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https://www.readbyqxmd.com/read/29036832/neuropathological-comparison-of-adult-onset-and-juvenile-huntington-s-disease-with-cerebellar-atrophy-a-report-of%C3%A2-a%C3%A2-father-and-son
#1
Caitlin S Latimer, Margaret E Flanagan, Patrick J Cimino, Suman Jayadev, Marie Davis, Zachary S Hoffer, Thomas J Montine, Luis F Gonzalez-Cuyar, Thomas D Bird, C Dirk Keene
BACKGROUND: Huntington's disease (HD) is an autosomal dominant neurodegenerative disease caused by a trinucleotide (CAG) repeat expansion in huntingtin (HTT) on chromosome 4. Anticipation can cause longer repeat expansions in children of HD patients. Juvenile Huntington's disease (JHD), defined as HD arising before age 20, accounts for 5-10% of HD cases, with cases arising in the first decade accounting for approximately 1%. Clinically, JHD differs from the predominately choreiform adult onset Huntington's disease (AOHD) with variable presentations, including symptoms such as myoclonus, seizures, Parkinsonism, and cognitive decline...
October 11, 2017: Journal of Huntington's Disease
https://www.readbyqxmd.com/read/29034969/experience-with-mad-on-children-with-epilepsy-in-egypt-after-classic-kd-failure
#2
O F El Rashidy, M F Nassar, Y G El Gendy, S M Deifalla, S Gaballa
BACKGROUND AND AIM: Modified Atkins diet (MAD) is a less restrictive type of ketogenic diet (KD) as compared to the classic one. The aim of this study was to evaluate the impact of 9 months MAD treatment on the growth and seizure control in patients with intractable epilepsy as well as the quality of life (QoL) of their mothers. SUBJECTS AND METHODS: The study included 15 patients with intractable epilepsy who could not tolerate their classic KD management plan...
October 16, 2017: Acta Neurologica Scandinavica
https://www.readbyqxmd.com/read/29034451/cortical-light-scattering-during-interictal-epileptic-spikes-in-frontal-lobe-epilepsy-in-children-a-fast-optical-signal-and-electroencephalographic-study
#3
Mana Manoochehri, Mahdi Mahmoudzadeh, Emilie Bourel-Ponchel, Fabrice Wallois
OBJECTIVE: Interictal epileptic spikes (IES) represent a signature of the transient synchronous and excessive discharge of a large ensemble of cortical heterogeneous neurons. Epilepsy cannot be reduced to a hypersynchronous activation of neurons whose functioning is impaired, resulting on electroencephalogram (EEG) in epileptic seizures or IES. The complex pathophysiological mechanisms require a global approach to the interactions between neural synaptic and nonsynaptic, vascular, and metabolic systems...
October 16, 2017: Epilepsia
https://www.readbyqxmd.com/read/29031192/growth-and-endocrine-function-in-children-with-dravet-syndrome
#4
Krista Eschbach, Sharon Scarbro, Elizabeth Juarez-Colunga, Victoria Allen, Stephanie Hsu, Kelly Knupp
PURPOSE: Dravet syndrome is an intractable childhood epilepsy syndrome most often associated with an SCN1A mutation. In our clinical practice, several patients with Dravet syndrome were noted to have short stature and endocrine dysfunction. This has not been reported in the literature. Our study aim was to describe growth measurements and endocrine abnormalities in children with Dravet syndrome. METHOD: A retrospective chart review was performed at a single institution...
September 29, 2017: Seizure: the Journal of the British Epilepsy Association
https://www.readbyqxmd.com/read/29030525/neurodevelopment-at-age-10-years-of-children-born-28-weeks-with-fetal-growth-restriction
#5
Steven J Korzeniewski, Elizabeth N Allred, Robert M Joseph, Tim Heeren, Karl C K Kuban, T Michael O'Shea, Alan Leviton
OBJECTIVES: We sought to evaluate the relationships between fetal growth restriction (FGR) (both severe and less severe) and assessments of cognitive, academic, and adaptive behavior brain function at age 10 years. METHODS: At age 10 years, the Extremely Low Gestational Age Newborns Cohort Study assessed the cognitive function, academic achievement, social-communicative function, psychiatric symptoms, and overall quality of life of 889 children born before 28 weeks' gestation...
