Journal of Pediatric Epilepsy - Volume 3, issue 3

Authors: Castillo, Eduardo M.

Article Type: Other

DOI: 10.3233/PEP-14086

Citation: Journal of Pediatric Epilepsy, vol. 3, no. 3, pp. 129-130, 2014

Authors: Korostenskaja, Milena | Griskova-Bulanova, Inga | Lee, Ki H. | Chen, Po-Ching | Kleineschay, Tara S. | Cook, Jane C. | Schaal, Alex E. | Castillo, Eduardo M.

Article Type: Review Article

Abstract: Childhood epileptic encephalopathies (CEE) are often characterized by abnormal brain development in which epileptic electrical discharges may contribute to cognitive deficits, behavioral disturbances, or psychomotor dysfunction. Electroencephalography (EEG) is the primary diagnostic imaging tool used by care providers in order to observe the aggressive paroxysmal activity that manifests from CEE. Though EEG is an effective diagnostic tool, it provides limited insight into the etiology and pathophysiology that can be obtained through other imaging techniques. The aim of this paper is to review current neuroimaging techniques, such as intracranial EEG, magnetoencephalography, functional magnetic resonance imaging, and positron emission tomography and discuss …how they complement EEG findings and contribute to our understanding of CEE. In addition, we address the value of imaging techniques in defining and predicting cognitive changes in CEE. Show more

Keywords: Electrocorticography, EEG, encephalopathy, epilepsy, epilepsy surgery, functional MRI, neuroimaging, pediatrics, MEG, PET

DOI: 10.3233/PEP-14087

Citation: Journal of Pediatric Epilepsy, vol. 3, no. 3, pp. 131-156, 2014

Authors: Salinas, Christine M. | Westerveld, Michael | Lee, Ki Hyeong

Article Type: Review Article

Abstract: Epileptic encephalopathies are severe pediatric syndromes in which aggressive epileptiform discharges and seizures are thought to lead to profound cognitive impairments due to delay or failure of developmental skills to emerge, arrest of early normal development resulting in a cognitive plateau, and regression or deterioration of previously acquired cognitive skills. Children with epileptic encephalopathy usually have a combination of early seizure onset, sometimes as early as in infancy with severe pathology, chronicity, and multiple seizure types, including but not limited to status events or frequent abnormal interictal patterns, requiring antiepileptic drugs polypharmacy, making these some of the most challenging epilepsy …cases with poor cognitive and behavioral prognosis. We describe prognostic indicators for cognitive and behavioral comorbidity in childhood epilepsy. The main focus of the paper is to provide an overview of several epileptic encephalopathies by summarizing their clinical features, providing updates on recent advances in imaging and genetics, and discussing neurodevelopmental outcomes with respect to various treatment modalities, including epilepsy surgery. Show more

Keywords: Epilepsy, children, encephalopathy, neuropsychology, cognitive, development

DOI: 10.3233/PEP-14090

Citation: Journal of Pediatric Epilepsy, vol. 3, no. 3, pp. 157-171, 2014

Authors: Caraballo, Roberto H. | Vilte, Carolina | Chamorro, Noelia | Fortini, Pablo S. | Ramirez, Belen | Cersósimo, Ricardo

Article Type: Research Article

Abstract: We present an update of a series of patients with myoclonic status in non-progressive encephalopaties (MSNPE) describing the electroclinical features and outcome. Between February 1, 1990 and June 30, 2011, 41 patients who met diagnostic criteria of MSNPE were seen at our department and followed up to the present time. Three main subgroups were identified. The first subgroup of 27 patients presented with myoclonic absences and rhythmic myoclonias followed by a brief silent period associated with subcontinuous delta-theta activity involving the central areas and rhythmic delta waves with superimposed spikes mainly involving the parietooccipital regions and often activated by eye …closure. This pattern was found in all children with a genetic etiology. The second subgroup included seven patients showing a pattern characterized by inhibitory phenomena associated with a dystonic component and sudden irregular rapid lightning-like jerks. The electroencephalography showed subcontinuous multifocal slow spike-waves, predominating in frontocentral regions. These patients had a cortical malformation or the etiology was unknown. The third subgroup included seven children who initially suffered from myoclonic absences. The status was initially characterized by subcontinuous generalized spike-wave-type paroxysms related to rhythmic myoclonia of face and limbs. After 1 to 3 wk, the electroencephalography showed sharp theta waves with very slow pseudorhythmic continuous spikes in the central regions and vertex. The etiology was found to be perinatal anoxic injury. MSNPE is a well-defined epileptic syndrome in the group of epileptic encephalopathy secondary to different, but mostly genetic, etiologies. Show more

Keywords: Angelman syndrome, epileptic encephalopathy, myoclonic seizures, negative myoclonia, non-progressive encephalopathy, refractory epilepsy

DOI: 10.3233/PEP-14088

Citation: Journal of Pediatric Epilepsy, vol. 3, no. 3, pp. 173-180, 2014

Authors: Caraballo, Roberto H. | Sebastian, Fortini | Vilte, Carolina | Cersósimo, Ricardo

Article Type: Research Article

Abstract: Here we present an update of the electroclinical features and evolution of patients with epileptic spasms (ES) in clusters without hypsarrhythmia, with or without focal or generalized paroxysmal discharges on the interictal electroencephalography (EEG). Between February 1990 and July 2012, 26 patients met the electroclinical diagnostic criteria of ES in clusters without hypsarrhythmia. The etiology of the ES was unknown in 15 patients and structural in 11. Age at onset of the ES was between 2 and 32 mo, with a mean age of 9 mo. Fifteen patients had seizures before the onset of ES. Focal spikes were observed in …12 patients, bilateral spikes and spikes and waves in nine, multifocal spikes in three, and two patients had a normal EEG. The ictal EEG recording showed diffuse high-amplitude slow waves in 15 patients, diffuse slow waves followed by voltage attenuation in six patients, and diffuse fast rhythms in five. ES resolved in six patients after a mean follow-up of 9 yr. Neuropsychological development has been normal in these six patients. Twenty patients continue with seizures refractory to antiepileptic drugs after a mean follow-up of 13 yr. Two of them were operated; they are seizure free, with a normal neuropsychological development. Of the 18 remaining patients, 10 have severe mental retardation, five have moderate mental retardation, and three have mild mental retardation. All but 14 of them show behavioral disturbances. Our patients represent an epileptic encephalopathy that may be considered a distinct epileptic syndrome rather than a variant of West syndrome. Show more

Keywords: Childhood, clusters, encephalopathy, epileptic spasms, infantile spasms, hypsarrhythmia

DOI: 10.3233/PEP-14089

Citation: Journal of Pediatric Epilepsy, vol. 3, no. 3, pp. 181-188, 2014