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Pediatric Neurology

Ryan Gise, Timothy Truong, Afshin Parsikia, Joyce N Mbekeani
No abstract text is available yet for this article.
September 14, 2018: Pediatric Neurology
Amanda J Saraf, Ghada Elhawary, Jonathan L Finlay, Suzanne Scott, Randal Olshefski, Mark Halverson, Daniel R Boue, Mohamed S AbdelBaki
BACKGROUND: Surgical resection is the treatment of choice for pleomorphic xanthoastrocytoma, while chemotherapy and radiation therapy are typically used in patients with anaplasia, metastasis, or sometimes in subtotally resected cases, especially upon recurrence. Extracranial dissemination has been only rarely reported. We describe a five years old boy with the rare occurrence multiply recurrent and extracranially disseminated anaplastic pleomorphic xanthoastrocytoma. A complete resolution of his tumor was achieved for greater than two years thus far after administering everolimus...
September 13, 2018: Pediatric Neurology
Vasco Deon Kidd
No abstract text is available yet for this article.
September 12, 2018: Pediatric Neurology
Nathan Watemberg, Sveta Afunevitz, Esther Ganelin-Cohen, Muhammad Mahajnah
BACKGROUND: Benign epilepsy with centrotemporal spikes (BECTs) usually follows a self-limited course, with a single or few seizures. Assessing the likelihood of a second seizure after the first event may help establishing the need for antiepileptic drug therapy at the time of diagnosis. We aimed at identifying clinical and/or electrographic features that could predict the occurrence of a second seizure. METHODS: We reviewed the clinical and electroencephalographic (EEG) data of children diagnosed with BECTs between 2006 and 2012...
September 5, 2018: Pediatric Neurology
Gillian S Townend, Raymond van de Berg, Laurèl H M de Breet, Monique Hiemstra, Laura Wagter, Eric Smeets, Josine Widdershoven, Herman Kingma, Leopold M G Curfs
BACKGROUND: Individuals with Rett syndrome (RTT) are notoriously reliant on the use of eye gaze as a primary means of communication. Underlying an ability to communicate successfully via eye gaze is a complex matrix of requirements, with an intact oculomotor system being just one element. To date, the underlying neural and motor pathways associated with eye gaze are relatively under-researched in RTT. PURPOSE: This study was undertaken to plug this gap in knowledge and to further the understanding of RTT in one specific area of development and function, namely oculomotor function...
August 30, 2018: Pediatric Neurology
Anna Rubegni, Anna Rita Ferrari, Rosa Pasquariello, Raffaello Canapicchi, Filippo M Santorelli, Claudia Nesti
No abstract text is available yet for this article.
August 24, 2018: Pediatric Neurology
Eliana B Saltzman, Schneider K Rancy, Darryl B Sneag, Joseph H Feinberg Md, Dale J Lange, Scott W Wolfe
BACKGROUND: Acute flaccid myelitis is associated with enterovirus D68 induced inflammation and destruction of cervical anterior horn cells. To date, no medical intervention has altered the disease course. METHODS: We report two pediatric patients who were treated with nerve transfer in three limbs with sustained upper extremity neuropathy. Postoperative outcomes included muscle strength, graded on the British Medical Research Council (BMRC) scale, range of motion, and electromyography...
August 20, 2018: Pediatric Neurology
Debopam Samanta, Raghu Ramakrishnaiah
No abstract text is available yet for this article.
August 16, 2018: Pediatric Neurology
David E Mandelbaum
No abstract text is available yet for this article.
August 16, 2018: Pediatric Neurology
Rachel E Rodin, Christopher A Walsh
No abstract text is available yet for this article.
August 11, 2018: Pediatric Neurology
Anat Cohen, Rajkumar Agarwal, Ahmad Farooqi, Nirupama Kannikeswaran
BACKGROUND: Seizures are a common presenting symptom to the emergency department (ED) in children with intraventricular shunts (IVS). The incidence of shunt malfunction and the yield of imaging studies in children with IVS presenting with seizures is unknown. OBJECTIVES: Weassessed the utility and diagnostic yield of evaluation for shunt malfunction in patients with IVS with seizure and identified clinical predictors for shunt malfunction in these children. METHODS: We performed a retrospective review of children aged zero to 21years with IVS who presented to the ED with seizure between 2011 and 2015...
August 10, 2018: Pediatric Neurology
Michael Shevell
No abstract text is available yet for this article.
