Atypical Evolutions of Idiopathic Focal Epilepsies in Childhood

2016 ◽  
Vol 05 (03) ◽  
pp. 122-132
Author(s):  
Roberto Caraballo ◽  
Santiago Flesler ◽  
Natalio Fejerman
Keyword(s):  
Status Epilepticus ◽  
Focal Epilepsy ◽  
Childhood Epilepsy ◽  
Significant Percentage ◽  
Epileptic Encephalopathies ◽  
Atypical Features ◽  
Eeg Abnormalities ◽  
Number Of Patients ◽  
Panayiotopoulos Syndrome ◽  
Eeg Features

Clinical and electroencephalographic (EEG) features of the three well-defined idiopathic focal epilepsies in childhood (IFEC)—benign childhood epilepsy with centrotemporal spikes (BCECTS), Panayiotopoulos syndrome, and the Gastaut type of idiopathic occipital epilepsy of childhood (IOEC-G)—have been clearly described and reported. It is also known that a significant percentage of children with IFEC present what were named atypical features in seizures and EEG abnormalities. We are studying here the small number of patients with IFEC who not only present atypical features, but also evolve into the spectrum of epileptic encephalopathies related with continuous spike-and-wave during sleep (CSWSS) or electrical status epilepticus during sleep (ESES), which includes atypical benign focal epilepsy of childhood, status of BCECTS, Landau-Kleffner syndrome, and CSWSS or ESES syndrome. We also emphasize that some patients with these encephalopathic course of IFEC present a mix or a sequence in time of the four mentioned subsyndromes.

scholarly journals Early Onset Benign Occipital Epilepsy (Panayiotopoulos syndrome): a case Report & Literature Review

2015 ◽  
Vol 1 (1) ◽  
pp. 27-30
Author(s):  
Md Enayet Hussain ◽  
Rajib Nayan Chowdhury ◽  
Md Nahidul Islam ◽  
AFM Al Masum Khan ◽  
Md Ferdous Miah
Keyword(s):  
Case Report ◽  
Status Epilepticus ◽  
Literature Review ◽  
Febrile Seizures ◽  
Great Variability ◽  
Epilepsy Syndrome ◽  
Childhood Epilepsy ◽  
High Prevalence ◽  
Occipital Spikes ◽  
Panayiotopoulos Syndrome

Panayiotopoulos syndrome is a type of benign childhood epilepsy syndrome characterized by episodic autonomic and mainly emetic symptoms. It predominantly affects children of 3-6 years of age (13% of those with one or more non-febrile seizures). EEG shows great variability, with occipital, extra-occipital spikes or brief generalised discharges alone or in combination; it may also be consistently normal. Despite the high prevalence of autonomic status epilepticus, the prognosis of Panayiotopoulos syndrome is usually excellent and most do not require anti epileptic medications. Remission usually occurs within 1-2 years from onset. One third have a single seizure but 5-10% may have more than 10 seizures or a more prolonged course. We present a 5 year old boy with this condition, who presented with episodes of severe vomiting, deviation of eyes, focal seizure, altered sensorium, characteristic EEG in the form of multiple occipital spikes and normal neuroimaging studies.J. Natl Inst. Neurosci Bangladesh 2015;1(1):27-30

scholarly journals Panayiotopoulos syndrome: a clinico-electroencephalographic presentation of three cases from Nigeria

2019 ◽  
Vol 6 (2) ◽  
pp. 875
Author(s):  
Christian Chukwukere Ogoke
Keyword(s):  
Status Epilepticus ◽  
Febrile Seizures ◽  
Focal Seizure ◽  
Seizure Susceptibility ◽  
Epileptic Syndrome ◽  
Cardiogenic Syncope ◽  
Impaired Awareness ◽  
Panayiotopoulos Syndrome ◽  
Eeg Features

Panayiotopoulos syndrome (PS) is a common benign, childhood, focal, seizure susceptibility syndrome presenting with mainly focal aware or focal impaired awareness autonomic seizures and autonomic status epilepticus that is under diagnosed in our locality. Increased awareness through reporting will aid diagnosis, reduce misdiagnosis and prevent aggressive and deleterious interventions. The classic clinical and electroencephalographic (EEG) features of three cases are described and the literature reviewed. The aim is to underscore the presence in our locality of this remarkably benign epileptic syndrome that must be differentiated from febrile seizures, encephalopathy, migraine or cardiogenic syncope.

