (p =.0002), seizures on electroencephalogram (EEG; p =.03), and imaging evidence of limbic encephalitis (LE; p =.03). Death occurred in 48% throughout the course of follow‐up, with a higher mortality in patients with LE than in those without LE (p =.04). Of 31 surviving patients at last follow‐up, 55% continued to have intermittent seizures. Significance: Seizures in the setting of high‐risk paraneoplastic antibodies are frequently resistant to treatment. Ongoing seizures are associated with ANNA1‐IgG and Ma2‐IgG, high seizure frequency, and EEG and imaging abnormalities. Although a subset of patients may respond to immunotherapy and achieve seizure freedom, poor outcomes are frequently encountered. Death was more common among patients with LE. [ABSTRACT FROM AUTHOR]

Transcranial magnetic stimulation (TMS) is a non-invasive modality of focal brain stimulation in which a fluctuating magnetic field induces electrical currents within the cortex. It remains unclear to what extent TMS alters EEG biomarkers and how EEG biomarkers may guide treatment of focal epilepsy. We present a case of a 48-year-old man with focal epilepsy, refractory to multiple medication trials, who experienced a dramatic reduction in seizures after targeting the area of seizure onset within the left parietal-occipital region with low-frequency repetitive TMS (rTMS). Prior to treatment, he experienced focal seizures that impacted cognition including apraxia at least 50–60 times daily. MRI of the brain showed a large focal cortical dysplasia with contrast enhancement involving the left occipital-parietal junction. Stimulation for 5 consecutive days was well-tolerated and associated with a day-by-day reduction in seizure frequency. In addition, he was monitored with continuous video EEG, which showed continued and progressive changes in spectral power (decreased broadband power and increased infraslow delta activity) and a gradual reduction in seizure frequency and duration. One month after initial treatment, 2-day ambulatory EEG demonstrated seizure-freedom and MRI showed resolution of focal contrast enhancement. He continues to receive 2–3 days of rTMS every 2–4 months. He was seizure-free for 6 months, and at last follow-up of 17 months was experiencing auras approximately every 2 weeks without progression to disabling seizures. This case demonstrates that rTMS can be a well-tolerated and effective means of controlling medication-refractory seizures, and that EEG biomarkers change gradually in a fashion in association with seizure frequency. TMS influences cortical excitability, is a promising non-invasive means of treating focal epilepsy, and has measurable electrophysiologic effects. [ABSTRACT FROM AUTHOR]

Background: Identification of an underlying mitochondrial disorder can be challenging due to the significant phenotypic variability between and within specific disorders. Epilepsy can be a presenting symptom with several mitochondrial disorders. In this study, we evaluated clinical, electrophysiologic, and imaging features in patients with epilepsy and mitochondrial disorders to identify common features, which could aid in earlier identification of a mitochondrial etiology. Methods: This is a retrospective case series from January 2011 to December 2019 at a tertiary referral center of patients with epilepsy and a genetically confirmed diagnosis of a mitochondrial disorder. A total of 164 patients were reviewed with 20 patients fulfilling inclusion criteria. Results: A total of 20 patients (14 females, 6 males) aged 0.5–61 years with epilepsy and genetically confirmed mitochondrial disorders were identified. Status epilepticus occurred in 15 patients, with focal status epilepticus in 13 patients, including 9 patients with visual features. Abnormalities over the posterior cerebral regions were seen in 66% of ictal recordings and 44% of imaging studies. All the patients were on nutraceutical supplementation with no significant change in disease progression seen. At last follow-up, eight patients were deceased and the remainder had moderate-to-severe disability. Discussion: In this series of patients with epilepsy and mitochondrial disorders, we found increased propensity for seizures arising from the posterior cerebral regions. Over time, electroencephalogram (EEG) and imaging abnormalities increasingly occurred over the posterior cerebral regions. Focal seizures and focal status epilepticus with visual symptoms were common. Additional study is needed on nutraceutical supplementation in mitochondrial disorders. [ABSTRACT FROM AUTHOR]

