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Pediatric Neurology

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https://www.readbyqxmd.com/read/30075875/hospital-emergency-treatment-of-convulsive-status-epilepticus-comparison-of-pathways-from-ten-pediatric-research-centers
#1
Alejandra Vasquez, Marina Gaínza-Lein, Iván Sánchez Fernández, Nicholas S Abend, Anne Anderson, J Nicholas Brenton, Jessica L Carpenter, Kevin Chapman, Justice Clark, William D Gaillard, Tracy Glauser, Joshua Goldstein, Howard P Goodkin, Yi-Chen Lai, Tobias Loddenkemper, Tiffani L McDonough, Mohamad A Mikati, Anuranjita Nayak, Eric Payne, James Riviello, Dmitry Tchapyjnikov, Alexis A Topjian, Mark S Wainwright, Robert C Tasker
OBJECTIVE: We aimed to evaluate and compare the status epilepticus treatment pathways used by pediatric status epilepticus research group (pSERG) hospitals in the United States and the American Epilepsy Society (AES) status epilepticus guideline. METHODS: We undertook a descriptive analysis of recommended timing, dosing, and medication choices in 10 pSERG hospitals' status epilepticus treatment pathways. RESULTS: One pathway matched the timeline in the AES guideline; nine pathways described more rapid timings...
July 11, 2018: Pediatric Neurology
https://www.readbyqxmd.com/read/30082241/jeavons-syndrome-clinical-features-and-response-to-treatment
#2
Kelsey M Smith, Paul E Youssef, Elaine C Wirrell, Katherine C Nickels, Eric T Payne, Jeffrey W Britton, Cheolsu Shin, Gregory D Cascino, Marc C Patterson, Lily C Wong-Kisiel
BACKGROUND: Jeavons syndrome is an underreported epileptic syndrome characterized by eyelid myoclonia, eyelid closure-induced seizures or electroencephalography paroxysms, and photosensitivity. Drug-resistant epilepsy is common, but the prognostic factors and clinical course leading to drug resistance have not been well characterized. METHODS: We identified 30 patients who met the diagnostic criteria of Jeavons syndrome at a single institution between January 1, 2000 and December 15, 2016...
July 10, 2018: Pediatric Neurology
https://www.readbyqxmd.com/read/30077551/anti-myelin-oligodendrocyte-glycoprotein-antibody-associated-with-gray-matter-predominant-transverse-myelitis-mimicking-acute-flaccid-myelitis-a-presentation-of-two-cases
#3
Cynthia Wang, Ram Narayan, Benjamin Greenberg
BACKGROUND: Anti-myelin oligodendrocyte glycoprotein (MOG) antibody associated disorders frequently manifest as optic neuritis, transverse myelitis, and acute disseminated encephalomyelitis. While their clinical phenotypes overlap with relapsing inflammatory Central nervous system (CNS) conditions such as multiple sclerosis and neuromyelitis optica spectrum disorder, MOG-related syndromes frequently occur in a younger age group. In children, longitudinally extensive transverse myelitis (LETM) is less specific for anti-aquaporin-4 associated neuromyelitis optica spectrum disorder, and has also been reported in pediatric multiple sclerosis, idiopathic transverse myelitis, and acute flaccid myelitis...
July 10, 2018: Pediatric Neurology
https://www.readbyqxmd.com/read/30082242/bizarre-beading-in-a-child-with-stroke
#4
Nupur Parakh, Vinit Baliyan, Puneet Jain, Suvasini Sharma, Atin Kumar
No abstract text is available yet for this article.
July 4, 2018: Pediatric Neurology
https://www.readbyqxmd.com/read/30078644/use-of-flow-cytometry-for-diagnosis-of-epilepsy-associated-with-homozygous-pigw-variants
#5
Gretchen Kissel Foskett, Edgar Engleman, Jenna Klotz, Okmi Choi, Lorna Tolentino, Aaina Kochhar, Qian Zhou Yang, David A Stevenson
BACKGROUND: Biallelic variants in PIGW have been suggested to cause infantile spasms and hyperphosphatasia. PIGW encodes for a protein involved in the third step of glycosylphosphatidylinositol (GPI) synthesis. GPI anchored proteins are increasingly recognized as important structures for cellular interactions and neuronal development. METHODS: Molecular testing of PIGW was performed followed by fluorescence activating cell sorting analysis of granulocytes, lymphocytes, and monocytes, and compared to controls...
June 5, 2018: Pediatric Neurology
https://www.readbyqxmd.com/read/30054195/decreasing-seizure-treatment-time-through-quality-improvement-reduces-critical-care-utilization
#6
Adam P Ostendorf, Kelsey Merison, T Arthur Wheeler, Anup D Patel
BACKGROUND: Rapid, effective treatment for status epilepticus reduces associated morbidity and mortality, yet medication delivery remains slow in many hospitalized patients. We utilized quality improvement (QI) methodology to improve treatment times for hospitalized children with status epilepticus. We hypothesized rapid initial seizure treatment would decrease seizure morbidity. METHODS: We utilized QI and statistical process control analysis in a nonintensive care setting within a tertiary care pediatric hospital...
