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 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.

Epileptic Encephalopathy in Adams–Oliver Syndrome Associated to a New DOCK6 Mutation: A Peculiar Behavioral Phenotype

2018 ◽  
Vol 49 (03) ◽  
pp. 217-221 ◽  
Author(s):  
Livia Pisciotta ◽  
Valeria Capra ◽  
Andrea Accogli ◽  
Thea Giacomini ◽  
Giulia Prato ◽  
...  
Keyword(s):  
Epileptiform Activity ◽  
Epileptic Encephalopathy ◽  
Compound Heterozygosity ◽  
Limb Defects ◽  
Aplasia Cutis Congenita ◽  
Scalp Defects ◽  
Brain Malformations ◽  
Limb Malformations ◽  
Electroencephalogram Eeg ◽  
Aplasia Cutis

AbstractAdams–Oliver syndrome (AOS) is characterized by a combination of congenital scalp defects (aplasia cutis congenita) and terminal transverse limb malformations of variable severity. When neurological findings are present, patients are reported as AOS variants. We describe a child with compound heterozygosity of the DOCK6 gene, aplasia cutis, terminal transverse limb defects, cardiovascular impairment, intellectual disability, and brain malformations with intracranial calcifications. He suffers from a severe refractory epileptic encephalopathy characterized by polymorphic seizures with prolonged periods of electroencephalogram (EEG), continuous epileptiform activity related to clinical inactivity, and closure of eyes with an “ON-OFF” behavior.

Rotavirus-Induced Neonatal Epileptic Encephalopathy—A Disease Spectrum Illustrated by Monochorionic Twins

2019 ◽  
Vol 51 (01) ◽  
pp. 062-067 ◽  
Author(s):  
Esther M. Hopmans ◽  
Astrid van der Heide ◽  
Pui Khi Chung ◽  
Daniëlle Brinkman ◽  
Mariet C.W. Feltkamp ◽  
...  
Keyword(s):  
White Matter ◽  
Epileptic Encephalopathy ◽  
Neonatal Seizures ◽  
Monochorionic Twins ◽  
Rotavirus Infection ◽  
Disease Spectrum ◽  
Pattern Characteristic ◽  
Magnetic Resonance Imaging Mri ◽  
Electroencephalogram Eeg ◽  
Eeg Pattern

AbstractRotavirus has been associated with neonatal seizures and specific white matter magnetic resonance imaging (MRI) abnormalities. We describe monochorionic twins who not only tested positive for rotavirus with these white matter MRI abnormalities but who also showed an electroencephalogram (EEG) pattern characteristic of early infantile epileptic encephalopathy (EIEE), which has so far solely been described in epileptic encephalopathies with a poor prognosis. This report suggests that rotavirus infection must be added to the list of causes of EIEE EEG, and that the outcome then is likely more favorable. As MRI and EEG signs of rotavirus encephalopathy were present in one twin with only subtle neurologic symptoms, rotavirus may well cause insidious central nervous system complications more often. We suggest considering rotavirus infection in neonates presenting with seizures, and to add rotavirus infection to the differential diagnosis of EIEE.

scholarly journals Non-epileptiform EEG abnormalities: an overview

2011 ◽  
Vol 69 (5) ◽  
pp. 829-835 ◽  
Author(s):  
Maria Emilia Cosenza Andraus ◽  
Soniza Vieira Alves-Leon
Keyword(s):  
Neurological Disorders ◽  
Epileptiform Activity ◽  
Brain Dysfunction ◽  
Accurate Information ◽  
Cerebral Function ◽  
Periodic Patterns ◽  
Eeg Abnormalities ◽  
Pubmed Database ◽  
Electroencephalogram Eeg ◽  
The Brain