The objective of this study was to describe serological association of musicogenic epilepsy and to evaluate clinical features and outcomes of seropositive cases. Through retrospective chart review, musicogenic epilepsy patients were identified. Among 16 musicogenic epilepsy patients, nine underwent autoantibody evaluations and all had high‐titer glutamic acid decarboxylase 65–immunoglobulin G (GAD65‐IgG; >20 nmol·L–1, serum, normal ≤.02 nmol·L–1, eight women). Median GAD65‐IgG serum titer was 294 nmol·L–1 (20.3–3005 nmol·L–1), and median cerebrospinal fluid titer (n = 4) was 14.7 nmol·L–1. All patients had temporal lobe epilepsy, and bitemporal epileptiform abnormalities were common. Right temporal lobe seizures were most frequently captured when seizures were induced by music on electroencephalogram (3/4; 75%). Intravenous (IV) methylprednisolone and/or IV Ig (IVIG) was utilized in four patients, with one having greater than 50% reduction. Rituximab (n = 2) and mycophenolate (n = 1) were ineffective. Two patients underwent right temporal lobe resections but continued to have seizures. Vagus nerve stimulation was effective at reducing seizures in one patient by 50%, and an additional patient was seizure‐free by avoiding provoking music. Right temporal lobe epilepsy was more common among patients with musicogenic epilepsy when compared to nonmusicogenic GAD65 epilepsies (n = 71, 89% vs. 47%, p =.03). GAD65‐IgG should be tested in patients with musicogenic epilepsy, given implications for management and screening for comorbid autoimmune conditions. [ABSTRACT FROM AUTHOR]

Objective: To assess the frequency and characteristics of interictal and postictal headaches (using International Classification of Headache Disorders, 3rd edition criteria) in a population of patients with epilepsy admitted to the Mayo Clinic Rochester epilepsy monitoring unit and assess their localizing value. Methods: This was a cross‐sectional study. Participants were voluntarily recruited upon admission to the epilepsy monitoring unit. Two separate questionnaires were then administered. The first was to assess the presence and character of headaches experienced in the past 12 months. The second was to assess characteristics of postictal headaches experienced during their admission including localization. Results: One‐hundred and twenty subjects (77%) met inclusion criteria and completed the initial questionnaire. Mean age was 38.1 years (range 18‐82), and 67 (55.8%) were female. Interictal headaches were reported in 97 of 120 (81%) subjects, and these met ICHD3 criteria for migraine in 48 (50%). Postictal headaches were reported by 75 of 120 (63%) subjects on the initial admission questionnaire, representing migraine in 38 (51%). Thirty‐nine (32%) subjects completed the secondary questionnaire related to postictal headaches experienced during admission, of which nine (23%) met criteria for migraine. There was no seizure lateralizing or localizing value noted based on postictal headache localization. Significance: Migraine was frequent in this cohort and appears to be the dominant interictal and postictal headache type in patients with epilepsy. In this study, the first to assess incident postictal headache in the setting of an ictal EEG, headache localization was of no seizure localizing value. Few patients were being actively treated; suggesting headache management is often overlooked in the epilepsy population. [ABSTRACT FROM AUTHOR]

