Focal Impaired Awareness Seizures Research Articles

Juxtacortical lesions are associated with seizures in cerebral small vessel disease.

Small vessel cerebrovascular disease (SVCD) can manifest with epileptic seizures and transient ischemic attacks (TIA). This study was designed to test if the extent and spatial distribution of SVCD differs in patients with focal impaired awareness seizures (FIAS) from patients with TIA. This is a retrospective single-center case-control study of elderly patients at a high cardiovascular risk. 118 patients with FIAS (cases) were compared to a matched control group of 118 patients with TIA. The extent and spatial distribution of white matter hyperintensities (WMH) characteristic for SVCD and medial temporal lobe atrophy were analyzed on magnetic resonance imaging (MRI) obtained at admission. The Fazekas, Wahlund, and Scheltens scales were used for grading. Juxtacortical small lesions were analyzed separately. FIAS patients were observed to have more extensive WMH (p < 0.001) and more pronounced medial temporal lobe atrophy (p < 0.001) than TIA patients. WMH in FIAS patients were predominantly localized in supratentorial white matter compared to TIA patients (p < 0.001). Juxtacortical hyperintensities were far more common in FIAS patients than in TIA patients (80.5% vs. 22.0%; p < 0.001). Multivariate analysis revealed juxtacortical small lesions as strong independent predictor (OR, 95% CI 12.8, 6.7-24.3) and medial temporal lobe atrophy as further independent predictor of FIAS (3.1, 1.3-7.1). Juxtacortical small lesions and to a smaller extent medial temporal lobe atrophy are associated with epileptic seizures in elderly patients at a high cardiovascular risk. This observation may provide a structural explanation for epilepsy in SVCD. Juxtacortical small lesions in SVCD should be considered a structural cause for epilepsy and promote anticonvulsive therapy after a first seizure.

Depression in people with epilepsy in West China: Status, risk factors and treatment gap

PurposeTo evaluate the prevalence, suicidality and treatment gap (TG) of depression in people with epilepsy (PWE) in West China, and to explore the related risk factors. MethodsA consecutive cohort of PWE from West China Hospital was recruited. The Chinese version of the Neurological Disorders Depression Inventory for Epilepsy scale was used to assess depression and suicidality. Prevalence and TG of depression were calculated. Logistic regression analysis was used to assess risk factors of depression and suicidality. ResultsAmong a total of 461 participants, there were 29.9% with depression and 15.8% had suicidality. Being female, an unmarried status, disease course, seizure frequency, seizure occurrence in the last 6 months, and focal impaired awareness seizure were risk factors for depression. Depression was an independent risk factor for suicidality. The TG of depression was 72.5%. The TG was larger in the 30–40 year age group, in rural populations, and among those with a disease course between 5 and 10 years. The TG figures dropped with increasing annual family income. ConclusionsThe prevalence of depression and the rate of suicide risk for PWE in West China are high. A large TG for depression exists in PWE. Clinicians should focus on depression and suicidality for PWE and identify the related risk factors in a timely manner so as to reduce the incidence of depression and suicide. Management including public education as well as professional training of neurologists are necessary to fill the TG.

Alterations in Brain Metabolites in Patients with Epilepsy with Impaired Consciousness: A Case-Control Study of Interictal Multivoxel 1H-MRS Findings.

Previous studies have shown perfusion abnormalities in the thalamus and upper brain stem in patients with epilepsy with impaired consciousness. We hypothesized that these areas associated with consciousness will also show metabolic abnormalities. However, metabolic abnormalities in those areas correlated with consciousness has not been characterized with multiple-voxel 1H-MRS. In this study, we investigated the metabolic alterations in these brain regions and assessed the correlation between seizure features and metabolic alterations. Fifty-seven patients with epilepsy and 24 control subjects underwent routine MR imaging and 3D multiple-voxel 1H-MRS. Patients were divided into 3 subgroups: focal impaired awareness seizures (n = 18), primary generalized tonic-clonic seizures (n = 19), and secondary generalized tonic-clonic seizures (n = 20). The measured metabolite alterations in NAA/Cr, NAA/(Cr + Cho), and Cho/Cr ratios in brain regions associated with the consciousness network were compared between the patient and control groups. ROIs were placed in the bilateral inferior frontal gyrus, supramarginal gyrus, cingulate gyrus, precuneus, thalamus, and upper brain stem. Correlations between clinical parameters (epilepsy duration and seizure frequency) and metabolite alterations were analyzed. Significantly lower NAA/Cr and NAA/(Cho + Cr) ratios (P < .05 and < .01, respectively) were observed in the bilateral thalamus and upper brain stem in all experimental groups, and significantly high Cho/Cr ratios (P < .05) were observed in the right thalamus in the focal impaired awareness seizures group. There were no significant differences in metabolite ratios among the 3 patient groups (P > .05). The secondary generalized tonic-clonic seizures group showed a negative correlation between the duration of epilepsy and the NAA/(Cr + Cho) ratio in the bilateral thalamus (P < .05). Metabolic alterations were observed in the brain stem and thalamus in patients with epilepsy with impaired consciousness.

