scholarly journals Epileptic Encephalopathies with Status Epilepticus during Sleep: New Techniques for Understanding Pathophysiology and Therapeutic Options

2012 ◽  
Vol 2012 ◽  
pp. 1-6 ◽  
Author(s):  
Daniela Brazzo ◽  
Maria Carmela Pera ◽  
Marco Fasce ◽  
Grazia Papalia ◽  
Umberto Balottin ◽  
...  
Keyword(s):  
Status Epilepticus ◽  
Treatment Guidelines ◽  
Task Force ◽  
Epileptiform Activity ◽  
Clinical Manifestations ◽  
Epileptic Encephalopathy ◽  
Computer Assisted ◽  
Paroxysmal Activity ◽  
New Techniques ◽  
Eeg Abnormalities

Encephalopathy with status epilepticus during sleep (ESES) is an epileptic encephalopathy, as defined by the International League Against Epilepsy (ILAE) Task Force on Classification and Terminology, that is, a condition in which the epileptic processes themselves are believed to contribute to the disturbance in cerebral function. Clinical manifestations of ESES are heterogeneous: apart from different seizure types, they consist in combinations of cognitive, motor, and behavioural disturbances associated with a peculiar electroencephalographic pattern of paroxysmal activity significantly activated during slow sleep, which culminates in a picture of continuous spikes and waves during sleep (CSWS). The pathophysiological mechanisms underlying this condition are still incompletely understood. Establishing a clear-cut correlation between EEG abnormalities and clinical data, though interesting, is very complex. Computer-assisted EEG analyses especially if combined with functional magnetic resonance imaging (EEG-fMRI) and metabolic neuroimaging have recently emerged as useful approaches to better understand the pathophysiological processes underlying ESES. Treatment of ESES is not just limited to seizures control but it should be focused on controlling neuropsychological outcome through an improvement of the continuous epileptiform activity. General agreement on treatment guidelines is still lacking. Implementation of new techniques might allow a better understanding of the pathophysiology of ESES and could enhance therapeutics options.

scholarly journals Autonomic Status Epilepticus in a Patient with Parasellar Meningioma: A Case Report

2021 ◽  
pp. 807-812
Author(s):  
Giacomo Evangelista ◽  
Fedele Dono ◽  
Stefano Consoli ◽  
Dario Calisi ◽  
Marco Onofrj ◽  
...  
Keyword(s):  
Heart Rate ◽  
Case Report ◽  
Status Epilepticus ◽  
Adult Patient ◽  
Present Report ◽  
Epileptiform Activity ◽  
Clinical Manifestations ◽  
Common Finding ◽  
Panayiotopoulos Syndrome ◽  
Visual Abnormalities

Autonomic status epilepticus (Aut SE) is a condition characterized by ongoing focal autonomic seizure lasting for >30 min. Aut SE can show a variety of clinical manifestations including vomiting, nausea, changes in heart rate, piloerection, pupillary abnormalities, and visual abnormalities. Although Aut SE is a common finding in childhood in the context of Panayiotopoulos syndrome, few reports have described this condition during adulthood. In the present report, we describe a case of Aut SE in an adult patient with parasellar meningioma and bilateral frontotemporal epileptiform activity on EEG record.

Electrical Status Epilepticus during Sleep and Evaluating the Electroencephalogram

2021 ◽  
Author(s):  
Michael Drees ◽  
Neil Kulkarni ◽  
Jorge Vidaurre
Keyword(s):  
Status Epilepticus ◽  
Clinical Manifestations ◽  
Epileptic Encephalopathy ◽  
Brain Lesions ◽  
Genetic Mutations ◽  
Immunological Markers ◽  
Age Related ◽  
Slow Sleep ◽  
Continuous Spikes And Waves ◽  
Structural Brain

AbstractElectrical status epilepticus during sleep (ESES) is an age-related, self-limited epileptic encephalopathy characterized by heterogeneous clinical manifestations and a specific electroencephalographic pattern of continuous spikes and waves during slow sleep. The etiology of ESES is not completely clear, although structural brain lesions, abnormal immunological markers, and genetic mutations have been associated with the syndrome. ESES was first described in 1971 and since then, the diagnostic criteria have changed multiple times. Additionally, inconsistency between authors in how to record and evaluate the electroencephalogram also leads to variability between studies. These inconsistencies hamper objectivity, comparison, and generalization. Because of this, one of the first priorities of physicians treating this condition should be defining the parameters of this disease so that cooperative building can occur.