August 10, 2018: Pediatric Neurology
Megan Barry, Truc M Le, Melissa C Gindville, Lori C Jordan
BACKGROUND: Pediatric stroke alerts or "code strokes" allow for rapid evaluation, imaging, and treatment of children presenting with stroke-like symptoms. In a previous study of emergency department-initiated pediatric stroke alerts, 24% of children had confirmed strokes. The purpose of this study was to characterize in-hospital pediatric stroke alerts. METHODS: Demographic and clinical information was obtained from a quality improvement database and medical records for children (zero to 20 years) at a single institution for whom a stroke alert was activated after hospital admission between April 2011 and December 2016...
August 10, 2018: Pediatric Neurology
Laurie H Seaver, Steven DeRoos, Nicholas J Andersen, Brad Betz, Jeremy Prokop, Nick Lannen, Renee Jordan, Surender Rajasekaran
BACKGROUND: Infantile epileptic encephalopathy is a heterogeneous condition that has been associated with variants in more than 200 genes. The variability in findings and prognosis creates challenges to making the correct diagnosis and initiating the appropriate therapy. Biallelic variants in NARS2, a mitochondrial aminoacyl-tRNA synthetase gene, were recently associated with neurodegenerative disorders that include epilepsy. METHODS: We describe two infant brothers who presented with focal status epilepticus that progressed to lethal epileptic encephalopathy...
August 4, 2018: Pediatric Neurology
Cydni N Williams, Juan Piantino, Cynthia McEvoy, Nora Fino, Carl O Eriksson
BACKGROUND: Disorders requiring pediatric neurocritical care (PNCC) affect thousands of children annually. We aimed to quantify the burden of PNCC through generation of national estimates of disease incidence, utilization of critical care interventions (CCI), and hospital outcomes. METHODS: We performed a retrospective cohort analysis of the Kids Inpatient Database over three years to evaluate pediatric traumatic brain injury, neuro-infection or inflammatory diseases, status epilepticus, stroke, hypoxic ischemic injury after cardiac arrest, and spinal cord injury...
August 4, 2018: Pediatric Neurology
Rachel L Randell, Ashley V Adams, Heather Van Mater
BACKGROUND: Autoimmune encephalitis can result in significant neurological and psychiatric morbidity and mortality in patients of all ages and often does not respond to standard therapies. Recent reports suggest efficacy of tocilizumab, a monoclonal antibody against interleukin 6, in refractory autoimmune encephalitis. RESULTS: We describe three children with refractory autoimmune encephalitis who experienced a robust, immediate clinical response following treatment with tocilizumab...
August 3, 2018: Pediatric Neurology
Shashank Shekhar, Prashant Natteru, Majid A Khan, Jagdish Desai, Riddhiben Patel
No abstract text is available yet for this article.
July 29, 2018: Pediatric Neurology
Fehime K Eroglu, Fatih Ozaltin, Nazlı Gönç, Hülya Nalçacıoğlu, Z Birsin Özçakar, Dilek Yalnızoğlu, Şafak Güçer, Diclehan Orhan, Fatma Tuba Eminoğlu, Rahşan Göçmen, Ayfer Alikaşifoğlu, Rezan Topaloğlu, Ali Düzova
BACKGROUND: COQ2 mutations cause a rare infantile multisystemic disease with heterogeneous clinical features. Promising results have been reported in response to Coenzyme Q10 treatment, especially for kidney involvement, but little is known about the long-term outcomes. METHODS: We report four new patients from two families with the c.437G→A (p.Ser146Asn) mutation in COQ2 and the outcomes of two patients after long-term coenzyme Q10 treatment. RESULTS: Index cases from two families presented with vomiting, nephrotic range proteinuria, and diabetes in early infancy...
July 27, 2018: Pediatric Neurology
Krystal Hay, MaryAnn Nelin, Helen Carey, Olena Chorna, Melissa Moore-Clingenpeel Ma Mas, Nathalie Maitre
BACKGROUND: The Hammersmith Infant Neurological Examination is one of several useful tools for early identification of cerebral palsy; however, cut-off scores for cerebral palsy do not consistently distinguish infants with hemiplegia from those typically developing. We hypothesized that use of an asymmetry score, in addition to the assessment's standard total cutoff score, could remedy this problem in a clinical setting. METHODS: This retrospective study of a neonatal intensive care follow-up program with consistent clinical use of the Hammersmith Infant Neurological Examination matched infants with a diagnosis of cerebral palsy to infants without motor delays or evidence of neurodevelopmental impairments...
July 25, 2018: Pediatric Neurology
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...
August 2018: Pediatric Neurology
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