Localization value of ictal turning prone

PurposeIctal semiology complements ictal EEG in identifying the likely epileptogenic zone. Ictal turning prone (ITP) with body turning of 90 ° or more can be seen with frontal lobe epilepsies. The aim of our study was to evaluate the localizing value of ITP in a general population of patients undergoing long term video-EEG monitoring. MethodsWe reviewed our epilepsy monitoring unit database for adult patients with recorded habitual seizures with ITP. All 16 patients identified had continuous video-EEG monitoring using standard scalp electrodes; eight patients also had intracranial EEG monitoring. We only included focal seizures without evolution to bilateral tonic-clonic activity. ResultsWe identified 16 patients with ITP, mean age of 32.5 years (range 18–50). ITP was consistently seen in at least one focal impaired awareness seizure of all patients. Ictal onset zone on scalp EEG was left temporal in five, right temporal in three, left frontal convexity in two, right frontal convexity in two, probable right medial frontal in three and probable left medial frontal in one patient. Direction of ITP was uni-directional in 12 patients while 4 patients had ITP in opposite direction in different seizures.Nine patients underwent epilepsy surgery; five patients had Engel class I outcome and four patients had Engel class III outcome. ConclusionsIctal turning prone does not have a consistent single localizing or lateralizing value and can be seen with various epileptogenic zones including medial frontal, lateral frontal or temporal. ITP direction can vary even with a single epileptogenic zone.

Frequency and Stratification of Epileptogenic Lesions in Elderly With New Onset Seizures.

Objective: To evaluate prospectively the frequency of epileptogenic lesions in a consecutive cohort of elderly patients presenting with new onset unprovoked seizures, and who underwent a complete evaluation including dedicated epilepsy protocol MRI.Methods and materials: We included all consecutive patients 60 years or older who participated in a prospective study on new onset epilepsy. The work-up included the acquisition of a dedicated epilepsy protocol MRI and a 3 h video/EEG recording. We evaluated the frequency and types of epileptogenic lesions in the whole cohort and stratified those variables by age, gender, types and number of seizures at presentation. We also correlated the EEG findings with the clinical characteristics and neuroimaging results.Results: Of the 101 patients enrolled in the study and who underwent an epilepsy protocol MRI, an epileptogenic lesion was identified in 67% of cases. The most common etiologies were vascular events, followed by tumoral causes and traumatic brain injuries. Epileptogenic lesions were more likely to be identified in patients who presented with only focal aware and impaired awareness seizures. In addition, patients with tumoral epilepsy were significantly more likely to only experience those seizure types compared to patients with other pathological substrates. Interictal/ictal discharges were detected in the EEG of 21% of patients. Epileptiform discharges were significantly more frequent in patients with an epileptogenic lesion on brain MRI, especially in those with a brain tumor.Conclusions: Our results stress the importance of obtaining a dedicated epilepsy protocol MRI in elderly patients with new onset seizures. An epileptogenic lesion will be identified in approximately two thirds of patients with important implications regarding initiation of treatment. In addition, the data underscore the value of distinguishing the types of seizures experienced at presentation as this will apprise the treating physician on the likelihood of identifying an epileptogenic lesion and on the probable etiologies.

