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Landau Kleffner

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https://www.readbyqxmd.com/read/27866088/is-ketogenic-diet-treatment-hepatotoxic-for-children-with-intractable-epilepsy
#1
Nur Arslan, Orkide Guzel, Engin Kose, Unsal Yılmaz, Pınar Kuyum, Betül Aksoy, Tansel Çalık
PURPOSE: Long-term ketogenic diet (KD) treatment has been shown to induce liver steatosis and gallstone formation in some in vivo and clinical studies. The aim of this retrospective study was to evaluate the hepatic side effects of KD in epileptic children. METHOD: A total of 141 patients (mean age: 7.1±4.1years [2-18 years], 45.4% girls), receiving KD at least one year for intractable epilepsy due to different diagnoses (congenital brain defects, GLUT-1 deficiency, West syndrome, tuberous sclerosis, hypoxic brain injury, etc...
November 13, 2016: Seizure: the Journal of the British Epilepsy Association
https://www.readbyqxmd.com/read/27544470/the-expanding-clinical-spectrum-of-genetic-pediatric-epileptic-encephalopathies
#2
Rolla Shbarou, Mohamad A Mikati
Pediatric epileptic encephalopathies represent a clinically challenging and often devastating group of disorders that affect children at different stages of infancy and childhood. With the advances in genetic testing and neuroimaging, the etiologies of these epileptic syndromes are now better defined. The various encephalopathies that are reviewed in this article include the following: early infantile epileptic encephalopathy or Ohtahara syndrome, early myoclonic encephalopathy, epilepsy of infancy with migrating focal seizures, West syndrome, severe myoclonic epilepsy in infancy (Dravet syndrome), Landau-Kleffner syndrome, Lennox-Gastaut syndrome, and epileptic encephalopathy with continuous spike-and-wave during sleep...
May 2016: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/27544466/pediatric-epileptic-encephalopathies-pathophysiology-and-animal-models
#3
REVIEW
Li-Rong Shao, Carl E Stafstrom
Epileptic encephalopathies are syndromes in which seizures or interictal epileptiform activity contribute to or exacerbate brain function, beyond that caused by the underlying pathology. These severe epilepsies begin early in life, are associated with poor lifelong outcome, and are resistant to most treatments. Therefore, they represent an immense challenge for families and the medical care system. Furthermore, the pathogenic mechanisms underlying the epileptic encephalopathies are poorly understood, hampering attempts to devise novel treatments...
May 2016: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/27435520/idiopathic-focal-epilepsies-the-lost-tribe
#4
Deb K Pal, Colin Ferrie, Laura Addis, Tomoyuki Akiyama, Giuseppe Capovilla, Roberto Caraballo, Anne de Saint-Martin, Natalio Fejerman, Renzo Guerrini, Khalid Hamandi, Ingo Helbig, Andreas A Ioannides, Katsuhiro Kobayashi, Dennis Lal, Gaetan Lesca, Hiltrud Muhle, Bernd A Neubauer, Tiziana Pisano, Gabrielle Rudolf, Caroline Seegmuller, Takashi Shibata, Anna Smith, Pasquale Striano, Lisa J Strug, Pierre Szepetowski, Thalia Valeta, Harumi Yoshinaga, Michalis Koutroumanidis
The term idiopathic focal epilepsies of childhood (IFE) is not formally recognised by the ILAE in its 2010 revision (Berg et al., 2010), nor are its members and boundaries precisely delineated. The IFEs are amongst the most commonly encountered epilepsy syndromes affecting children. They are fascinating disorders that hold many "treats" for both clinicians and researchers. For example, the IFEs pose many of the most interesting questions central to epileptology: how are functional brain networks involved in the manifestation of epilepsy? What are the shared mechanisms of comorbidity between epilepsy and neurodevelopmental disorders? How do focal EEG discharges impact cognitive functioning? What explains the age-related expression of these syndromes? Why are EEG discharges and seizures so tightly locked to slow-wave sleep? In the last few decades, the clinical symptomatology and the respective courses of many IFEs have been described, although they are still not widely appreciated beyond the specialist community...