October 13, 2017: Pediatrics
https://www.readbyqxmd.com/read/29029800/surgical-treatment-of-neuronal-glial-tumors-of-mesial-basal-part-of-temporal-lobe-long-term-outcome-and-control-of-epilepsy-in-pediatric-patients
#6
Paweł Daszkiewicz, Paweł Kowalczyk, Marcin Roszkowski
BACKGROUND: Neuronal-glial tumors (ganglioglioma and dysembryoplastic neuroepithelial tumor) are a frequent cause of focal, drug-resistant and epilepsy in children and young adults, that is amenable for surgical treatment. AIM OF PAPER: Assessment of late outcome of surgical treatment and degree of seizure control, as well as prognostic significance of selected clinical factors. MATERIAL AND METHOD: 52 Pediatric patients presenting with epilepsy, lesion of mesio-basal temporal lobe and histologically verified neuronal-glial tumor treated at our facility since 2000-2011...
April 19, 2017: Neurologia i Neurochirurgia Polska
https://www.readbyqxmd.com/read/29028437/stimulants-do-not-increase-the-risk-of-seizure-related-hospitalizations-in-children-with-epilepsy
#7
Xinyue Liu, Paul R Carney, Regina Bussing, Richard Segal, Linda B Cottler, Almut G Winterstein
OBJECTIVE: To evaluate the safety of stimulants in children with epilepsy. METHODS: In a retrospective cohort study based on Medicaid Analytic eXtract billing records from 26 U.S. states from 1999 to 2010, we identified incident stimulant use among children with epilepsy through outpatient encounter claims and pharmacy claims. We established a control group of nonusers and used frequency matching to generate index dates. We followed both cohorts for 12 months and calculated hazard ratios [HRs] of current and former use of stimulants versus no use on the outcome of seizure-related hospitalization using multivariate Cox proportional hazard models...
October 13, 2017: Journal of Child and Adolescent Psychopharmacology
https://www.readbyqxmd.com/read/29027866/magnetic-resonance-imaging-guided-laser-interstitial-thermal-therapy-as-treatment-for-intractable-insular-epilepsy-in-children
#8
M Scott Perry, David J Donahue, Saleem I Malik, Cynthia G Keator, Angel Hernandez, Rohit K Reddy, Freedom F Perkins, Mark R Lee, Dave F Clarke
OBJECTIVE Seizure onset within the insula is increasingly recognized as a cause of intractable epilepsy. Surgery within the insula is difficult, with considerable risks, given the rich vascular supply and location near critical cortex. MRI-guided laser interstitial thermal therapy (LiTT) provides an attractive treatment option for insular epilepsy, allowing direct ablation of abnormal tissue while sparing nearby normal cortex. Herein, the authors describe their experience using this technique in a large cohort of children undergoing treatment of intractable localization-related epilepsy of insular onset...
October 13, 2017: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/29026617/cerebral-vasculitis-and-intracranial-multiple-aneurysms-in-a-child-with-lyme-neuroborreliosis
#9
Elisa Kortela, Jukka Hytönen, Jussi Numminen, Margit Overmyer, Harri Saxen, Jarmo Oksi
Introduction. Lyme borreliosis is a multisystem tick-borne disease caused by Borrelia burgdorferi. Neurological manifestations are reported in up to 15 % of adult patients with Lyme disease, while the frequency among children is higher. The most common manifestations are painful radiculopathy, facial nerve paresis and lymphocytic meningitis. Epileptic seizures and cerebral vasculitis with stroke or aneurysms are very rare complications. Case presentation. We describe a paediatric patient with sensorineural auditory dysfunction, headache, fatigue and epileptic seizures as sequelae of meningoencephalitis/Lyme neuroborreliosis (LNB) caused by B...
April 2017: JMM Case Reports
https://www.readbyqxmd.com/read/29023307/interrater-agreement-of-eeg-interpretation-after-pediatric-cardiac-arrest-using-standardized-critical-care-eeg-terminology
#10
Nicholas S Abend, Shavonne L Massey, Mark Fitzgerald, France Fung, Natalie J Atkin, Rui Xiao, Alexis A Topjian
PURPOSE: We evaluated interrater agreement of EEG interpretation in a cohort of critically ill children resuscitated after cardiac arrest using standardized EEG terminology. METHODS: Four pediatric electroencephalographers scored 10-minute EEG segments from 72 consecutive children obtained 24 hours after return of circulation using the American Clinical Neurophysiology Society's (ACNS) Standardized Critical Care EEG terminology. The percent of perfect agreement and the kappa coefficient were calculated for each of the standardized EEG variables and a predetermined composite EEG background category...