Clinical and electrographic characteristics of seizures in LGI1-antibody encephalitis

PurposeThe purpose of this study was to analyze the clinical and electrographic characteristics of seizures in LGI1-antibody encephalitis. MethodsThe methods utilized in this study were prospective analysis of the clinical manifestations, types of seizures, electroencephalogram (EEG), adjuvant examination, treatment and prognosis of 19 cases of LGI1-antibody encephalitis diagnosed from January 2017 to February 2018 in First Affiliated Hospital of Zhengzhou University, and reviewed related literatures. ResultsThe 15/19 (79%) patients were male, and the average onset age was 58 years (23–82). The following cases were observed: 17 (89%) with epilepsy seizures, 14 (73%) with mental disorders, and 13 (68%) with cognitive impairment. Types of epilepsy were including focal aware seizures, focal-impaired awareness seizures, focal to bilateral tonic–clonic seizures, and status epilepticus. The motor events were most commonly clonus or automatisms, and the sensory events were frequently body shuddering. The 13 patients had faciobrachial dystonic seizures (FBDS); the median frequency was 48 per day (range 5–180). In some video-EEGs, multifocal ictal epileptiform discharges from frontal, temporal, and apical regions, and interictal slow wave activity were observed in patients. Normal EEG appeared in all patients during FBDS. Five patients had hyponatremia, and brain magnetic resonance imaging (MRI) results of 5 cases were abnormal. All patients were treated with antiepileptic drugs and immunotherapy, and their clinical symptoms were improved. During the follow-up period, 13 patients recovered basically, and 6 patients relapsed. One patient died of status epilepticus after relapse. ConclusionsFaciobrachial dystonic seizure and various types of epileptic seizures are characteristic manifestations of LGI1-antibody encephalitis, which can assist in early diagnosis. Once this has been diagnosed, antiepileptic drugs and immunotherapy should be given as soon as possible to the patient.

Short duration of focal onset awareness and impaired awareness seizure are characteristic features of epilepsy in elderly patients.

PurposeDiagnosing epilepsy in the elderly population can be difficult due to mimicking symptoms. Furthermore, epileptic symptoms can also be masked by various symptoms. We hypothesized that elderly patients with epilepsy exhibit specific clinical features among the various symptoms.Patients and methodsFrom 2009 to 2017, 177 patients who were older than 65 years were referred to our epilepsy center. Out of this group, the onset of symptoms occurred after reaching the age of 50 years in 152 of the patients, who were additionally being treated at our clinic. We divided their symptoms in accordance with their consciousness levels, which were defined as follows: full wakefulness level I, impaired awareness level II, and loss of consciousness level III. We also classified the duration of the symptoms as <10 seconds, ≥10 seconds but <1 minute, ≥1 minute but <5 minutes, ≥5 minutes but <10 minutes, ≥10 minutes but <1 hour, and ≥1 hour.ResultsAmong the 152 patients analyzed (mean age 72.9 years, standard deviation 6.71, range 65–92), 84 patients had epilepsy (epilepsy group) while 68 did not exhibit epilepsy (nonepilepsy group). For the consciousness levels, there were more level I patients in the nonepilepsy vs epilepsy group (P<0.028), with symptom duration lasting <1 minute (sensitivity 0.857, specificity 1) in the epilepsy group. In contrast, there were more level II patients in the epilepsy vs the nonepilepsy group (P=0.015), with the duration of symptoms lasting <1 minute (sensitivity 0.8125, specificity 0.930) in the epilepsy group. For the level III consciousness, convulsions were more commonly seen in the epilepsy group (P=0.044).ConclusionSymptoms that last <1 minute with awareness and impaired awareness might be epileptic in the elderly population.

Lacosamide in children with drug-resistant epilepsy.

To assess the effectiveness and tolerability of lacosamide in paediatric clinical practice. A search of our hospital's pharmacy database yielded all children <16 years old dispensed lacosamide for drug-resistant epilepsy between January 2011 and June 2016. Medical records were reviewed for clinical and drug details. Continued treatment for ≥12 months was considered an indicator of effectiveness and tolerability. A total of 107 children (61 boys) satisfied inclusion criteria. Median age at lacosamide commencement was 9.9 years (interquartile range 6.7-13.7). Of those children, 57 (53%) children had focal epilepsy, with focal motor or impaired awareness seizures most commonly reported; 50 (47%) children had generalised epilepsy, most with tonic-clonic seizures, tonic seizures or epileptic spasms; 83 (78%) children had an intellectual disability, 24 (22%) had a physical disability and 22 (21%) had an autism spectrum disorder; 69 (65%) children continued lacosamide for ≥12 months. Reasons for discontinuation before 12 months in 38 (35%) children included ineffectiveness in 25 (66%), adverse events in 7 (18%) and worsening of seizures in 2 (5%). The most common adverse events were drowsiness, behavioural changes, unsteadiness, nausea and vomiting. Epilepsy type and comorbidities were not associated with continuation or reasons for discontinuation. Most children continued treatment with lacosamide, suggesting effectiveness and tolerability. Lacosamide may prove to be a useful, 'broad-spectrum' antiepileptic medication in children for focal and generalised epilepsies and in association with comorbidities.