Purpose: Previous studies have established the importance of the insular cortex and temporal lobe in cardiovascular autonomic modulation. Some investigators, based on the results of cortical stimulation response, functional imaging, EEG recordings of seizures, and lesional studies, have suggested that cardiac sympathetic and parasympathetic function may be lateralized, with sympathetic representation lateralized to the right insula, and parasympathetic, to the left. These studies have suggested that ictal bradycardia is most commonly a manifestation of activation of the left temporal and insular cortex. However, the evidence for this is inconsistent. We sought to assess critically the predictable value of ictal bradycardia for seizure localization and lateralization. Methods: In this study, we reviewed the localization of seizure activity in 13 consecutive patients with ictal bradycardia diagnosed during prolonged video-EEG monitoring at Mayo Clinic Rochester. The localization of electrographic seizure activity at seizure onset and bradycardia onset was identified in all patients. In addition, we performed a comprehensive review of the ictal bradycardia literature focusing on localization of seizure activity in ictal bradycardia cases. Results: All occurrences of ictal bradycardia in the 13 identified patients were associated with temporal lobe–onset seizures. However, no consistent lateralization of seizure activity was found at onset of seizure activity or at onset of bradycardia in this population. Seizure activity was bilateral at bradycardia onset in nine of 13 patients. The results from the literature review also showed that a predominance of patients had bilateral activity at bradycardia onset; however, more of the ictal bradycardia cases from the literature had left hemispheric localization of seizure onset. Conclusions: Ictal bradycardia most often occurs in association with bilateral hemispheric seizure activity and is not a consistent lateralizing sign in localizing seizure onset. Our data do not support the existence of a strictly unilateral parasympathetic cardiomotor representation in the left hemisphere, as has been suggested. [ABSTRACT FROM AUTHOR]

Although pathophysiologically distinct, syncope and seizures share clinical characteristics which may make diagnosis difficult. Adding to diagnostic complexity are the facts that seizures and syncope may coexist in the same patient, syncope may be associated with seizure-like motor manifestations, and seizures may be complicated by cardiac arrhythmia and syncope. Combined EEG/ECG telemetry is sometimes necessary to establish the correct diagnosis. These techniques also provide an opportunity to study the role of certain cortical regions in the modulation of cardiac function. There is an increasing understanding of the central autonomic pathways involved in the genesis of the cardiovascular changes that occur during epileptic seizures. This article reviews the use of EEG/ECG telemetry in the evaluation of syncope and seizures, and the neuroanatomic circuitry involved in the production of the cardiovascular manifestations of seizures. [ABSTRACT FROM AUTHOR]

Syncope and seizures are common conditions and pose diagnostic challenges for the clinician. The cumulative lifetime incidence of epilepsy is 3%, and the incidence of a first attack of syncope is 6.2 per 1000 person-years in the general population. Although the underlying pathophysiological processes are distinct, seizures and syncope share some clinical characteristics which may lead to diagnostic confusion. Confounding matters further is the fact that seizures and syncope may coexist in a given patient. For example, some conditions such as prolonged QT syndrome may result in both arrhythmia-associated syncope and seizures, and in some cases, seizures may result in cardiac arrhythmia and syncope. The clinical history, examination, electrocardiography, and combined EEG/ECG telemetry are all helpful in the diagnosis of seizures and syncope. [ABSTRACT FROM AUTHOR]

: IntroductionTransient neuroimaging findings associated with seizure activity have received relatively little attention in the neurosurgical literature. These abnormalities may mimick neoplastic or ischemic changes on magnetic resonance imaging (MRI), possibly leading to additional studies and surgical treatment.: Case descriptionA 17-year-old right-handed male was transferred to emergency room in status epilepticus. A MRI obtained 5 months before admission was negative. On the day of admission, he had multiple intermittent upper-left extremity partial seizures and prolonged secondary generalized seizures. An electroencephalogram (EEG) showed frequent epileptiform discharges over the right hemisphere posteriorly. A MRI study performed 2 days after admission revealed non-hemorrhagic abnormalities involving the right occipital region that were hyperintense on fluid-attenuated inversion recovery (FLAIR) and T2 weighted sequences. The apparent diffusion coefficient map was unremarkable. Follow-up MRIs, 3 and 11 months after admission, showed complete resolution of these lesions.: ConclusionImaging findings after status epilepticus may raise suspicion of ischemic or neoplastic lesions. These findings may be reversible. Further follow-up imaging may prevent unnecessary intervention. [Copyright &y& Elsevier]