October 10, 2017: Journal of Clinical Neurophysiology: Official Publication of the American Electroencephalographic Society
https://www.readbyqxmd.com/read/29019723/accidental-hanging-injuries-in-children-recognition-and-management
#11
Siba P Paul, Ruby Paul, Paul A Heaton
Accidental hanging is rare in childhood but is often fatal. Window blind cords pose a particular and unique risk to young children in the UK, accounting for one to two deaths annually. These accidents are frequently associated with non-adherence to the safety instructions provided by the manufacturers. Early discovery of the child and initiation of effective cardiopulmonary resuscitation at the site of the incident are likely to improve the outcome. Prolonged suspension, children who are pulseless at first contact by the emergency paramedic responder, and patients with prolonged periods of remaining in asystole before return of spontaneous circulation after starting cardiopulmonary resuscitation are unlikely to have intact neurological survival...
October 2, 2017: British Journal of Hospital Medicine
https://www.readbyqxmd.com/read/29018634/detection-of-paroxysms-in-long-term-single-channel-eeg-monitoring-of-patients-with-typical-absence-seizures
#12
Troels W Kjaer, Helge B D Sorensen, Sabine Groenborg, Charlotte R Pedersen, Jonas Duun-Henriksen
Absence seizures are associated with generalized 2.5-5 Hz spike-wave discharges in the electroencephalogram (EEG). Rarely are patients, parents, or physicians aware of the duration or incidence of seizures. Six patients were monitored with a portable EEG-device over four times 24 h to evaluate how easily outpatients are monitored and how well an automatic seizure detection algorithm can identify the absences. Based on patient-specific modeling, we achieved a sensitivity of 98.4% with only 0.23 false detections per hour...
2017: IEEE Journal of Translational Engineering in Health and Medicine
https://www.readbyqxmd.com/read/29017522/analysis-of-plasma-multiplex-cytokines-and-increased-level-of-il-10-and-il-1ra-cytokines-in-febrile-seizures
#13
Kyungmin Kim, Byungok Kwak, Aram Kwon, Jongseok Ha, Soojin Kim, Sunwhan Bae, Jaesung Son, Soonyung Kim, Ran Lee
BACKGROUND: Febrile seizures are the most common form of childhood seizures. Fever generation involves many cytokines, including both pro- and anti-inflammatory cytokines. Some of these cytokines also induce febrile seizures. We compared cytokine production in children with a fever alone (healthy control group) and febrile seizure children group. Also, we evaluated the cytokine level of children with a fever alone and febrile seizure history. METHODS: Fifty febrile seizure patients and 39 normal control patients who visited the emergency department of Konkuk University Hospital from December 2015 to December 2016 were included in this study...
October 10, 2017: Journal of Neuroinflammation
https://www.readbyqxmd.com/read/29017283/reduced-severity-of-pertussis-in-persons-with-age-appropriate-pertussis-vaccination-united-states-2010-2012
#14
Lucy A McNamara, Tami Skoff, Amanda Faulkner, Lisa Miller, Kathy Kudish, Cynthia Kenyon, Marisa Bargsten, Shelley Zansky, Amy D Sullivan, Stacey Martin, Elizabeth Briere
Background: In 2012, >48000 pertussis cases were reported in the United States. Many cases occurred in vaccinated persons, showing that pertussis vaccination does not prevent all pertussis cases. However, pertussis vaccination may have an impact on disease severity. Methods: We analyzed data on probable and confirmed pertussis cases reported through Enhanced Pertussis Surveillance (Emerging Infections Program Network) between 2010 and 2012. Surveillance data were collected through physician and patient interview and vaccine registries...
September 1, 2017: Clinical Infectious Diseases: An Official Publication of the Infectious Diseases Society of America
https://www.readbyqxmd.com/read/29017268/the-spectrum-and-burden-of-influenza-associated-neurological-disease-in-children-combined-encephalitis-and-influenza-sentinel-site-surveillance-from-australia-2013-2015
#15
Philip N Britton, Christopher C Blyth, Kristine Macartney, Russell C Dale, Jean Li-Kim-Moy, Gulam Khandaker, Nigel W Crawford, Helen Marshall, Julia E Clark, Elizabeth J Elliott, Robert Booy, Allen C Cheng, Cheryl A Jones
Background: There are few longitudinal studies of seasonal influenza-associated neurological disease (IAND) and none from the Southern Hemisphere. Methods: We extracted prospectively acquired Australian surveillance data from 2 studies nested within the Paediatric Active Enhanced Disease Surveillance (PAEDS) network: the Influenza Complications Alert Network (FluCAN) study and the Australian Childhood Encephalitis (ACE) study between 2013 and 2015. We described the clinical features and severity of IAND in children, including influenza-associated encephalitis/encephalopathy (IAE)...
