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Gauthey M.,University of Geneva | Poloni C.B.,University of Lausanne | Ramelli G.-P.,Pediatric Neurology | Roulet-Perez E.,University of Lausanne | Korff C.M.,University of Geneva
Epilepsia | Year: 2010

Epilepsy is frequent in fragile X syndrome (FXS), the most common cause of inherited mental retardation. Status epilepticus (SE), however, seems exceptional in FXS, particularly as an initial epileptic manifestation. To our knowledge, SE was reported in only four FXS patients. We report the clinical features and electroencephalography (EEG) findings of five children with FXS, who presented with SE as their initial seizure. © 2010 International League Against Epilepsy. Source

Gillick B.T.,University of Minnesota | Krach L.E.,University of Minnesota | Feyma T.,Pediatric Neurology | Rich T.L.,Occupational Therapy | And 5 more authors.
Developmental Medicine and Child Neurology | Year: 2014

Aim: The aim of this study was to determine the feasibility and efficacy of five treatments of 6 Hz primed, low-frequency, repetitive transcranial magnetic stimulation (rTMS) combined with constraint-induced movement therapy (CIMT) to promote recovery of the paretic hand in children with congenital hemiparesis. Method: Nineteen children with congenital hemiparesis aged between 8 and 17 years (10 males, nine females; mean age 10 years 10 months, SD 2 years 10 months; Manual Ability Classification Scale levels I-III) underwent five sessions of either real rTMS (n=10) or sham rTMS (n=9) alternated daily with CIMT. CIMT consisted of 13 days of continuous long-arm casting with five skin-check sessions. Each child received a total of 10 hours of one-to-one therapy. The primary outcome measure was the Assisting Hand Assessment (AHA) and the secondary outcome variables were the Canadian Occupational Performance Measure (COPM) and stereognosis. A Wilcoxon signed-rank sum test was used to analyze differences between pre- and post-test scores within the groups. Analysis of covariance was used to compute mean differences between groups adjusting for baseline. Fisher's exact test was used to compare individual change in AHA raw scores with the smallest detectable difference (SDD) of 4 points. Results: All participants receiving treatment finished the study. Improvement in AHA differed significantly between groups (p=0.007). No significant differences in the secondary outcome measures were found. Eight out of 10 participants in the rTMS/CIMT group showed improvement greater than the SDD, but only two out of nine in the sham rTMS/CIMT group showed such improvement (p=0.023). No serious adverse events occurred. Interpretation: Primed, low-frequency rTMS combined with CIMT appears to be safe, feasible, and efficacious in pediatric hemiparesis. Larger clinical trials are now indicated. This article is commented on by Hoare on pages 5-6 of this issue. © 2013 Mac Keith Press. Source

Jehi L.E.,Cleveland Clinic | Irwin A.I.,Cleveland Clinic | Kayyali H.,Pediatric Neurology | Vadera S.,Cleveland Clinic | And 2 more authors.
Epilepsia | Year: 2012

Purpose: To study the prognostic implications of antiepileptic drug (AED) use on seizure freedom following temporal lobe resections for intractable epilepsy. Methods: Seizure outcome implications of epilepsy characteristics and AED use were studied in patients who underwent temporal lobectomy patients at the Cleveland Clinic between September 1995 and December 2006. Survival analysis and multivariate regression with Cox proportional hazard modeling were used. Complete seizure freedom was defined as a favorable outcome. Key Findings: Records of 312 patients were analyzed (mean ± standard deviation follow-up 3.5 ± 1.7 years). The estimated probability of complete seizure freedom was 69% at 12 months (95% confidence interval [CI] 66-72%), and 48% at 36 months (95% CI 45-52%). The mean number of AEDs used per patient at the time of surgery was 1.78 (range 1-4), dropping to 1.02 at last follow-up (range 0-4). Following multivariate analysis, a lower preoperative seizure frequency and perioperative use of levetiracetam predicted a favorable outcome (risk ratio [RR] 0.62, 95% CI 0.43-0.89, and RR = 0.57, 95% CI 0.39-0.83, respectively), whereas nonspecific pathology (RR 1.71, 95% CI 1.15-2.47) and a higher number of AEDs used at the time of surgery correlated with higher rates of seizure recurrence (whole-model log-rank test p-value < 0.0001). Better outcomes within the levetiracetam group were seen despite a higher proportion of several poor prognostic indicators within this patient group, and started as early as 4 months after surgery, gradually increasing to a 15-20% survival advantage by 5 years. No similar outcome correlations were identified with another AED. Significance: AED use may be a potential new modifiable seizure-outcome predictor after temporal lobectomy. This possible prognostic indicator is discussed in light of proposed seizure recurrence mechanisms. © Wiley Periodicals, Inc. © 2012 International League Against Epilepsy. Source

Abend N.S.,Childrens Hospital of Philadelphia | Chapman K.E.,University of Colorado at Denver | Gallentine W.B.,Duke University | Goldstein J.,Northwestern University | And 5 more authors.
Current Neurology and Neuroscience Reports | Year: 2013

Continuous electroencephalographic (CEEG) monitoring is used with increasing frequency in critically ill children to provide insight into brain function and to identify electrographic seizures. CEEG monitoring use often impacts clinical management, most often by identifying electrographic seizures and status epilepticus. Most electrographic seizures have no clinical correlate, and thus would not be identified without CEEG monitoring. There are increasing data showing that electrographic seizures and electrographic status epilepticus are associated with worse outcome. Seizure identification efficiency may be improved by further development of quantitative electroencephalography trends. This review describes the clinical impact of CEEG data, the epidemiology of electrographic seizures and status epilepticus, the impact of electrographic seizures on outcome, the utility of quantitative electroencephalographic trends for seizure identification, and practical considerations regarding CEEG monitoring. © 2013 Springer Science+Business Media New York. Source

Fluss J.,Pediatric Neurology | Ferey S.,Pediatric Radiology | Menache-Starobinski C.,Pediatric Neurology | Delavelle J.,University of Geneva | And 2 more authors.
European Journal of Paediatric Neurology | Year: 2010

Mild influenza-associated encephalitis/encephalopathy with a reversible splenial lesion is a rare entity recently reported almost exclusively in Asiatic individuals. Hallmarks of this clinical-radiological syndrome include severe encephalopathy at onset, prompt and complete recovery, minimal to absent pleocytosis and rapidly reversible involvement of the splenium of the corpus callosum. We report herein a young Caucasian child who in addition had cerebellar involvement and presented a transient mutism during the recovery phase. © 2009 European Paediatric Neurology Society. Source

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