scholarly journals Continuous Spikes and Waves during Sleep: Electroclinical Presentation and Suggestions for Management

2013 ◽  
Vol 2013 ◽  
pp. 1-12 ◽  
Author(s):  
Iván Sánchez Fernández ◽  
Kevin E. Chapman ◽  
Jurriaan M. Peters ◽  
Chellamani Harini ◽  
Alexander Rotenberg ◽  
...  
Keyword(s):  
Epileptiform Activity ◽  
Epileptic Encephalopathy ◽  
High Dose ◽  
Genetic Associations ◽  
Neurocognitive Deficits ◽  
Eeg Abnormalities ◽  
Electroencephalogram Eeg ◽  
Early Developmental ◽  
Continuous Spikes And Waves ◽  
Eeg Pattern

Continuous spikes and waves during sleep (CSWS) is an epileptic encephalopathy characterized in most patients by (1) difficult to control seizures, (2) interictal epileptiform activity that becomes prominent during sleep leading to an electroencephalogram (EEG) pattern of electrical status epilepticus in sleep (ESES), and (3) neurocognitive regression. In this paper, we will summarize current epidemiological, clinical, and EEG knowledge on CSWS and will provide suggestions for treatment. CSWS typically presents with seizures around 2–4 years of age. Neurocognitive regression occurs around 5-6 years of age, and it is accompanied by subacute worsening of EEG abnormalities and seizures. At approximately 6–9 years of age, there is a gradual resolution of seizures and EEG abnormalities, but the neurocognitive deficits persist in most patients. The cause of CSWS is unknown, but early developmental lesions play a major role in approximately half of the patients, and genetic associations have recently been described. High-dose benzodiazepines and corticosteroids have been successfully used to treat clinical and electroencephalographic features. Corticosteroids are often reserved for refractory disease because of adverse events. Valproate, ethosuximide, levetiracetam, sulthiame, and lamotrigine have been also used with some success. Epilepsy surgery may be considered in a few selected patients.

scholarly journals P.020 Novel GRIN2A variant in family members with variable phenotypic expression of epilepsy

2018 ◽  
Vol 45 (s2) ◽  
pp. S21-S21
Author(s):  
A Alanezi ◽  
C Campbell ◽  
N Karp ◽  
A Andrade
Keyword(s):  
Status Epilepticus ◽  
Language Development ◽  
Clinical Phenotype ◽  
Family Members ◽  
Phenotypic Expression ◽  
Epileptic Encephalopathy ◽  
Eeg Abnormalities ◽  
Language Regression ◽  
Eeg Features ◽  
Spike Wave

Background: Epilepsy aphasia spectrum of disorders is characterized by developmental and language regression with EEG abnormalities that include electrical status epilepticus of sleep (ESES). Landau-Kleffner syndrome (LKS) and epileptic encephalopathy with continuous spike-wave during sleep (CSWS) are the most severe presentations. GRIN2A mutations have been recognized as causative. Methods: we present two sisters with different epilepsy phenotypes. A variant of unknown clinical significance (VUS) in GRIN2A gene was found in one of the sisters and her similarly affected father. Results: The first sister presented with focal onset seizures at the age of 3 years accompanied by language and cognitive regression and EEG features consistent of ESES, meeting criteria for LKS. Multiple anticonvulsants were tried until she responded well to steroids regaining developmental milestones. Her 5-year-old sister recently presented with focal onset seizures. Her language development is appropriate. Her EEG showed independent multifocal spikes but no ESES during sleep. Her seizures were controlled on monotherapy anticonvulsants. Conclusions: We observed a variable EEG-clinical phenotype and different severity among these family members as expected with GRIN2A-related disorders. This report contributes to evidence of the GRIN2A variant pathogenicity.

The forsaking of the clinical EEG by psychiatry: how justified?

