Journal Watch

karenskjeiKaren L. Skjei, M.D.
Assistant Professor,
Department of Neurology Co-Director,
Epilepsy Surgery Program Director,
Epilepsy Monitoring Unit,
Norton Children's Hospital

Dr. Karen Skjei is an assistant professor of Neurology and Pediatrics at the University of Louisville. She completed her undergraduate studies at Duke University, and her medical education at the University of California at San Francisco School of Medicine. She completed a residency in Pediatrics and a fellowship in Child and Adolescent Neurology at the Mayo Clinic in Rochester, New York. She completed a fellowship in Neurophysiology and Epilepsy at the Children’s Hospital of Philadelphia. Dr Skjei is currently Editor of the ICNApedia Journal Watch

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ICNA
ICNA
May 26, 2019 893 0
Lancet 2019
Background Phenytoin is the recommended second-line intravenous anticonvulsant for treatment of paediatric convulsive status epilepticus in the UK; however, some evidence suggests that levetiracetam could be an effective and safer alternative. This trial compared the efficacy and safety of phenytoin and levetiracetam for second-line management of paediatric convulsive status epilepticus.
 
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Biju Hameed
Biju Hameed
Updated May 25, 2019 837 0
Neurology 92 (15):e1698-e1708
Liberato and colleagues retrospectively analyzed brain magnetic resonance imaging (MRI) scans for brain lesion volume, Loes score, and growth over time in a cohort of boys biochemically or genetically diagnosed with adrenoleukodystrophy. Demographic data, genotype information, biochemical data, follow-up intervals, and brain lesion characteristics were recorded in all MRI scans. White matter changes were scored using the Loes MRI severity scoring system, and lesions were subdivided into 5 patterns based on primary anatomic distribution.
 
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Biju Hameed
Biju Hameed
Updated May 25, 2019 487 0
Epilepsia 60 (5):872-884
Modest improvements in IQ in children with epilepsy who underwent epilepsy surgery, a finding which was not seen in children with severe epilepsy who did not undergo surgery. 
 
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ICNA
ICNA
Updated June 11, 2018 1455 0
The Lancet May 23, 2018
Paolo Curatolo, David N Franz, John A Lawson, Zuhal Yapici, Hiroko Ikeda, Tilman Polster, Rima Nabbout, Petrus J de Vries, Dennis J Dlugos, Jenna Fan, Antonia Ridolfi, Diana Pelov, Maurizio Voi, Jacqueline A French https://doi.org/10.1016/S2352-4642(18)30099-3 Background Epilepsy occurs in 70–90% of patients with tuberous sclerosis complex. We aimed to assess the efficacy and safety of adjunctive everolimus for treatment-refractory seizures associated with tuberous sclerosis complex in paediatric patients enrolled in the EXIST-3 trial, a double-blind, placebo-controlled, randomised, phase 3 study. Methods This post-hoc analysis focused on paediatric patients...
 
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ICNA
ICNA
Updated July 23, 2018 1713 0
N Engl J Med 2018; 378:1888-1897
BACKGROUND Cannabidiol has been used for treatment-resistant seizures in patients with severe early-onset epilepsy. We investigated the efficacy and safety of cannabidiol added to a regimen of conventional antiepileptic medication to treat drop seizures in patients with the Lennox–Gastaut syndrome, a severe developmental epileptic encephalopathy.  
 
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ICNA
ICNA
November 23, 2017 3193 1
N Engl J Med. 2017 Nov 2;377(18):1723-1732
Findings from a randomized, double-blind, phase 3 efficacy and safety trial published in the New England Journal of Medicine suggest that infants with spinal muscular atrophy who receive nusinersen experience greater survival and motor function improvement compared with controls.
 
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ICNA
ICNA
Updated November 14, 2017 2884 0
JAMA Pediatr. 2017;171(9)
This paper looks at the diagnostic yield of genetic testing when used for children with newly presenting early-life epilepsy. 
 
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ICNA
ICNA
Updated October 24, 2017 2653 0
Neurology. 2017 Apr 25;88(17):1674-1680
OBJECTIVE: To determine the incidence rates of sudden unexpected death in epilepsy (SUDEP) in different epilepsy populations and address the question of whether risk factors for SUDEP have been identified. METHODS: Systematic review of evidence; modified Grading Recommendations Assessment, Development, and Evaluation process for developing conclusions; recommendations developed by consensus.
 
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ICNA
ICNA
Updated October 22, 2017 1713 0
Epilepsia Volume 58, Issue 9 September 2017
To identify potential pharmacokinetic interactions between the pharmaceutical formulation of cannabidiol (CBD; Epidiolex) and the commonly used antiepileptic drugs (AEDs) through an open-label safety study. Serum levels were monitored to identify interactions between CBD and AEDs.
 
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ICNA
ICNA
Updated July 27, 2017 3173 1
Lancet. 2017 Jul 17
Background Duchenne muscular dystrophy (DMD) is a severe, progressive, and rare neuromuscular, X-linked recessive disease. Dystrophin deficiency is the underlying cause of disease; therefore, mutation-specific therapies aimed at restoring dystrophin protein production are being explored. We aimed to assess the efficacy and safety of ataluren in ambulatory boys with nonsense mutation DMD.
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