June 2, 2018: Pediatric Neurology
https://www.readbyqxmd.com/read/30072240/dramatic-improvement-of-a-rare-syndrome-with-high-dose-riboflavin-treatment
#7
Eva B Forman, A Reghan Foley, Mary D King
No abstract text is available yet for this article.
June 1, 2018: Pediatric Neurology
https://www.readbyqxmd.com/read/30089552/cockayne-syndrome-complicated-by-moyamoya-vasculopathy-and-stroke
#8
Robert C Stowe, Andres Jimenez-Gomez, Alfred Balasa, Gary D Clark
No abstract text is available yet for this article.
May 18, 2018: Pediatric Neurology
https://www.readbyqxmd.com/read/30049426/sedation-and-analgesia-influence-electroencephalography-monitoring-in-pediatric-neurocritical-care
#9
Giulia M Benedetti, Faye S Silverstein, Stephanie M Rau, Shannon G Lester, Marco H Benedetti, Renée A Shellhaas
OBJECTIVES: We assessed neuroactive medication use in critically ill children who require neurological consultation and evaluated the associations between administration of these medications and continuous electroencephalography (cEEG) utilization and seizure frequency. METHODS: We evaluated exposure to sedatives, analgesics, anesthetics, and paralytics in consecutive patients (0 days to 18 years) for whom neurological consultation was requested in three intensive care units (ICUs) [neonatal (NICU), pediatric (PICU), and cardiothoracic (PCTU)]) at one children's hospital...
May 18, 2018: Pediatric Neurology
https://www.readbyqxmd.com/read/30049425/home-based-therapist-assisted-therapy-for-young-children-with-primary-complex-motor-stereotypies
#10
Harvey S Singer, Shreenath Rajendran, H Richard Waranch, E Mark Mahone
BACKGROUND: Complex motor stereotypies (CMS) typically begin before age three years and include rhythmic, repetitive, fixed movements that last for seconds to minutes and can be interrupted with distraction. OBJECTIVE: We evaluated the effectiveness of a home-based, parent-provided therapy accompanied by scheduled telephone calls with a therapist, in five- to seven-year old children with primary CMS. METHODS: Eligible families received an instructional digital versatile disk (DVD) written instructions, and scheduled telephone contacts with a therapist at baseline (DVD receipt), one, three, and eight weeks later...
May 18, 2018: Pediatric Neurology
https://www.readbyqxmd.com/read/29909138/narcolepsy-and-hypothalamic-region-tumors-presentation-and-evolution
#11
Alexander G Weil, Katherine Muir, Juliette Hukin, Alex Desautels, Véronique Martel, Sébastien Perreault
OBJECTIVE: Because most cases of brain tumor-associated narcolepsy have been published in the form of case reports or small series, the clinical presentation and evolution have not been well described. We sought to better define the epidemiology, etiology, and outcome of brain tumor-related narcolepsy. METHODS: We conducted an extensive review of the literature to identify cases of narcolepsy associated with brain tumors. Only cases of brain tumors involving the hypothalamic region including the suprasellar, sellar, and thalamus were included in this study...
July 2018: Pediatric Neurology
https://www.readbyqxmd.com/read/29887039/xenon-combined-with-hypothermia-in-perinatal-hypoxic-ischemic-encephalopathy-a-noble-gas-a-noble-mission
#12
REVIEW
Ashraf R Amer, Dorothy E Oorschot
Perinatal hypoxia-ischemia is a major cause of neonatal morbidity. It generates primary neuronal damage of the neonatal brain and later secondary damage when reperfusion of the ischemic brain tissue causes a surge of oxygen free radicals and inflammation. This post-hypoxic-ischemic brain damage is a leading cause of motor and intellectual disabilities in survivors. Research worldwide has focused on mitigating this injury. Mild or moderate hypothermia is the standard treatment in many centers. However, its benefit is modest and the search for combinatorial effective neuroprotectants continues...
July 2018: Pediatric Neurology
https://www.readbyqxmd.com/read/29886041/outcomes-after-acute-symptomatic-seizures-in-children-admitted-to-a-neonatal-neurocritical-care-service
#13
Hannah C Glass, Adam L Numis, Dawn Gano, Varun Bali, Elizabeth E Rogers
BACKGROUND: Neonatal seizures due to acute brain injury are associated with high rates of death, disability, and epilepsy. Our objective was to examine incidence of and risk factors for epilepsy among survivors of acute symptomatic neonatal seizures who were cared for by a neonatal neurocritical care service. METHODS: Neonates with acute symptomatic seizures who were admitted to UCSF Benioff Children's Hospital Neuro-Intensive Care Nursery from July 2008 to June 2014 were considered for inclusion...