More than 80 years after its introduction by Hans Berger, the electroencephalogram (EEG) remains as an important supplementary examination in the investigation of neurological disorders and gives valuable and accurate information about cerebral function. Abnormal EEG findings may include ictal patterns, interictal epileptiform activity and non-epileptiform abnormalities. The aim of this study is to make an overview on the main non-epileptiform EEG abnormalities, emphasizing the pathologic findings and the importance of their recognition, excluding periodic patterns and EEG physiologic changes. Scientific articles were selected from MEDLINE and PubMed database. The presence of non-epileptiform EEG abnormalities provide evidence of brain dysfunction that are not specific to a particular etiology and may be related to a number of disorders affecting the brain. Although these abnormalities are not specific, they can direct attention to the diagnostic possibilities and guide the best treatment choice.

scholarly journals Diazepam induced sleep spindle increase correlates with cognitive recovery in a child with epileptic encephalopathy

BMC Neurology ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
S. M. Stoyell ◽  
B. S. Baxter ◽  
J. McLaren ◽  
H. Kwon ◽  
D. M. Chinappen ◽  
...  
Keyword(s):  
Cognitive Function ◽  
Epileptiform Activity ◽  
Epileptic Encephalopathy ◽  
Sleep Spindles ◽  
High Dose ◽  
Sleep Spindle ◽  
Epileptic Encephalopathies ◽  
Cognitive Improvement ◽  
Epileptiform Discharges ◽  
Neuropsychological Evaluations

Abstract Background Continuous spike and wave of sleep with encephalopathy (CSWS) is a rare and severe developmental electroclinical epileptic encephalopathy characterized by seizures, abundant sleep activated interictal epileptiform discharges, and cognitive regression or deceleration of expected cognitive growth. The cause of the cognitive symptoms is unknown, and efforts to link epileptiform activity to cognitive function have been unrevealing. Converging lines of evidence implicate thalamocortical circuits in these disorders. Sleep spindles are generated and propagated by the same thalamocortical circuits that can generate spikes and, in healthy sleep, support memory consolidation. As such, sleep spindle deficits may provide a physiologically relevant mechanistic biomarker for cognitive dysfunction in epileptic encephalopathies. Case presentation We describe the longitudinal course of a child with CSWS with initial cognitive regression followed by dramatic cognitive improvement after treatment. Using validated automated detection algorithms, we analyzed electroencephalograms for epileptiform discharges and sleep spindles alongside contemporaneous neuropsychological evaluations over the course of the patient’s disease. We found that sleep spindles increased dramatically with high-dose diazepam treatment, corresponding with marked improvements in cognitive performance. We also found that the sleep spindle rate was anticorrelated to spike rate, consistent with a competitively shared underlying thalamocortical circuitry. Conclusions Epileptic encephalopathies are challenging electroclinical syndromes characterized by combined seizures and a deceleration or regression in cognitive skills over childhood. This report identifies thalamocortical circuit dysfunction in a case of epileptic encephalopathy and motivates future investigations of sleep spindles as a biomarker of cognitive function and a potential therapeutic target in this challenging disease.

scholarly journals Reiterating the role of corpus callosum in generalization of interictal and ictal epileptiform discharges: a case report with post-callosotomy intracranial electroencephalography in Lennox–Gastaut syndrome

2021 ◽  
Vol 13 (3) ◽  
pp. 249-253
Author(s):  
S. Gopinath ◽  
A. Pillai ◽  
A. G. Diwan ◽  
J. V. Pattisapu ◽  
K. Radhakrishnan
Keyword(s):  
Corpus Callosum ◽  
Epileptic Encephalopathy ◽  
Drop Attacks ◽  
Epileptiform Discharges ◽  
Interictal Epileptiform Discharges ◽  
Eeg Abnormalities ◽  
Before And After ◽  
Intracranial Electroencephalography ◽  
Electroencephalogram Eeg

Lennox–Gastaut syndrome (LGS) is an epileptic encephalopathy characterized by delayed mental development and intractable multiple seizure types, predominantly tonic. Drop attacks are the commonest and the most disabling type of seizures. Resective surgery is often not possible in LGS as the electroencephalogram (EEG) abnormalities are usually multifocal and generalized, and magnetic resonance image is often either normal or multilesional. We report a case of LGS with bilateral parieto-occipital gliosis where EEG before and after callosotomy demonstrated synchronized bilateral interictal epileptiform discharges and ictal discharges becoming desynchronized and running down. This phenomenon emphasizes the role of the corpus callosum in secondary bilateral synchrony.