The Revised Definition and Classification of Epilepsy for Neurodiagnostic Technologists

ABSTRACTThe definition of who has epilepsy, classification of seizure types, and types of epilepsy have all recently been revised. The classical definition of epilepsy as a person having two or more unprovoked seizures more than 24 hours apart has been expanded also to include those with one seizure and a high likelihood (more than 60%) of having another. In the new definition, epilepsy is considered to be resolved when a person is seizure-free for 10 years, the terminal 5 being off seizure medicines, or when an age-dependent syndrome has been outgrown. The new seizure type classification revises the 1981 system but maintains the primary distinction of focal- versus generalized-onset seizures. Seizures also can be of unknown onset. Focal seizures may demonstrate retention or impairment of awareness, resulting in focal-aware or focal-impaired awareness seizures. Several new focal and generalized seizure types are introduced. Classification of the epilepsies is now by grouping of seizure types, etiologies, comorbidities, and epilepsy syndromes. The goal of the new terminology is greater clarity of communication and more accurate grouping of seizure types for research. Neurodiagnostic technologists can be of great help in observing clinical and electrographic features that will define the type of seizure.

An Interesting Eating Epilepsy Case Induced by Bread Eating

Eating epilepsy is evaluated in reflex epilepsies (RE). Different genetic, ethnic, acquired factors and to the bulky meals rich in carbohydrates consumed may play a role in etiology of RE or supported by a brain lesion. In this situation, seizures provoked by eating is a rare entity and the ictal semiology differs from patient to patient. Focal impaired awareness seizure is most commonly described. Diffuse cerebral damage is often noted on MRI. Reproduction of these seizures during EEG recording is often difficult as the stimulus is frequently complex, involving different components of eating, such as the sight of food, proprioceptive, olfactive or gustative stimulations, chewing, salivation, and gastric distension of eating. The case presented here was an eating epilepsy case triggered by bread eating and implicated, based on EEG and MRI.

Routine replacement of a vagal nerve stimulator generator leading to asystole

A 52-year-old female with a longstanding history of drug-resistant epilepsy that included focal impaired awareness seizure presented at end of service of her vagus nerve stimulator (VNS) generator. She had undergone a generator replacement in 2010 without complication. However, her latest replacement was accompanied by multiple bouts of asystole. We discuss the case, possible causes of the asystole, and its relevance to the future of VNS generator replacement and epilepsy treatment.

Seizure outcome after hippocampal deep brain stimulation in patients with refractory temporal lobe epilepsy: A prospective, controlled, randomized, double-blind study.

We designed a prospective, randomized, controlled, double-blind study to evaluate the efficacy of hippocampal deep brain stimulation (Hip-DBS) in patients with refractory temporary lobe epilepsy (TLE). Sixteen adult patients with refractory TLE were studied. Patient's workup included medical history, interictal and ictal electroencephalography (EEG), and high-resolution 1.5T magnetic resonance imaging (MRI). Patients were randomized on a 1:1 proportion to an active (stimulation on) or to a control (no stimulation) arm. After implantation, patients were allowed to recover for 1 month, which was followed by a 1-month titration (or sham) period. The 6-month blinded phase started immediately afterward. A postoperative MRI confirmed the electrode's position in all patients. All patients received bipolar continuous stimulation. Stimulus duration was 300 μs and frequency was 130 Hz; final intensity was 2 V. Patients were considered responders when they had at least 50% seizure frequency reduction. All patients had focal impaired awareness seizures (FIAS, complex partial seizures), and 87% had focal aware seizures (FAS, simple partial seizures). Mean preoperative seizure frequency was 12.5 ± 9.4 (mean ± standard deviation) per month. MRI findings were normal in two patients, disclosed bilateral mesial temporal sclerosis (MTS) in three, left MTS in five, and right MTS in six patients. An insertional effect could be noted in both control and active patients. In the active group (n = 8), four patients became seizure-free; seven of eight were considered responders and one was a nonresponder. There was a significant difference regarding FIAS frequency between the two groups from the first month of full stimulation (p < 0.001) until the end of the blinded phase (p < 0.001). This was also true for FAS, except for the third month of the blinded phase. Hip-DBS was effective in significantly reducing seizure frequency in patients with refractory TLE in the active group, as compared to the control group. Fifty-percent of the patients in the active group became seizure-free. The present study is the larger prospective, controlled, double-blind study to evaluate the effects of Hip-DBS published to date.