CNS Spectrums ◽  
2017 ◽  
Vol 23 (3) ◽  
pp. 196-204 ◽  
Author(s):  
Nash N. Boutros
Keyword(s):  
Clinical Significance ◽  
Psychiatric Patient ◽  
Psychiatric Symptoms ◽  
Epileptiform Activity ◽  
Panic Attacks ◽  
Paroxysmal Activity ◽  
Eeg Abnormalities ◽  
Anticonvulsant Treatment ◽  
The Many ◽  
Clinical Eeg

Despite decades of publications attesting to the role of the clinical EEG in diagnosing and managing psychiatric disorders, the procedure remains highly underutilized in the practice of psychiatry. The visually inspected EEG (vEEG) can detect various forms of abnormalities, each with its own clinical significance. Abnormalities can be paroxysmal (i.e., suggestive of an epileptic-like process) or stationary. The most important unanswered question remains the value of detecting epileptiform activity in a nonepileptic psychiatric patient in predicting favorable responses to anticonvulsant treatment. Despite the many shortcomings of vEEG, the available evidence suggests that in the presence of paroxysmal activity in a nonepileptic psychiatric patient a trial of a psychotropic anticonvulsant may be warranted if standard treatment has failed. More research on the contribution of paroxysmal EEG abnormalities to the problem of episodic psychiatric symptoms (e.g., panic attacks, dissociative episodes, repeated violence) is sorely needed. It is postulated that at least some of these conditions may represent an epilepsy spectrum disorder. Similarly, the significance of the presence of a slow-wave activity (whether focal or generalized) also deserves further well-designed research to ascertain the exact clinical significance. Nonetheless, the available data suggest that further medical workup is necessary to ascertain the nature and degree of the pathology when present.

scholarly journals B.6 High dose diazepam treatment for Electrical Status Epilepticus in Sleep (ESES): Is it effective?

2021 ◽  
Vol 48 (s3) ◽  
pp. S17-S17
Author(s):  
C Gorodetsky ◽  
T Go ◽  
S Weiss
Keyword(s):  
Status Epilepticus ◽  
Epileptiform Activity ◽  
Case Series ◽  
Epileptic Encephalopathy ◽  
Epilepsy Syndrome ◽  
Pediatric Epilepsy ◽  
High Dose ◽  
Retrospective Case ◽  
Cognitive Improvement ◽  
Clinically Significant

Background: Epileptic encephalopathy with electrical status epilepticus in sleep (ESES) is a pediatric epilepsy syndrome with sleep induced epileptic discharges and acquired impairment in cognition, language and/or behavior. Despite the widespread use of high dose diazepam, there is limited research on its efficacy. Methods: Single-center, retrospective case-series of children presenting with cognitive/ language regression and ESES from 2014-2019. All children underwent baseline overnight EEG followed by diazepam (1mg/kg) administered per rectum, and continuation of 0.5 mg/kg of oral diazepam for 3 months. Follow up EEGs were performed following the first dose and after 6-9 weeks of treatment. Results: 23 children were included [male 14 (60%); mean age 7 years (4 -12)]. 10 children (45%) had symptomatic epilepsy (defined by abnormal MRI and/or genetic evaluation). Decrease in more than 25% of the spike activity was seen in 18 (78%). This effect was sustained in 11 children (47%) after 6 weeks. Only 6 (60%) children from the symptomatic group had EEG response, while 11 (91%) responded from the idiopathic group. 5 children (21%) had clinically significant cognitive/ language improvement. Conclusions: Treatment with diazepam reduces epileptiform activity in ESES in majority of children. Despite this reduction only minority of patients experience clinically significant cognitive improvement.

Serial thin sectioning for pre- and post-embedding immunohistochemistry: Achieving consistent and reliable results for a 3-D reconstruction

1993 ◽  
Vol 51 ◽  
pp. 328-329
Author(s):  
Antonia M. Milroy
Keyword(s):  
Nervous System ◽  
Computer Assisted ◽  
Serial Sectioning ◽  
Electron Microscopic ◽  
High Quality ◽  
New Techniques ◽  
3 Dimensional ◽  
Multiple User ◽  
Thin Sectioning ◽  
Subcellular Level

In recent years many new techniques and instruments for 3-Dimensional visualization of electron microscopic images have become available. Higher accelerating voltage through thicker sections, photographed at a tilt for stereo viewing, or the use of confocal microscopy, help to analyze biological material without the necessity of serial sectioning. However, when determining the presence of neurotransmitter receptors or biochemical substances present within the nervous system, the need for good serial sectioning (Fig. 1+2) remains. The advent of computer assisted reconstruction and the possibility of feeding information from the specimen viewing chamber directly into a computer via a camera mounted on the electron microscope column, facilitates serial analysis. Detailed information observed at the subcellular level is more precise and extensive and the complexities of interactions within the nervous system can be further elucidated.We emphasize that serial ultra thin sectioning can be performed routinely and consistently in multiple user electron microscopy laboratories. Initial tissue fixation and embedding must be of high quality.