Serial EEG Monitoring in a Patient With Anti-NMDA Receptor Encephalitis

2016 ◽  
Vol 48 (4) ◽  
pp. 301-303 ◽  
Author(s):  
Yanfang Shi
Keyword(s):  
Clinical Symptoms ◽  
Eeg Monitoring ◽  
Nmda Receptor Encephalitis ◽  
Aspartate Receptor ◽  
Nmdar Encephalitis ◽  
Eeg Abnormalities ◽  
Severity Of The Disease ◽  
Electroencephalogram Eeg ◽  
Eeg Pattern ◽  
Video Eeg

Electroencephalogram (EEG) abnormalities are very common in anti- N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis. Extreme delta brush (EDB) is a distinctive EEG pattern that is can be suggestive of the diagnosis; however, the etiology of the EDB remains unclear. Furthermore, there is question with regard to its ictal or interictal nature. We report a 20-year-old woman with anti-NMDAR encephalitis whose serial video-EEG monitoring was obtained at 2, 2.5, 4, and 6 months after admission. There was a long-standing EDB lasting up to several hours, with no evolution in frequency, amplitude, or morphology, and without clear association her frequent orofacial dyskinesia. Intravenous benzodiazepine administrations did not change the EDB pattern. As her clinical symptoms improved, the EDB gradually became less prominent and less frequent, with complete resolution at 6 months after admission. These findings suggest that EDB is more likely a marker of the severity of the disease in contrast to an epileptic seizure and is useful for diagnosis and monitoring of treatment response in conjunction with clinical improvement.

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.

scholarly journals Depth Electrode Guided Anterior Insulectomy: 2-Dimensional Operative Video

2021 ◽  
Author(s):  
Mauricio Mandel ◽  
Layton Lamsam ◽  
Pue Farooque ◽  
Dennis Spencer ◽  
Eyiyemisi Damisah
Keyword(s):  
Right Hemisphere ◽  
Preoperative Evaluation ◽  
Epileptiform Activity ◽  
Neurological Deficits ◽  
Depth Electrodes ◽  
Depth Electrode ◽  
Patient Reported ◽  
Frontal Operculum ◽  
History Of ◽  
Electroencephalogram Eeg

Abstract The insula is well established as an epileptogenic area.1 Insular epilepsy surgery demands precise anatomic knowledge2-4 and tailored removal of the epileptic zone with careful neuromonitoring.5 We present an operative video illustrating an intracranial electroencephalogram (EEG) depth electrode guided anterior insulectomy.  We report a 17-yr-old right-handed woman with a 4-yr history of medically refractory epilepsy. The patient reported daily nocturnal ictal vocalization preceded by an indescribable feeling. Preoperative evaluation was suggestive of a right frontal-temporal onset, but the noninvasive results were discordant. She underwent a combined intracranial EEG study with a frontal-parietal grid, with strips and depth electrodes covering the entire right hemisphere. Epileptiform activity was observed in contact 6 of the anterior insula electrode. The patient consented to the procedure and to the publication of her images.  A right anterior insulectomy was performed. First, a portion of the frontal operculum was resected and neuronavigation was used for the initial insula localization. However, due to unreliable neuronavigation (ie, brain shift), the medial and anterior borders of the insular resection were guided by the depth electrode reference. The patient was discharged 3 d after surgery with no neurological deficits and remains seizure free.  We demonstrate that depth electrode guided insular surgery is a safe and precise technique, leading to an optimal outcome.

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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