The epileptology of Koolen‐de Vries syndrome: Electro‐clinico‐radiologic findings in 31 patients

This study was designed to describe the spectrum of epilepsy phenotypes in Koolen-de Vries syndrome (KdVS), a genetic syndrome involving dysmorphic features, intellectual disability, hypotonia, and congenital malformations, that occurs secondary to 17q21.31 microdeletions and heterozygous mutations in KANSL1. We were invited to attend a large gathering of individuals with KdVS and their families. While there, we recruited individuals with KdVS and seizures, and performed thorough phenotyping. Additional subjects were included who approached us after the family support group brought attention to our research via social media. Inclusion criteria were genetic testing results demonstrating 17q21.31 deletion or KANSL1 mutation, and at least one seizure. Thirty-one individuals were studied, aged 2-35 years. Median age at seizure onset was 3.5 years, and 9 of 22 had refractory seizures 2 years after onset. Focal impaired awareness seizures were the most frequent seizure type occurring in 20 of 31, usually with prominent autonomic features. Twenty-one patients had prolonged seizures and, at times, refractory status epilepticus. Electroencephalography (EEG) showed focal/multifocal epileptiform discharges in 20 of 26. MRI studies of 13 patients were reviewed, and all had structural anomalies. Corpus callosum dysgenesis, abnormal hippocampi, and dilated ventricles were the most common, although periventricular nodular heterotopia, focal cortical dysplasia, abnormal sulcation, and brainstem and cerebellum abnormalities were also observed. One patient underwent epilepsy surgery for a lesion that proved to be an angiocentric glioma. The typical epilepsy phenotype of KdVS involves childhood-onset focal seizures that are prolonged and have prominent autonomic features. Multifocal epileptiform discharges are the typical EEG pattern. Structural brain abnormalities may be universal, including signs of abnormal neuroblast migration and abnormal axonal guidance. Epilepsy surgery should be undertaken with care given the widespread neuroanatomic abnormalities; however, tumors are a rare, yet important, occurrence.

Instruction manual for the ILAE 2017 operational classification of seizure types.

This companion paper to the introduction of the International League Against Epilepsy (ILAE) 2017 classification of seizure types provides guidance on how to employ the classification. Illustration of the classification is enacted by tables, a glossary of relevant terms, mapping of old to new terms, suggested abbreviations, and examples. Basic and extended versions of the classification are available, depending on the desired degree of detail. Key signs and symptoms of seizures (semiology) are used as a basis for categories of seizures that are focal or generalized from onset or with unknown onset. Any focal seizure can further be optionally characterized by whether awareness is retained or impaired. Impaired awareness during any segment of the seizure renders it a focal impaired awareness seizure. Focal seizures are further optionally characterized by motor onset signs and symptoms: atonic, automatisms, clonic, epileptic spasms, or hyperkinetic, myoclonic, or tonic activity. Nonmotor-onset seizures can manifest as autonomic, behavior arrest, cognitive, emotional, or sensory dysfunction. The earliest prominent manifestation defines the seizure type, which might then progress to other signs and symptoms. Focal seizures can become bilateral tonic-clonic. Generalized seizures engage bilateral networks from onset. Generalized motor seizure characteristics comprise atonic, clonic, epileptic spasms, myoclonic, myoclonic-atonic, myoclonic-tonic-clonic, tonic, or tonic-clonic. Nonmotor (absence) seizures are typical or atypical, or seizures that present prominent myoclonic activity or eyelid myoclonia. Seizures of unknown onset may have features that can still be classified as motor, nonmotor, tonic-clonic, epileptic spasms, or behavior arrest. This "users' manual" for the ILAE 2017 seizure classification will assist the adoption of the new system.

