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Pediatric encephalopathy

Raquel Núñez-Ramos, Soledad Montoro, Marcello Bellusci, María Rosa Del Fresno-Valencia, Marta Germán-Díaz, Pedro Urruzuno, Enrique Medina, Javier Manzanares
PURPOSE: The aims of this study were to analyze the characteristics of patients with acute liver failure (ALF) in our center and evaluate the prognostic value of the Pediatric End-Stage Liver Disease (PELD) score calculated at admission. PATIENTS AND METHODS: A retrospective analysis of patients with ALF younger than 15 years between 2005 and 2013 was performed. Information collected included age, sex, etiology of ALF, laboratory tests, PELD score, stage of encephalopathy, and need for liver support devices such as MARS and/or liver transplant (LT) and survival...
September 30, 2016: Pediatric Emergency Care
Kenneth Habetz, Raghu Ramakrishnaiah, Sunil Kumar Raina, Ryan T Fitzgerald, Archana Hinduja
BACKGROUND: Posterior reversible encephalopathy syndrome (PRES) is an acute neurotoxic syndrome that, although characteristically reversible, can result in long-term disability. Our aim was to identify the clinical and radiological factors that are unique to children with PRES compared with adults with the syndrome in a single center. METHODS: We retrospectively reviewed the clinical and radiological records of all patients with PRES admitted at a tertiary care medical center from 2007 to 2014...
September 13, 2016: Pediatric Neurology
Wanli Li, Xinjiang An, Mingyu Fu, Chunli Li
Pneumonia refers to lung inflammation caused by different pathogens or other factors, and is a common pediatric disease occurring in infants and young children. It is closely related to the anatomical and physiological characteristics of infants and young children and is more frequent during winter and spring, or sudden changes in temperature. Pneumonia is a serious disease that poses a threat to children's health and its morbidity and mortality rank first, accounting for 24.5-65.2% of pediatric inpatients...
October 2016: Experimental and Therapeutic Medicine
Abdelrahim Abdrabou Sadek, Mostafa Ashry Mohamed, Ashraf Abou-Taleb, Marwa Ibrahim Mohammed
INTRODUCTION: Acute disseminated encephalomyelitis (ADEM) is an immune mediated disease of the brain. Although it occurs in all ages, most reported cases are in children and adolescents. The aims of this study were to study the clinical pattern and outcome of ADEM in children in a tertiary center in Upper Egypt and to determine the effect of combined use of steroids and IVIg on outcome. METHODS: This observational study was carried out from January 2014 through December 2014 in the Pediatric Department of Sohag University Hospital (Egypt)...
July 2016: Electronic Physician
Rose M Ayoob, Andrew L Schwaderer
The most common acute glomerulonephritis in children is poststreptococcal glomerulonephritis (PSGN) usually occurring between 3 and 12 years old. Hypertension and gross hematuria are common presenting symptoms. Most PSGN patients do not experience complications, but rapidly progressive glomerulonephritis and hypertensive encephalopathy have been reported. This paper reports 17 patients seen in 1 year for PSGN including 4 with atypical PSGN, at a pediatric tertiary care center. Seventeen children (11 males), mean age of 8 years, were analyzed...
2016: International Journal of Nephrology
Jun-Ichi Kawada, Yusuke Okuno, Yuka Torii, Ryo Okada, Satoshi Hayano, Shotaro Ando, Yasuko Kamiya, Seiji Kojima, Yoshinori Ito
Acute encephalitis/encephalopathy is a severe neurological syndrome that is occasionally associated with viral infection. Comprehensive virus detection assays are desirable because viral pathogens have not been identified in many cases. We evaluated the utility of next-generation sequencing (NGS) for detecting viruses in clinical samples of encephalitis/encephalopathy patients. We first determined the sensitivity and quantitative performance of NGS by comparing the NGS-determined number of sequences of human herpesvirus-6 (HHV-6) in clinical serum samples with the HHV-6 load measured using real-time PCR...
2016: Scientific Reports
Sriram Pothapregada, Banupriya Kamalakannan, Mahalaskhmy Thulasingham, Srinivasan Sampath
OBJECTIVE: To study the clinical profile and outcome of dengue fever in children at a tertiary care hospital in Puducherry. MATERIALS AND METHODS: All children (0-12 years of age) diagnosed and confirmed as dengue fever from August 2012 to January 2015 were reviewed retrospectively from hospital case records as per the revised World Health Organization guidelines for dengue fever. The diagnosis was confirmed by NS1 antigen-based ELISA test or dengue serology for IgM and IgG antibodies, and the data were analyzed using SPSS 16...