August 15, 2017: Clinical Infectious Diseases: An Official Publication of the Infectious Diseases Society of America
https://www.readbyqxmd.com/read/29017082/temporal-lobe-surgery-for-intractable-epilepsy-in-children-what-to-do-with-the-hippocampus
#16
Mony Benifla, Odeya Bennet-Back, Zamir Shorer, Iris Noyman, Rima Bar-Yosef, Dana Ekstein
PURPOSE: Resection of the hippocampus can cause verbal memory decline, especially in the pediatric population. Thus, preservation of the hippocampus can be crucial for the quality of life of children with intractable temporal lobe epilepsy (TLE) who are candidates for epilepsy surgery. We investigated techniques that determine whether the hippocampus is part of the epileptogenic zone and the outcomes of pediatric surgery aimed to spare the hippocampus. METHODS: We accessed data of children with normal hippocampus on MRI, who underwent surgery for medically refractory TLE...
October 2, 2017: Seizure: the Journal of the British Epilepsy Association
https://www.readbyqxmd.com/read/29017081/safety-of-intravenous-lacosamide-in-critically-ill-children
#17
Sarah S Welsh, Nan Lin, Alexis A Topjian, Nicholas S Abend
PURPOSE: Acute seizures are common in critically ill children. These patients would benefit from intravenous anti-seizure medications with few adverse effects. We reviewed the usage and effects of intravenous lacosamide in critically ill children with seizures or status epilepticus. METHODS: This retrospective series included consecutive patients who received at least one dose of intravenous lacosamide from April 2011 to February 2016 in the pediatric intensive care unit of a quaternary care children's hospital, including patients with new lacosamide initiation and continuation of outpatient oral lacosamide...
October 3, 2017: Seizure: the Journal of the British Epilepsy Association
https://www.readbyqxmd.com/read/28993435/impact-of-intercurrent-illness-on-calcium-homeostasis-in-children-with-hypoparathyroidism-a-case-series
#18
A Chinoy, M Skae, A Babiker, D Kendall, M Z Mughal, R Padidela
BACKGROUND: Hypoparathyroidism is characterised by hypocalcaemia, and standard management is with an active vitamin D analogue and adequate oral calcium intake (dietary and/or supplements). Little is described in the literature about the impact of intercurrent illnesses on calcium homeostasis in children with hypoparathyroidism. METHODS: We describe three children with hypoparathyroidism in whom intercurrent illnesses led to hypocalcaemia and escalation of treatment with alfacalcidol (1-hydroxycholecalciferol) and calcium supplements...
November 2017: Endocrine Connections
https://www.readbyqxmd.com/read/28992560/effectiveness-and-tolerability-of-perampanel-in-children-adolescents-and-young-adults-with-refractory-epilepsy-a-uk-national-multicentre-study
#19
N Swiderska, H J Tan, A Rajai, A Silwal, A Desurkar, T Martland
PURPOSE: Perampanel is one of the latest antiepileptic drugs (AEDs) approved for the treatment of focal and generalised epilepsy in individuals with epilepsy aged 12 years and older. There is sparse data on the use of Perampanel in children under 12. We conducted a study amongst paediatric neurologists in the United Kingdom to investigate its effectiveness and tolerability as an adjunctive therapy in children of all ages with refractory epilepsy. METHODS: Data was collected via an online questionnaire sent to paediatric neurologists in the UK...
September 14, 2017: Seizure: the Journal of the British Epilepsy Association
https://www.readbyqxmd.com/read/28991830/development-and-validation-of-an-empiric-tool-to-predict-favorable-neurologic-outcomes-among-picu-patients
#20
Punkaj Gupta, Mallikarjuna Rettiganti, Jeffrey M Gossett, Jennifer Daufeldt, Tom B Rice, Randall C Wetzel
OBJECTIVES: To create a novel tool to predict favorable neurologic outcomes during ICU stay among children with critical illness. DESIGN: Logistic regression models using adaptive lasso methodology were used to identify independent factors associated with favorable neurologic outcomes. A mixed effects logistic regression model was used to create the final prediction model including all predictors selected from the lasso model. Model validation was performed using a 10-fold internal cross-validation approach...
October 6, 2017: Critical Care Medicine
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