ILAE CLASSIFICATION OF THE EPILEPSIES: THE 2017 REVISION AND UPDATE

The International League Against Epilepsy (ILAE) has developed and approved the Operational classification of seizure types 2017 and the Classification of the Epilepsies 2017 . According to the ILAE 2017 Operational classification of seizure types, seizures can be initially divided into focal or generalized; and the seizure onset is of crucial importance in this case. Focal seizures are optionally subdivided into focal aware seizures and focal impaired awareness seizures. Several new types of generalized-onset seizures were implemented. Specific motor and non-motor classifiers may be added. Unknown onset seizures are placed separately. Three levels of diagnostics are specified in the 2017 ILAE Classification of the Epilepsies: seizure type (defined in accordance with the 2017 ILAE Operational classification of seizure types), epilepsy type (focal, generalized, combined generalized and focal, unknown), and epilepsy syndrome. An etiologic diagnosis should be considered at each step along the diagnostic pathway. A patient ’s epilepsy may be classified into more than one etiological category. The term “benign” is replaced by the terms “self-limited” and “pharmacoresponsive” to be used where appropriate. The term “developmental and epileptic encephalopathy” can be applied in whole or in part where appropriate. The 2017 Operational classification of seizure types and the 2017 ILAE Classification of the Epilepsies can be helpful both in routine clinical practice and scientific research that will assist in improving epilepsy care and quality of life of patients with epilepsies.

Practical Applications of Dynamic Choice and Optimal Annuitization

<h3>Abstract</h3> Loss of consciousness (LOC) is a hallmark of many epileptic seizures and carries risks of serious injury and sudden death. While cortical sleep-like activities accompany LOC during focal impaired awareness (FIA) seizures, the mechanisms of LOC during focal to bilateral tonic-clonic (FBTC) seizures remain unclear. Quantifying differences in markers of cortical activation and ictal recruitment between FIA and FBTC seizures may also help to understand their different consequences for clinical outcomes and to optimize neuromodulation therapies. We quantified clinical signs of LOC and intracranial EEG (iEEG) activity during 129 FIA and 50 FBTC from 41 patients. We characterized iEEG changes both in the seizure onset zone (SOZ) and in areas remote from SOZ with a total of 3386 electrodes distributed across brain areas. First, we compared the dynamics of iEEG sleep-like activities: slow-wave activity (SWA; 1-4 Hz) and beta/delta ratio (B/D; a validated marker of cortical activation) during FIA vs. FBTC. Second, we quantified differences between FBTC and FIA for a marker validated to detect ictal cross-frequency coupling: phase-locked high-gamma (PLHG; high gamma phased locked to low frequencies) and a marker of ictal recruitment: the epileptogenicity index (i.e. the number of channels crossing an energy ratio threshold for high vs. low frequency power). Third, we assessed changes in iEEG activity preceding and accompanying behavioral generalization onset and their correlation with electromyogram (EMG) channels. In addition, we analyzed human cortical multi-unit activity recorded with Utah arrays during three FBTC. Compared to FIA, FBTC seizures were characterized by deeper LOC and by stronger increases in SWA in parieto-occipital cortex. FBTC also displayed more widespread increases in cortical activation (B/D), ictal cross-frequency coupling (PLHG) and ictal recruitment (epileptogenicity index). Even before generalization, FBTC displayed deeper LOC; this early LOC was accompanied by a paradoxical increase in B/D in fronto-parietal cortex. Behavioral generalization coincided with complete loss of responsiveness and a subsequent increase in high-gamma in the whole brain, which was especially synchronous in deep sources and could not be explained by EMG. Similarly, multi-unit activity analysis of FBTC revealed sustained increases in cortical firing rates during and after generalization onset in areas remote from the SOZ. Unlike during FIA, LOC during FBTC is characterized by a paradoxical increase in cortical activation and neuronal firing. These findings suggest differences in the mechanisms of ictal LOC between FIA and FBTC and may account for the more negative prognostic consequences of FBTC.