July 2016: Journal of Global Infectious Diseases
Kelly G Knupp, Erin Leister, Jason Coryell, Katherine C Nickels, Nicole Ryan, Elizabeth Juarez-Colunga, William D Gaillard, John R Mytinger, Anne T Berg, John Millichap, Douglas R Nordli, Sucheta Joshi, Renée A Shellhaas, Tobias Loddenkemper, Dennis Dlugos, Elaine Wirrell, Joseph Sullivan, Adam L Hartman, Eric H Kossoff, Zachary M Grinspan, Lorie Hamikawa
OBJECTIVE: Infantile spasms (IS) represent a severe epileptic encephalopathy presenting in the first 2 years of life. Recommended first-line therapies (hormonal therapy or vigabatrin) often fail. We evaluated response to second treatment for IS in children in whom the initial therapy failed to produce both clinical remission and electrographic resolution of hypsarhythmia and whether time to treatment was related to outcome. METHODS: The National Infantile Spasms Consortium established a multicenter, prospective database enrolling infants with new diagnosis of IS...
September 12, 2016: Epilepsia
Min Jeong Han, Soon Chul Kim, Chan Uhng Joo, Sun Jun Kim
RATIONALE FOR THIS CASE REPORT: Cerebral Salt-Wasting Syndrome (CSWS) is characterized by hyponatremia and sodium wasting in the urine. These conditions are triggered by various neurosurgical disorders such as subarachnoid hemorrhage, brain tumor, head injury, and brain surgery. To our knowledge, CSWS caused by Wernicke encephalopathy (WE) has been rarely reported. PRESENTING CONCERNS OF THE PATIENT: A 2-year-old male patient presented to our hospital due to a seizure attack...
September 2016: Medicine (Baltimore)
Rolla Shbarou
The management of early-onset, genetically determined epilepsies is often challenging. First-line anti-epileptic drugs (AEDs) often include phenobarbital, phenytoin, oxcarbazepine, carbamazepine, clonazepam, levetiracetam, and valproic acid. Combinations of medications are used in these patients with often intractable seizures, and they include topiramate, clobazam, felbamate, lacosamide, lamotrigine, rufinamide, vigabatrin, ACTH, oral steroids, and the ketogenic diet. Vagus nerve stimulator therapy offers some relief in selected patients...
October 2016: Current Treatment Options in Neurology
Wen-Xiong Chen, Hong-Sheng Liu, Si-Da Yang, Si-Hui Zeng, Yuan-Yuan Gao, Zhi-Hong Du, Xiao-Jing Li, Hai-Sheng Lin, Hui-Ci Liang, Jian-Ning Mai
OBJECTIVE: To describe clinical features of reversible splenial lesion syndrome (RESLES) in children. METHODS: Retrospectively analyzed clinical features of RESLES in children and compared differences between severe and non-severe group, classified by clinical global impression-scale; summarized clinical features of children with mild encephalitis/encephalopathy with a reversible splenial lesion (MERS) from case series. RESULTS: 16 episodes of RESLES occurring in 15 Chinese children were analyzed, with 13 episodes having MERS and 3 episodes with epilepsy...
November 2016: Brain & Development
Guillaume Mortamet, Manoelle Kossorotoff, Amandine Baptiste, Nathalie Boddaert, Martin Castelle, Philippe Hubert, Fabrice Lesage, Sylvain Renolleau, Mehdi Oualha
BACKGROUND: The authors aimed to collect all brain magnetic resonance imaging (MRI) performed in critically ill children in the authors' medical pediatric intensive care unit over a 2-year period (2012-2013) to (1) describe the findings and (2) assess its contribution on practical patient care. METHODS: This is a single-center and retrospective study. All children without traumatic brain injury who underwent a brain MRI during pediatric intensive care unit stays were included...
September 2, 2016: Journal of Child Neurology
Rodolfo Gonzalez, Milton H Hamblin, Jean-Pyo Lee
In neurological disorders, pathological lesions in the central nervous system (CNS) may be globally dispersed throughout the brain or localized to specific regions. Although native neural stem cells (NSCs) are present in the adult mammalian brain, intrinsic self-repair of injured adult CNS tissue is inadequate or ineffective. The brain's poor regenerative ability may be due to the fact that NSCs are restricted to discrete locations, are few in number, or are surrounded by a microenvironment that does not support neuronal differentiation...
