Capovilla G.,Epilepsy Center
Epileptic Disorders | Year: 2011
Non-epileptic attacks represent a heterogeneous group of clinical entities which frequently pose a challenge for the differential diagnosis of epilepsy. This is particularly the case when motor manifestations are the main clinical features. For the large majority of patients, such motor manifestations have a benign course. A correct diagnosis is important to avoid inappropriate investigations, unnecessary therapy, and parental anxiety. Here, a previously unreported form of non-epileptic attacks with infantile onset is described which is different from all subtypes of Fejerman syndrome and does not appear to be uncommon. Our series includes 23 patients with an age at onset of the paroxysmal events ranging from 3 to 8 months. The characteristic feature is side-to-side shaking movements of the trunk and limbs. Surprisingly, urinary infection is often a false diagnosis. Home video recording is particularly helpful in recognising the nature of these episodes once their existence is known. © 2011 John Libbey Eurotext and Springer.
Striano P.,University of Genoa |
Capovilla G.,Epilepsy Center
Current Neurology and Neuroscience Reports | Year: 2013
Epileptic encephalopathy with continuous spikes and waves during slow sleep (EECSWSS) is an age-related childhood condition characterized by epilepsy, cognitive or behavioral impairment, and electroencephalographic abnormality of continuous spike-wave discharges during slow sleep. Continuous spikes and waves during slow sleep (or electrical status epilepticus during sleep) is an electrographic pattern characterized by nearly continuous spike-wave discharges during non-REM sleep, with a frequency of 1.5-3 Hz and usually diffuse and bilateral in distribution. Most authors consider EECSWSS as wide spectrum of epileptic conditions of different origin associated with heterogeneous clinical manifestations and neuropsychological impairment of different severity in close temporal concordance with the appearance of the electroencephalographic pattern of electrical status epilepticus during sleep. The long-term prognosis of this condition is overall poor owing to the persistence of neuropsychological impairment. Therefore, early recognition and effective therapy are necessary to improve long-term prognosis. © 2013 Springer Science+Business Media New York.
Berg A.T.,Epilepsy Center |
Plioplys S.,Childrens Memorial Hospital
Epilepsy and Behavior | Year: 2012
Increasingly, there has been an interest in the association between epilepsy and autism. The high frequency of autism in some of the early-onset developmental encephalopathic epilepsies is frequently cited as evidence of the relationship between autism and epilepsy. While these specific forms of epilepsy carry a higher-than-expected risk of autism, most, if not all, of the association may be due to intellectual disability (ID). The high prevalence of interictal EEG discharges in children with autism is also cited as further evidence although errors in the diagnosis of epilepsy seem to account for at least part of those findings. The prevalence of ID is substantially elevated in children with either epilepsy or autism. In the absence of ID, there is little evidence of a substantial, if any, increased risk of autism in children with epilepsy. Further, although the reported prevalence of autism has increased over the last several years, much of this increase may be attributable to changes in diagnostic practices, conceptualization of autism in the presence of ID, and laws requiring provision of services for children with autism. In the context of these temporal trends, any further efforts to tease apart the relationships between epilepsy, ID, and autism will have to address head-on the accuracy of diagnosis of all three conditions before we can determine whether there is, indeed, a special relationship between autism and epilepsy. © 2012 Elsevier Inc.
Berg A.T.,Epilepsy Center |
Berg A.T.,Northwestern University |
Levy S.R.,Yale University |
Testa F.M.,Yale University |
Blumenfeld H.,Yale University
Epilepsia | Year: 2014
Objective Examine the possible association between long-term seizure outcome in childhood absence epilepsy (CAE) and the initial treatment choice. Methods Children with CAE were prospectively recruited at initial diagnosis and followed in a community-based cohort study. Children presenting with convulsive seizures, significant imaging abnormalities, or who were followed <5 years were excluded. Early outcomes included success of initial medication, early remission, and pharmacoresistance. The primary long-term outcome was complete remission: ≥5 years both seizure free and medication free. Survival methods were used for analyses. Results The first medication was ethosuximde (ESM) in 41 (69%) and valproic acid (VPA) in 18 (31%). Initial success rates were 59% (ESM) and 56% (VPA). Early remission and pharmacoresistance were similar in each group. Apart from atypical electroencephalography (EEG) features (61% [VPA], 17% [ESM]), no clinical features varied substantially between the treatment groups. Complete remission occurred in 31 children (76%) treated with ESM and 7 (39%) who received VPA (p = 0.007). Children with versus without atypical EEG features were less likely to enter complete remission (50% vs. 71%, p = 0.03). In a Cox regression, ESM was associated with a higher rate of complete remission than VPA (hazards ratio [HR] 2.5, 95% confidence interval [CI] 1.1-6.0; p = 0.03). Atypical EEG features did not independently predict outcome (p = 0.15). Five-year and 10-year remission, regardless of continued treatment, occurred more often in children initially treated with ESM versus VPA. Significance These findings are congruent with results of studies in genetic absence models in rats and provide preliminary evidence motivating a hypothesis regarding potential disease-modifying effects of ESM in CAE. A PowerPoint slide summarizing this article is available for download in the Supporting Information section here. © 2014 International League Against Epilepsy.
Berg A.T.,Epilepsy Center
Epilepsia | Year: 2012
The Epidemiology Special Interest Group held at the 2012 American Epilepsy Society meeting was dedicated to a discussion of the concepts and terminology needed for effective communication about the causes of epilepsy. Diverse perspectives were represented ranging from needs in a developing country to those in a tertiary surgical center. The solutions to these different needs often seem in conflict with each other. Informatics is introduced as a discipline that may provide the necessary tools to construct an internally consistent conceptual and terminological system responsive to these many different settings and purposes and able to accommodate changes over time. © 2012 International League Against Epilepsy.