• Hemorrhage was found in 11% cases regardless of clinical symptoms and should be recognized to avoid mis-localization of seizure onset zones. • sEEG recordings in close proximity of hemorrhage may have electrographic correlates to identify hemorrhage during monitoring. • Despite the occurrence of hemorrhage, when surgical treatments were pursued, a vast majority of patients had favorable outcomes. Intracranial hemorrhage (ICH) is a known complication during stereo-electroencephalography (sEEG) however true rates remain unknown. We provide a comprehensive review of ICH during sEEG regardless of clinical symptoms. Secondly, we analyzed sEEG recordings to identify electrographic correlates of ICH. This is a retrospective study of patients undergoing sEEG between January 2016 and April 2022 at the Mayo Clinic in Rochester. We reviewed medical records and imaging studies to identify ICH. We analyzed ICH by type, electrode trajectories, timing, sEEG findings and outcomes. There were a total of 201 sEEG implants, of which 23 (11%) cases or 0.9% electrodes implanted had evidence of ICH. The majority of affected patients (82%) were either asymptomatic or had mild clinical neurological manifestations. In 90% of patients who proceeded with surgical treatments, outcomes were favorable. The most common sEEG finding in contacts in proximity of ICH was either focal slowing with interictal discharges or focal electrographic seizures. ICH associated with sEEG is likely under-reported in literature. We present electroencephalographic correlates of ICH that may aid identification of ICH in the course of performing sEEG monitoring. Our data provides clinically relevant information on potential risks and outcomes of ICH. Furthermore, our findings aid identification of ICH during sEEG. [ABSTRACT FROM AUTHOR]

Summary: This study aimed to determine the reliability of clinical history and seizure semiology for distinguishing between frontal lobe seizures (FLS) and temporal lobe seizures (TLS). FLS patients (n =23) were consecutively identified through an epilepsy surgery database. TLS patients (n =27) were selected randomly from 238 patients who had undergone temporal lobe surgery for epilepsy. The criterion standard for seizure localization was the location of resective epilepsy surgery that controlled seizures for a minimum of 2 years. Blinded comparisons of 13 historical information items (HII) and 19 video-recorded semiologic features (VSF) were made. We identified 3 HII (sex, history of febrile convulsions, and history of generalized tonic–clonic seizures) and 2 VSF (fencing posturing and postictal confusion) that significantly distinguished between FLS and TLS. The multivariate analysis model correctly identified 87% of FLS patients and 74% of TLS patients. No single HII or VSF is sufficient for distinguishing between FLS and TLS. A model integrating multiple HII and VSF may assist in this differentiation, but some patients still may be misclassified. [Copyright &y& Elsevier]

• This novel PEV trajectory allows good ANT coverage. • This novel PEV trajectory appears to have minimal complications. • This novel PEV trajectory allows relatively more electrodes in the ANT than those used in the MORE trial. Deep brain stimulation (DBS) of the anterior nucleus of the thalamus (ANT) is a promising treatment for refractory epilepsy; however, it remains challenging to successfully target the ANT. The results of Medtronic Registry for Epilepsy (MORE) supported a frontal transventricular(TV) compared to frontal extraventricular (EV) lead trajectory for ANT DBS may have better coverage of the ANT. Here we report the safety and targeting efficacy of a novel, posterior parietal extraventricular (PEV) approach to the ANT. We conducted a retrospective analysis of ten patients who underwent bilateral ANT DBS (20 total trajectories) for medically-refractory epilepsy. Similar targeting methodology as the MORE trial was used, and the DBS Intrinsic Template Atlas (DISTAL) was utilized for ANT localization and contact position relative to ANT. Clinical data were assessed for DBS targeting efficacy and surgical complications. The demonstrated PEV trajectory showed a successful ANT targeting rate of 90% bilaterally. Two or more contacts within ANT were presented in 75% of all leads. Mean contact number in ANT was 2.2 + 1.2. There were no intracranial hemorrhages, cerebrospinal fluid leakage, or permanent neurologic deficits. In this small series, the novel PEV for ANT DBS is feasible with good targeting accuracy and potential safety advantages. The high accuracy of the PEV trajectory suggests that it is a reasonable alternative trajectory for ANT DBS. Larger studies will be needed to assess this trajectory on clinical outcome of DBS treatment to epilepsy. [ABSTRACT FROM AUTHOR]