September 1, 2016: Epileptic Disorders: International Epilepsy Journal with Videotape
https://www.readbyqxmd.com/read/27355396/neurocognitive-and-behavioral-profiles-of-children-with-landau-kleffner-syndrome
#5
Cynthia A Riccio, Stephanie M Vidrine, Morris J Cohen, Delmaris Acosta-Cotte, Yong Park
This is a retrospective study of 14 cases of children with Landau-Kleffner syndrome (LKS), the most prominent feature of which is acquired aphasia. These children were followed at a tertiary care pediatric epilepsy center. From the research data base, all LKS cases with neuropsychological evaluation were extracted. Children ranged in age from 6 to 13 years (M = 9.12; SD = 2.19) at the time of assessment (1 to 10 years post-onset). The majority of the children were white males, and all but one continued to experience seizure activity...
June 29, 2016: Applied Neuropsychology. Child
https://www.readbyqxmd.com/read/27184947/psychiatric-and-behavioural-disorders-in-children-with-epilepsy-ilae-task-force-report-behavioural-effects-of-epilepsy-surgery
#6
Frank Besag, Rochelle Caplan, Albert Aldenkamp, David W Dunn, Giuseppe Gobbi, Matti Sillanpää
There are relatively few studies of the behavioural outcome of epilepsy surgery in children that have used standardised behavioural measures before and after the procedure. Those investigations that have used such measures are often on mixed groups with mixed pathology, implying that the numbers, when stratified, are very small. They are also often retrospective. Furthermore, because placebo surgery is generally not an option, the studies have been open and they are usually uncontrolled. The few available data suggest that, although individual children may benefit or deteriorate, there is little overall group effect of temporal or extratemporal surgery on behavioural/psychiatric outcome...
May 16, 2016: Epileptic Disorders: International Epilepsy Journal with Videotape
https://www.readbyqxmd.com/read/27184771/psychiatric-and-behavioural-disorders-in-children-with-epilepsy-ilae-task-force-report-subtle-behavioural-and-cognitive-manifestations-of-epilepsy-in-children
#7
Frank Besag, Giuseppe Gobbi, Albert Aldenkamp, Rochelle Caplan, David W Dunn, Matti Sillanpää
A subtle behavioural or cognitive manifestation of epilepsy can be defined in two ways. First, epileptiform discharges not presenting as obvious seizures may nevertheless affect cognition and/or behaviour. Second, the actual seizures may be obvious but the way they affect cognition or behaviour may not be. There is a growing body of evidence indicating that the epileptiform discharges in benign epilepsy with centrotemporal spikes can affect behaviour and cognition. The focal discharges in other forms of epilepsy can also be associated with behavioural change...
May 16, 2016: Epileptic Disorders: International Epilepsy Journal with Videotape
https://www.readbyqxmd.com/read/27184676/psychiatric-and-behavioural-disorders-in-children-with-epilepsy-ilae-task-force-report-behavioural-and-psychiatric-disorders-associated-with-childhood-epilepsy-syndromes
#8
Frank Besag, Giuseppe Gobbi, Albert Aldenkamp, Rochelle Caplan, David W Dunn, Matti Sillanpää
The categorisation of the childhood epilepsies into a number of different syndromes has allowed greater insight into the prognosis, not only with regard to seizure control but also in relation to cognitive and behavioural outcome. The role of genetics in determining both the syndrome and the behavioural outcome remains promising, although the promise is still largely unfulfilled. The behavioural/psychiatric outcome of a selection of the large number of childhood epilepsy syndromes is presented. The rate of autism in West syndrome, particularly in children who have tuberous sclerosis with temporal tubers, is high...
May 16, 2016: Epileptic Disorders: International Epilepsy Journal with Videotape
https://www.readbyqxmd.com/read/27184301/psychiatric-and-behavioural-disorders-in-children-with-epilepsy-ilae-task-force-report-epilepsy-and-autism
#9
Frank Besag, Albert Aldenkamp, Rochelle Caplan, David W Dunn, Giuseppe Gobbi, Matti Sillanpää
A high proportion of children with epilepsy have autism spectrum disorder. Although estimates vary, depending both on the population studied and the definitions used, a figure of around 20% has typically been reported. Autism can have a major impact on the life of the child and family. Despite the importance of this comorbidity and although many studies have been performed, a full understanding of the possible links between epilepsy and autism remains elusive. In a minority of cases, for example in the Landau-Kleffner syndrome, the autistic features can be the result of the epilepsy itself...