July 2018: Pediatric Neurology
https://www.readbyqxmd.com/read/29880444/systemic-lupus-erythematosus-presenting-with-severe-optic-disc-edema
#14
Shashank Shekhar, Hardik Sonani, Jagdish Desai, Riddhiben Patel
No abstract text is available yet for this article.
July 2018: Pediatric Neurology
https://www.readbyqxmd.com/read/29859722/effect-of-risperidone-on-the-motor-and-functional-disability-in-children-with-choreoathetoid-cerebral-palsy
#15
Mahesh Kamate, Nishant Mittal, Deepa Metgud
BACKGROUND: Therapeutic options for management of choreoathetoid cerebral palsy, which is a permanent disorder, are limited. Available medications either have significant side effects or are unsuitable for long-term use. Risperidone has shown promise in the management of chorea and has been found to be safe in children less than five years. METHODS: Children with choreoathetoid cerebral palsy were enrolled after parental consent and given risperidone for six-month period along with standard care...
July 2018: Pediatric Neurology
https://www.readbyqxmd.com/read/29859720/diffusion-weighted-imaging-changes-in-a-child-with-posterior-ischemic-optic-neuropathy
#16
Dana B Harrar, Jessica Solomon, Ankoor S Shah, Jennifer Vaughn, Adam D Durbin, Michael J Rivkin
BACKGROUND: Posterior ischemic optic neuropathy results from ischemia of the retrobulbar aspect of the optic nerve. It presents as acute loss of vision without optic disc swelling. This is rare in children, with only seven cases reported to date. Neuroimaging is frequently used to aid in the diagnosis of acute visual complaints in children; however, none of the cases described to date delineate the neuroimaging findings of this entity in children. METHODS: We retrospectively reviewed the electronic medical record...
July 2018: Pediatric Neurology
https://www.readbyqxmd.com/read/29859719/health-related-quality-of-life-for-patients-with-genetically-determined-leukoencephalopathy
#17
Amytice Mirchi, Félixe Pelletier, Luan T Tran, Stephanie Keller, Nancy Braverman, Davide Tonduti, Adeline Vanderver, Amy Pizzino, Marie-Emmanuelle Dilenge, Chantal Poulin, Michael Shevell, Annette Majnemer, Guillaume Sébire, Myriam Srour, Bradley Osterman, Renée-Myriam Boucher, Michel Vanasse, Elsa Rossignol, John Mitchell, Sunita Venkateswaran, Daniela Pohl, Marcelo Kauffman, Raphael Schiffmann, Cyril Goizet, Sebastien Moutton, Federico Roncarolo, Geneviève Bernard
BACKGROUND: We attempted to characterize the health-related quality of life in patients with genetically determined leukoencephalopathies as it relates to the severity of clinical features and the presence or absence of a precise molecular diagnosis. METHODS: Health-related quality of life was assessed using the Pediatric Quality of Life Inventory model (Pediatric Quality of Life Inventory 4.0 Self- and Proxy-reports) on 59 patients diagnosed with genetically determined leukoencephalopathies...
July 2018: Pediatric Neurology
https://www.readbyqxmd.com/read/29859718/intermittent-erythema-in-guillain-barr%C3%A3-syndrome
#18
Kouki Tomari, Hiromi Suzuki, Sahoko Miyama
No abstract text is available yet for this article.
July 2018: Pediatric Neurology
https://www.readbyqxmd.com/read/29853249/a-neurological-disorder-of-the-thalamus-mimicking-low-grade-glioma
#19
Katherine Longardner, Denise Malicki, John Crawford
No abstract text is available yet for this article.
July 2018: Pediatric Neurology
https://www.readbyqxmd.com/read/29803545/a-multidisciplinary-consensus-for-clinical-care-and-research-needs-for-sturge-weber-syndrome
#20
REVIEW
Alejandro J De la Torre, Aimee F Luat, Csaba Juhász, Mai Lan Ho, Davis P Argersinger, Kara M Cavuoto, Mabel Enriquez-Algeciras, Stephanie Tikkanen, Paula North, Craig N Burkhart, Harry T Chugani, Karen L Ball, Anna Lecticia Pinto, Jeffrey A Loeb
BACKGROUND: Sturge-Weber syndrome is a neurocutaneous disorder associated with port-wine birthmark, leptomeningeal capillary malformations, and glaucoma. It is associated with an unpredictable clinical course. Because of its rarity and complexity, many physicians are unaware of the disease and its complications. A major focus moving ahead will be to turn knowledge gaps and unmet needs into new research directions. METHODS: On October 1-3, 2017, the Sturge-Weber Foundation assembled clinicians from the Clinical Care Network with patients from the Patient Engagement Network of the Sturge-Weber Foundation to identify our current state of knowledge, knowledge gaps, and unmet needs...
July 2018: Pediatric Neurology
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