Background: Jeavons syndrome is an underreported epileptic syndrome characterized by eyelid myoclonia, eyelid closure-induced seizures or electroencephalography paroxysms, and photosensitivity. Drug-resistant epilepsy is common, but the prognostic factors and clinical course leading to drug resistance have not been well characterized.Methods: We identified 30 patients who met the diagnostic criteria of Jeavons syndrome at a single institution between January 1, 2000 and December 15, 2016. Criteria for Jeavons syndrome included all of the following: (1) eyelid myoclonia with or without absences, (2) eye-closure-induced seizures or electroencephalography paroxysms, and (3) seizure onset after 12 months of age. We reviewed and described the epilepsy history, antiepileptic drug trials, and response to treatments.Results: Mean age at seizure onset was 7.3 years, and 80% were female. Absence seizures (63%) and generalized tonic-clonic seizures (23%) were most common at onset. Diagnosis was delayed by an average of 9.6 years. After a median follow-up of two years, 80% of patients had drug resistant epilepsy and 70% experienced generalized tonic-clonic seizures. Generalized tonic-clonic seizures and seizure types other than absence seizures increased the risk of drug-resistant epilepsy (P values 0.049 and 0.03, respectively). Valproic acid, lamotrigine, ethosuximide, and levetiracetam were the most effective in reducing seizures by more than 50%.Conclusions: The diagnosis of Jeavons syndrome is often delayed. Generalized tonic-clonic seizures and seizure types other than absence seizures may be predictors of drug-resistant epilepsy among patients with Jeavons syndrome. [ABSTRACT FROM AUTHOR]

The objective of this study was to describe the sEEG-defined seizure onset zone (SOZ), seizure semiology, presurgical evaluations, surgical intervention and outcome in patients with midline onset noninvasive phase I monitoring. A single center sEEG database was reviewed to identify patients with seizures onset predominantly involving midline electrodes (FZ, CZ, PZ, OZ) on scalp EEG. Data abstracted included clinical factors, seizure semiology graded into lobar segmentation, imaging and electrographic findings, sEEG plan, interventions, and outcome. Twelve patients were identified (8 males, median age of sEEG 28 years) out of 100 cases of sEEG performed from January 2015-September 2019. "Frontal lobe" seizure semiology was the most common. sEEG-defined SOZ were frontal (5), diffuse (1), multifocal (1), frontal and insular (1), frontal and cingulate (1), insular (1), cingulate (1), and mesial temporal (1). CZ and/or FZ scalp EEG changes were present for all patients with SOZ involving the frontal, cingulate, and insular regions. PZ/OZ scalp involvement was present in one patient with mesial temporal SOZ. Four patients underwent a definitive resective or ablative surgery, and the remaining patients underwent a palliative intervention. Of those with follow-up information available, 8/11 had seizure reduction by ≥ 50%, including 4 with an Engel I outcome. No clinical factors were associated with outcome. SOZ for midline onset seizures from noninvasive phase I monitoring was most commonly in the frontal, cingulate, and insular regions. A complex cortical network between these regions may explain overlap in semiology and scalp EEG findings. While the number rendered seizure-free was limited, a significant proportion experienced a reasonably favorable outcome justifying use of sEEG to identify surgical options in these patients. • Midline onset seizures on scalp EEG are rare. • sEEG commonly shows frontal, insular, and/or cingulate onset for these seizures. • A complex seizure network is likely underlying this. [ABSTRACT FROM AUTHOR]

Highlights: [•] PGES is not rare in children; it followed 16.1% of seizures in 32.4% of children. [•] Only primary/secondarily generalized tonic clonic seizures were marked by PGES. [•] PGES was significantly associated with peri-ictal tachycardia and hypoxemia. [•] Children with PGES had higher SUDEP-7 Inventory scores than those without. [Copyright &y& Elsevier]