May 16, 2016: Epileptic Disorders: International Epilepsy Journal with Videotape
https://www.readbyqxmd.com/read/26992889/current-understanding-and-neurobiology-of-epileptic-encephalopathies
#10
REVIEW
Stéphane Auvin, Maria Roberta Cilio, Annamaria Vezzani
Epileptic encephalopathies are a group of diseases in which epileptic activity itself contributes to severe cognitive and behavioral impairments above and beyond what might be expected from the underlying pathology alone. These impairments can worsen over time. This concept has been continually redefined since its introduction. A few syndromes are considered epileptic encephalopathies: early myoclonic encephalopathy and Ohtahara syndrome in the neonatal period, epilepsy of infancy with migrating focal seizures, West syndrome or infantile spasms, Dravet syndrome during infancy, Lennox-Gastaut syndrome, epileptic encephalopathy with continuous spikes-and-waves during sleep, and Landau-Kleffner syndrome during childhood...
August 2016: Neurobiology of Disease
https://www.readbyqxmd.com/read/26806548/response-to-immunotherapy-in-a-patient-with-landau-kleffner-syndrome-and-grin2a-mutation
#11
Nina Fainberg, Amy Harper, Dmitry Tchapyjnikov, Mohamad A Mikati
Landau-Kleffner syndrome (LKS) has been demonstrated in the past to respond to immunotherapy. Recently, some cases of LKS have been shown to be secondary to glutamate receptor (GRIN2A) mutations. Whether such cases respond to immunotherapy is not known. Here, we present the case of a 3-year-old boy with LKS found to have a GRIN2A heterozygous missense mutation, whose clinical symptoms and EEG responded to a course of combination oral steroids and monthly infusions of intravenous immunoglobulin. He then relapsed after discontinuation of this therapy, and responded again after a second course of intravenous immunoglobulin...
March 1, 2016: Epileptic Disorders: International Epilepsy Journal with Videotape
https://www.readbyqxmd.com/read/26776343/syndrome-of-electrical-status-epilepticus-during-sleep-epileptic-encephalopathy-related-to-brain-development
#12
Xiao-Qiao Chen, Wei-Na Zhang, Lin-Yan Hu, Meng-Jia Liu, Li-Ping Zou
BACKGROUND: Epileptic encephalopathy with electrical status epilepticus during sleep is an age-related and self-limited disorder. The present study analyzed the etiology, demographics, and pathogenesis of patients with electrical status epilepticus during sleep to provide information on the diagnosis and therapy of this syndrome. METHODS: The etiologies of epileptic encephalopathy with electrical status epilepticus during sleep in patients admitted in Chinese People's Liberation Army General Hospital from 2009 to 2014 were retrospectively analyzed...
March 2016: Pediatric Neurology
https://www.readbyqxmd.com/read/26627294/landau-kleffner-syndrome
#13
REVIEW
Mia Tuft, Marte Årva, Marit Bjørnvold, John Aage Wilson, Karl O Nakken
No abstract text is available yet for this article.
December 1, 2015: Tidsskrift for Den Norske Lægeforening: Tidsskrift for Praktisk Medicin, Ny Række
https://www.readbyqxmd.com/read/26420702/landau-kleffner-syndrome-an-uncommon-dealt-with-case-in-southeast-asia
#14
Naresh Motwani, Suhaim Afsar, Nachiket S Dixit, Nitin Sharma
An 11-year-old boy was admitted with fever followed by convulsions. He had developed aphasia subsequent to this illness. His birth history was unremarkable, and he had normal growth and development including of language, hearing and vision. His neurological examination was normal except for aphasia. Investigations including cerebrospinal fluid study and MRI were normal. However, EEG was abnormal and the boy was diagnosed as a case of Landau-Kleffner syndrome (LKS) and treated with sodium valproate, levetiracetam and steroids...