August 15, 2016: CNS & Neurological Disorders Drug Targets
Dongling Dai, Feiqiu Wen, Sixi Liu, Shaoming Zhou
BACKGROUND: Both haemophagocytic lymphohistiocytosis and acute necrotizing encephalopathy are life-threatening condition. It presents major diagnostic difficulties, since it may have a diversity in clinical picture and with many conditions leading to the same clinical presentation. So it is key important to understand the disorders. CASE PRESENTATION: We report a pediatric case of haemophagocytic lymphohistiocytosis with specific presentation which predominantly featured as acute necrotizing encephalopathy of childhood...
2016: Italian Journal of Pediatrics
Theresa N Mann, Kirsten A Donald, Barbara Laughton, Robert P Lamberts, Nelleke G Langerak
AIM: To describe upper limb motor function and level of activity and participation in children with HIV encephalopathy (HIVE) and bilateral lower limb (BLL) spasticity. METHOD: Thirty ambulant children with HIVE and BLL spasticity and 20 typically developing children, between 5 years and 12 years, were recruited. Upper limb motor function was assessed using the Purdue Pegboard and level of activity and participation using the Computer-Adapted Pediatric Evaluation of Disabilities Inventory (PEDI-CAT)...
August 30, 2016: Developmental Medicine and Child Neurology
Daniela Pohl, Gulay Alper, Keith Van Haren, Andrew J Kornberg, Claudia F Lucchinetti, Silvia Tenembaum, Anita L Belman
Acute disseminated encephalomyelitis (ADEM) is an immune-mediated demyelinating CNS disorder with predilection to early childhood. ADEM is generally considered a monophasic disease. However, recurrent ADEM has been described and defined as multiphasic disseminated encephalomyelitis. ADEM often occurs postinfectiously, although a causal relationship has never been established. ADEM and multiple sclerosis are currently viewed as distinct entities, generally distinguishable even at disease onset. However, pathologic studies have demonstrated transitional cases of yet unclear significance...
August 30, 2016: Neurology
Fatima Yousif Ismail, Ali Fatemi, Michael V Johnston
BACKGROUND: Neuroplasticity refers to the inherently dynamic biological capacity of the central nervous system (CNS) to undergo maturation, change structurally and functionally in response to experience and to adapt following injury. This malleability is achieved by modulating subsets of genetic, molecular and cellular mechanisms that influence the dynamics of synaptic connections and neural circuitry formation culminating in gain or loss of behavior or function. Neuroplasticity in the healthy developing brain exhibits a heterochronus cortex-specific developmental profile and is heightened during "critical and sensitive periods" of pre and postnatal brain development that enable the construction and consolidation of experience-dependent structural and functional brain connections...
August 9, 2016: European Journal of Paediatric Neurology: EJPN
Lan Huang, Fengyan Zhao, Yi Qu, Li Zhang, Yan Wang, Dezhi Mu
Hypoxic-ischemic encephalopathy (HIE), a serious disease leading to neonatal death, is becoming a key area of pediatric neurological research. Despite remarkable advances in the understanding of HIE, the explicit pathogenesis of HIE is unclear, and well-established treatments are absent. Animal models are usually considered as the first step in the exploration of the underlying disease and in evaluating promising therapeutic interventions. Various animal models of HIE have been developed with distinct characteristics, and it is important to choose an appropriate animal model according to the experimental objectives...
August 25, 2016: Reviews in the Neurosciences
Abeer J Hani, Mohamad A Mikati
In this article, we review the treatment options for the pediatric epileptic encephalopathies and provide an update on the new and emerging therapies targeted at the underlying pathophysiology of many of these syndromes. We illustrate how the identification of the specific genetic and autoimmune causes has made possible the evaluation and development of novel, better targeted therapies, as and at times, avoidance of potentially offending agents.
May 2016: Seminars in Pediatric Neurology
Phillip L Pearl
Vitamin-dependent epilepsies and multiple metabolic epilepsies are amenable to treatment that markedly improves the disease course. Knowledge of these amenably treatable severe pediatric epilepsies allows for early identification, testing, and treatment. These disorders present with various phenotypes, including early onset epileptic encephalopathy (refractory neonatal seizures, early myoclonic encephalopathy, and early infantile epileptic encephalopathy), infantile spasms, or mixed generalized seizure types in infancy, childhood, or even adolescence and adulthood...
May 2016: Seminars in Pediatric Neurology
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