scholarly journals Autonomic seizures and autonomic status epilepticus in early onset benign childhood occipital epilepsy (Panayiotopoulos syndrome)

2006 ◽  
Vol 64 (3b) ◽  
pp. 723-726 ◽  
Author(s):  
Gloria Maria Almeida Souza Tedrus ◽  
Lineu Corrêa Fonseca
Keyword(s):  
Status Epilepticus ◽  
Early Onset ◽  
Brain Lesions ◽  
Motor Symptoms ◽  
Autonomic Symptoms ◽  
Visual Symptoms ◽  
Occipital Spikes ◽  
Panayiotopoulos Syndrome ◽  
Eeg Features ◽  
Spike Wave

To study clinical and EEG features of children with ictal vomiting and no underlying brain lesions (Panayiotopoulos syndrome). The subjects were 36 children aged 2-13 years. The onset of seizures occurred between 1 and 5 years of age. Fourteen children (38.8%) had a single seizure. Fourteen children (38.8%) had autonomic status epilepticus. Impairment of consciousness was reported in 30 (83.3%) children, eye deviation in 10 (27.7%) other autonomic symptoms and head deviation in 9, generalization in 8, visual symptoms in one child, and, speech arrest or hemifacial motor symptoms in 8 cases. The EEG showed occipital spikes or spike-wave complexes in 27 (75.0%) children, blocked by opening of the eyes in 8 (22.2%) cases. Nine patients (25%) also had rolandic spikes and 3 had extraoccipital spikes. Six (16.6%) patients had normal EEG. No clinical differences were observed between patients having occipital or extraoccipital spikes. In children only with autonomic seizures, the spikes are predominantly occipital but blockage by opening of the eyes is a less frequent feature. In some children there is an overlapping of different focal childhood idiopathic syndromes.

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.

scholarly journals Nonconvulsive status epilepticus associated with Alzheimer’s disease mimicking symptomatic focal epilepsy following the resection of a frontal parasagittal meningioma

2020 ◽  
Vol 11 ◽  
pp. 469
Author(s):  
Keisuke Abe ◽  
Nobutaka Mukae ◽  
Takato Morioka ◽  
Yuhei Sangatsuda ◽  
Ayumi Sakata ◽  
...  
Keyword(s):  
Alzheimer’S Disease ◽  
Alzheimer's Disease ◽  
Status Epilepticus ◽  
Focal Epilepsy ◽  
Nonconvulsive Status Epilepticus ◽  
Histopathological Diagnosis ◽  
Epileptogenic Lesion ◽  
Consciousness Disorder ◽  
Impaired Awareness ◽  
The Right

Background: Epilepsies are frequent in patients with Alzheimer’s disease (AD); however, epilepsies in AD can easily go unrecognized because they usually present as focal impaired awareness seizures or nonconvulsive status epilepticus (NCSE) and can overlap with other symptoms of AD. Case Description: We performed an epilepsy surgery in a 69-year-old woman with progressive cognitive impairment and consciousness disorder, who was diagnosed with focal NCSE related to the resected meningioma in the right frontal parasagittal region. Intraoperative electrocorticography revealed localized periodic paroxysmal discharges with beta and gamma activities in the neighboring cortex where the meningioma existed. The histopathological diagnosis of AD was first made from the resected epileptogenic cortex. Conclusion: Even when there is a suspected epileptogenic lesion that could cause focal NCSE, AD should be ruled out in elderly patients with progressive cognitive decline.

scholarly journals Corrigendum to Institutional Factors Contribute to Variation in Intubation Rates in Status Epilepticus

2020 ◽  
Vol 10 (2) ◽  
pp. 155-155
Keyword(s):  
Status Epilepticus ◽  
Nationwide Inpatient Sample ◽  
Personal Information ◽  
Data Use ◽  
Healthcare Research ◽  
Online Version ◽  
Institutional Factors ◽  
Restricted Access ◽  
Number Of Patients ◽  
Inpatient Database