scholarly journals 2021 Update on the Clinical Management and Diagnosis of Kawasaki Disease

2021 ◽  
Vol 23 (3) ◽  
Author(s):  
Frank Zhu ◽  
Jocelyn Y. Ang
Keyword(s):  
High Risk ◽  
Kawasaki Disease ◽  
Corticosteroid Therapy ◽  
Clinical Management ◽  
Treatment Guidelines ◽  
Day Treatment ◽  
Significant Proportion ◽  
Clinical Manifestations ◽  
Heart Association ◽  
Ivig Resistance

Abstract Purpose of Review Provide an updated review of the clinical management and diagnosis of Kawasaki disease with inclusion of potential diagnostic difficulties with multisystem inflammatory syndrome in children (MIS-C) given the ongoing COVID-19 pandemic. Recent Findings Adjunctive corticosteroid therapy has been shown to reduce the rate of coronary artery dilation in children at high risk for IVIG resistance in multiple Japanese clinical studies (most notably RAISE study group). Additional adjunctive therapies (etanercept, infliximab, cyclosporin) may also provide limited benefit, but data is limited to single studies and subgroups of patients with cardiac abnormalities. The efficacy of other agents (atorvastatin, doxycycline) is currently being investigated. MIS-C is a clinically distinct entity from KD with broad clinical manifestations and multiorgan involvement (cardiac, GI, hematologic, dermatologic, respiratory, renal). MIS-C with Kawasaki manifestations is more commonly seen in children < 5 years of age. Summary The 2017 American Heart Association (AHA) treatment guidelines have included changes in aspirin dosing (including both 80–100 mg/kg/day and 30–50 mg/kg/day treatment options), consideration of the use of adjuvant corticosteroid therapy in patients at high risk of IVIG resistance, and the change in steroid regimen for refractory KD to include both pulse-dose IVMP and longer course of prednisolone with an oral taper. A significant proportion of children diagnosed with MIS-C, a post-infectious syndrome of SARS-CoV-2 infection, meet criteria for Kawasaki disease. Further investigation is warranted to further delineate these conditions and optimize treatment of these conditions given the ongoing COVID-19 pandemic.

scholarly journals Electroencephalography in systemic lupus erythematosus patients with neuropsychiatric manifestations

2020 ◽  
Vol 32 (1) ◽  
Author(s):  
Howaida E. Mansour ◽  
Reem A. Habeeb ◽  
Noran O. El-Azizi ◽  
Heba H. Afeefy ◽  
Marwa A. Nassef ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Epileptiform Activity ◽  
Brain Mri ◽  
Periventricular White Matter ◽  
Normal Brain ◽  
Systemic Lupus ◽  
Specific Test ◽  
Eeg Abnormalities ◽  
Neuropsychiatric Manifestations

Abstract Background Neuropsychiatric manifestations are frequently reported in systemic lupus erythematosus (SLE) patients. This study was done to describe electroencephalographic (EEG) findings in SLE patients with neuropsychiatric manifestation (NPSLE). Results Among 60 SLE patients, there were 50 females (83.3%) and 10 males (16.7%). EEG abnormalities were reported in 12 patients out of 30 (40%) with NPSLE, while all patients with non-NPSLE (n = 30) had no EEG abnormalities; diffuse slowing (20%) was the most common abnormalities, followed by generalized epileptiform activity (13.3%), and lastly temporal epileptiform activity (6.7%). Seizure was the most reported neuropsychiatric disorder in 13 patients (43.3%); 8 of them had abnormal EEG (61.5%). Periventricular white matter lesion (23.3%) followed by infarction (13.3%) were the most common MRI brain findings among 53.3% of NPSLE group. Half of the cases with EEG abnormality had normal brain MRI. SLEDAI score and ACL IgM positivity were higher in the NPSLE group than the non-NPSLE group. EEG is not a sensitive or specific test for detecting NPSLE with sensitivity (37.5%) and specificity (57.1%). Conclusion Not all patients with NPSLE must have abnormal brain MRI or EEG. EEG is a useful assistant tool in the assessment of different manifestations of NPSLE, but it cannot be used as a screening test alone and must be supplemented by neuroimaging studies.

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