Background: Rasmussen encephalitis (RE) is a rare inflammatory disease affecting one hemisphere, causing progressive neurological deficits and intractable seizures.
Objective: To report long-term seizure outcomes, reoperations, and functional outcomes in patients with RE who underwent hemispherectomy at our institution.
Methods: Retrospective review was performed for all patients with RE who had surgery between 1998 and 2020.
Objective: The objectives of this study were to determine the relationship between the severity of pathology and seizure outcomes in patients who underwent hemispherectomy for Rasmussen encephalitis (RE) and to investigate which clinical factors correlated with severity of pathology.
Methods: In this retrospective cohort study, we collected and reviewed pathology and clinical variables. We ascertained seizure outcomes using Engel's classification, and Pardo stages were used to grade pathology.
Objective: Epilepsy with eyelid myoclonias(EMA) is a genetic generalized epilepsy (GGE) characterized by eyelid myoclonia, eye-closure sensitivity and photosensitivity. Data on EMA patients who specifically present with photoparoxysmal response on EEG is lacking. EMA is an under-recognized syndrome which is frequently misclassified as another GGE.
View Article and Find Full Text PDFObjective: Prior surgical series in children with drug-resistant epileptic spasms have reported use of intracranial EEG monitoring in up to two-third of patients. We report outcome after epilepsy surgery for drug-resistant epileptic spasms in a cohort of children without the use of intracranial EEG monitoring in any of the patients.
Methods: Medical records of all consecutive children aged 5 years or under who had epilepsy surgery for epileptic spasms at Cleveland Clinic between 2000 and 2018 were reviewed.
Purpose: The East London Health and Care Partnership (ELHCP) Data Repository was established to support commissioning decisions in London. This dataset comprises routine clinical data for the general practitioner (GP)-registered populations of two London boroughs, Tower Hamlets and City and Hackney, and provides a rich source of demographic, clinical and health service use data of relevance to clinicians, commissioners, researchers and policy makers. This paper describes the dataset in its current form, its representativeness and data completeness.
View Article and Find Full Text PDFObjective: To examine longitudinal seizure and functional outcomes after hemispherectomy in adults and adolescents.
Methods: We reviewed 47 consecutive patients older than 16 years who underwent hemispherectomy between 1996 and 2016 at our center. Clinical, electroencephalographic (EEG), imaging, neuropsychological, surgical, and functional status data were analyzed.
OBJECTIVEThe goal in the study was to describe the clinical outcomes associated with robot-assisted stereoelectroencephalography (SEEG) in children.METHODSThe authors performed a retrospective, single-center study in consecutive children with medically refractory epilepsy who were undergoing robot-assisted SEEG. Kaplan-Meier survival analysis was used to calculate the probability of seizure freedom.
View Article and Find Full Text PDFSafeguarding children teaching is a required component in all pre-registration nursing curricula. A structured approach to this teaching as part of preparation for registration as a children's nurse was developed jointly by the Course Leader and the Designated Nurse for Safeguarding Children. This approach aims to equip children's nurses with the necessary theoretical knowledge and practical skills needed for safe practice.
View Article and Find Full Text PDFCognitive dysfunction in children with epilepsy is primarily contributed by etiology, seizures, frequency of interictal epileptiform discharges, and adverse effects of antiepileptic drugs. The direct effect of epilepsy surgery on cognitive outcome depends on two key factors: the function that is present in the epileptogenic zone to be removed, and the dysfunction outside the epileptogenic zone caused by epilepsy. Studies on cognitive outcome in children after various types of epilepsy surgery estimate "no significant change" in about 70% of children, improvement in cognition in 10%-15%, and decline in 10%-15%.
View Article and Find Full Text PDFWe report four children who highlight the potentially under-recognized electroencephalogram pattern of bilaterally synchronous occipito-frontal sharp waves, which may occur in children with Panayiotopoulos type self-limited childhood epilepsy but may be easily confused with the patterns of symptomatic generalized epilepsy. Our patients were young, healthy children who had infrequent, predominantly nocturnal, fairly prolonged seizures characterized by altered consciousness, vomiting, and autonomic features, or in one case nocturnal secondary generalized tonic-clonic convulsion. Their electroencephalograms showed stereotyped abundant sleep-activated sharp waves with maximum negativity in bilateral occipital and frontal greater than centro-temporo-parietal regions.