Guterman EL, Burke JF, Josephson SA, and Betjemann JP. Institutional Factors Contribute to Variation in Intubation Rates in Status Epilepticus, The Neurohospitalist. 2019;9:133-139. DOI: 10.1177/1941874418819349 In this article, the supplemental table S1 included cells where the number of patients was < 10 which is in violation of the HCUP data use agreement. A revised online version of the supplemental material that is in compliance with the AHRQ guidelines has been posted online. This article is an analysis of the Nationwide Inpatient Sample (NIS), a large, restricted-access, publicly available dataset and the State Inpatient Database (SID), a restricted-access, publicly available dataset that are maintained by the Agency for Healthcare Research and Quality (AHRQ). One of the provisions of the Data Use Agreement (DUA), which the author signed when purchasing the data, is that no data involving less than or equal to 10 observations are to be published. This measure was put in place by AHRQ to protect individual patients’ privacy and to prevent the potential disclosure of personal information. In the manuscript, there are two tabulated cells in the supplemental table S1 that are in violation of this provision. This represents an oversight on the authors’ part and an inadvertent failure to comply with the Data Use Agreement, for which they apologize. While they are fully confident that nothing in the paper could be used to identify individuals or their personal information, the fact remains that the disclosures are in violation of the AHRQ guidelines

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 Higher mortality rate is associated with advanced age and periodic lateralized epileptiform discharges in patients with refractory status epilepticus

2013 ◽  
Vol 71 (3) ◽  
pp. 153-158 ◽  
Author(s):  
Paulo Breno Noronha Liberalesso ◽  
Eliana Garzon ◽  
Elza M.T. Yacubian ◽  
Américo C. Sakamoto
Keyword(s):  
Status Epilepticus ◽  
Mortality Rate ◽  
Clinical Data ◽  
Focal Epilepsy ◽  
Refractory Status Epilepticus ◽  
Advanced Age ◽  
Neurological Sequelae ◽  
Epileptiform Discharges ◽  
Refractory Status ◽  
Treatment Morbidity

ObjectiveTo evaluate clinical data, electroencephalogram, etiology, classification, treatment, morbidity, and mortality in acute refractory status epilepticus.MethodsFifteen patients, mean age of 41.3 years-old, six males, with refractory status epilepticus, were retrospectively studied. All of them were followed by serial electroencephalogram or continuous electroencephalographic monitoring.ResultsThe most common comorbidity was hypertension. Seven (46.7%) patients were diagnosed with previous symptomatic focal epilepsy. More than one etiology was identified in 40.0% of the cases. Status epilepticus partial complex was the most common (n=14, 93.3%), and discrete seizures were the most observed initial ictal pattern. Continuous intravenous midazolam was used in nine (60.0%) patients and continuous thiopental in three (20.0%). Nine (60.0%) participants died, one (6.6%) had neurological sequelae, and five (33.3%) presented no neurological sequelae.ConclusionsHigher mortality rate was associated with advanced age and periodic lateralized epileptiform discharges. Midazolam proved to be a safe drug. The refractory status epilepticus is related to high mortality.

scholarly journals The efficacy of lacosamide as monotherapy and adjunctive therapy in focal epilepsy and its use in status epilepticus: clinical trial evidence and experience

2016 ◽  
Vol 10 (2) ◽  
pp. 103-126 ◽  
Author(s):  
Sebastian Bauer ◽  
Laurent M. Willems ◽  
Esther Paule ◽  
Christine Petschow ◽  
Johann Philipp Zöllner ◽  
...  
Keyword(s):  
Status Epilepticus ◽  
Sodium Channels ◽  
Focal Epilepsy ◽  
Comparable Efficacy ◽  
Slow Inactivation ◽  
Seizure Reduction ◽  
Voltage Gated Sodium Channels ◽  
Clinical Trial Evidence ◽  
Post Marketing ◽  
Trial Evidence

Lacosamide (LCM) is approved for anticonvulsive treatment in focal epilepsy and exhibits its function through the slow inactivation of voltage-gated sodium channels (VGSCs). LCM shows comparable efficacy with other antiepileptic drugs (AEDs) licensed in the last decade: in three randomized placebo-controlled trials, significant median seizure reduction rates of 35.2% for 200 mg/day, 36.4–39% for 400 mg/day and 37.8–40% for 600 mg/day were reported. Likewise, 50% responder rates were 38.3–41.1% for 400 mg/day and 38.1–41.2% for 600 mg/day. Similar rates were reported in post-marketing studies. The main adverse events (AEs) are dizziness, abnormal vision, diplopia and ataxia. Overall, LCM is well tolerated and has no clinically-relevant drug–drug interactions. Due to the drug’s intravenous availability, its use in status epilepticus (SE) is increasing, and the available data are promising.

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