2015: BMJ Case Reports
https://www.readbyqxmd.com/read/26337264/outcome-following-multiple-subpial-transection-in-landau-kleffner-syndrome-and-related-regression
#15
Michelle Downes, Rebecca Greenaway, Maria Clark, J Helen Cross, Nicola Jolleff, William Harkness, Marios Kaliakatsos, Stewart Boyd, Steve White, Brian G R Neville
OBJECTIVE: To determine whether multiple subpial transection in the posterior temporal lobe has an impact on long-term outcome in children who have drug-resistant Landau-Kleffner syndrome (LKS) or other "electrical status epilepticus during sleep" (ESES)-related regression. Given the wide variability in outcomes reported in the literature, a secondary aim was to explore predictors of outcome. METHODS: The current study includes a surgery group (n = 14) comprising patients who underwent multiple subpial transection of the posterior temporal lobe and a nonsurgery comparison group (n = 21) comprising patients who underwent presurgical investigations for the procedure, but who did not undergo surgery...
November 2015: Epilepsia
https://www.readbyqxmd.com/read/26232266/evidence-for-normal-letter-sound-integration-but-altered-language-pathways-in-a-case-of-recovered-landau-kleffner-syndrome
#16
Pim Pullens, Will Pullens, Vera Blau, Bettina Sorger, Bernadette M Jansma, Rainer Goebel
Landau-Kleffner Syndrome (LKS) is a rare form of acquired aphasia in children, characterized by epileptic discharges, which occur mostly during sleep. After normal speech and language development, aphasia develops between the ages of 3-7 years in a period ranging from days to months. The epileptic discharges usually disappear after reaching adulthood, but language outcomes are usually poor if no treatment focused on restoration of (non-) verbal communication is given. Patients often appear deaf-mute, but sign language, as part of the treatment, may lead to recovery of communication...
October 2015: Brain and Cognition
https://www.readbyqxmd.com/read/26230617/acetazolamide-for-electrical-status-epilepticus-in-slow-wave-sleep
#17
Anthony L Fine, Elaine C Wirrell, Lily C Wong-Kisiel, Katherine C Nickels
Electrical status epilepticus in slow-wave sleep (ESES) is characterized by nearly continuous spike-wave discharges during non-rapid eye movement (REM) sleep. ESES is present in Landau-Kleffner syndrome (LKS) and continuous spike and wave in slow-wave sleep (CSWS). Sulthiame has demonstrated reduction in spike-wave index (SWI) in ESES, but is not available in the United States. Acetazolamide (AZM) is readily available and has similar pharmacologic properties. Our aims were to assess the effect of AZM on SWI and clinical response in children with LKS and CSWS...
September 2015: Epilepsia
https://www.readbyqxmd.com/read/26179689/landau-kleffner-syndrome-an-acquired-epileptic-aphasia
#18
Hsin-Yu Lo, Der-Shin Ke, Wun-Tsong Chaou
No abstract text is available yet for this article.
March 2015: Acta Neurologica Taiwanica
https://www.readbyqxmd.com/read/26178515/-the-atypical-developments-of-rolandic-epilepsy-are-predictable-complications
#19
Gabriela Pesántez-Ríos, Antonio Martínez-Bermejo, Joaquín Arcas, Milagros Merino-Andreu, Arturo Ugalde-Canitrot
INTRODUCTION: The development of atypical features in rolandic epilepsy is part of a clinical spectrum of phenotypes that are variable, idiopathic and age-dependent, as well as having a genetically determined predisposition. AIM: To study the electroclinical characteristics suggesting an atypical development in rolandic epilepsy. PATIENTS AND METHODS: A retrospective search was performed in 133 children diagnosed with atypical benign focal epilepsy (ABFE), Landau-Kleffner syndrome and continuous spike-wave during sleep (CSWS)...
August 1, 2015: Revista de Neurologia
https://www.readbyqxmd.com/read/26003806/epilepsy-genetics-the-ongoing-revolution
#20
REVIEW
G Lesca, C Depienne
Epilepsies have long remained refractory to gene identification due to several obstacles, including a highly variable inter- and intrafamilial expressivity of the phenotypes, a high frequency of phenocopies, and a huge genetic heterogeneity. Recent technological breakthroughs, such as array comparative genomic hybridization and next generation sequencing, have been leading, in the past few years, to the identification of an increasing number of genomic regions and genes in which mutations or copy-number variations cause various epileptic disorders, revealing an enormous diversity of pathophysiological mechanisms...
June 2015: Revue Neurologique
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