View Article and Find Full Text PDFThe optimal treatment for medically refractory epilepsy in Aicardi syndrome (AS) is still unclear. Palliative surgical treatment, including vagus nerve stimulation and corpus callosotomy, has therefore been used. There is limited data on the role of resective epilepsy surgery as a treatment choice in patients with AS.
View Article and Find Full Text PDFAJNR Am J Neuroradiol
December 2016
Background And Purpose: Rasmussen syndrome, also known as Rasmussen encephalitis, is typically associated with volume loss of the affected hemisphere of the brain. Our aim was to apply automated quantitative volumetric MR imaging analyses to patients diagnosed with Rasmussen encephalitis, to determine the predictive value of lobar volumetric measures and to assess regional atrophy differences as well as monitor disease progression by using these measures.
Materials And Methods: Nineteen patients (42 scans) with diagnosed Rasmussen encephalitis were studied.
Cognitive and developmental outcomes in patients with epileptic encephalopathy are hypothesized to result from an interplay between the underlying epileptic pathologic substrate and the acquired consequences of frequent and repetitive seizures and epileptiform discharges that often straddle the interictal and ictal boundaries. This article briefly reviews the evidence related to this assumption, presents critical questions that need to be answered to clarify this relationship, and advances a set of concrete steps that may help improve developmental patient outcomes.
View Article and Find Full Text PDFIntroduction: The term "hemispheric malformation of cortical development" (MCD) has come into the medical lexicon in the past 20 years as improvements and availability of advanced imaging techniques have permitted more precise diagnosis of a variety of brain developmental disorders that affect large regions of brain. These conditions are united by their propensity to elicit seizures that are difficult to control with medication in the children who suffer them.
Purpose: The goal of surgical intervention is always to achieve seizure freedom and thereby give the affected child the best possible hope for neurological development.
Background: Although stereoelectroencephalography (SEEG) has been shown to be a valuable tool for preoperative decision making in focal epilepsy, there are few reports addressing the utility and safety of SEEG methodology applied to children and adolescents.
Objective: To present the results of our early experience using SEEG in pediatric patients with difficult-to-localize epilepsy who were not considered candidates for subdural grid evaluation.
Methods: Thirty children and adolescents with the diagnosis of medically refractory focal epilepsy (not considered ideal candidates for subdural grids and strip placement) underwent SEEG implantation.
Stereo-electroencephalography (SEEG) has advantages for exploring deeper epileptic foci. Nevertheless, SEEG can only sample isolated cortical areas and its spatial limitation, with the inability to record contiguous cortical regions, may cause difficulties in interpretation. In light of these limitations, the authors describe the hybrid technique of SEEG and subdural strip electrode placement.
View Article and Find Full Text PDFPurpose: To examine the long-term functional outcomes and their predictors using a patient/family centered approach in a cohort of children who had hemispherectomy. Functional outcome measures studied were the following: ambulation ability, visual symptoms, spoken language, reading skills, and behavioral problems.
Methods: We reviewed 186 consecutive children who underwent hemispherectomy between 1997 and 2009 at our center.
The spectrum of focal epileptogenic lesions and their clinical manifestations in children differ substantially from those seen in adults. In adults, mesial temporal sclerosis is the common lesion in surgical series; but in children, MTS is uncommon, and when it does occur, it exists frequently as dual pathology. The most common lesions in pediatric epilepsy surgery candidates are malformations of cortical development, developmental tumors, or encephalomalacia from infarction, hypoxia, trauma, or infection.
View Article and Find Full Text PDFObjective: Data on longitudinal seizure outcome after hemispherectomy in children are limited. This study explores the posthemispherectomy longitudinal seizure outcome and its predictors.
Methods: We reviewed 186 consecutive children who underwent hemispherectomy between 1997 and 2009 at our center.
The coexistence of focal and idiopathic generalized epilepsy (IGE) is rarely observed, and the mechanism underlying this situation remains unknown. We report a 13-year-old girl with well-controlled generalized epilepsy and medically-refractory left temporal lobe epilepsy. She underwent intracranial EEG recording, which demonstrated two characteristic seizure patterns of generalized ictal onset and left mesial temporal onset.
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