490 results match your criteria Landau-Kleffner Syndrome


Regression in children with epilepsy.

Neurosci Biobehav Rev 2018 Dec 8;96:210-218. Epub 2018 Dec 8.

Department of Pediatrics, Dalhousie University and the IWK Health Centre, Canada.

Regression in children with epilepsy may involve loss of cognitive abilities, failure to progress or a slowing of developmental trajectory. A few seizures do not lead to regression. Large numbers of seizures may be associated with regression but the cause is an important cofounder. Read More

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http://dx.doi.org/10.1016/j.neubiorev.2018.12.008DOI Listing
December 2018

Addressing sequelae of developmental regression associated with developmental disabilities: A systematic review of behavioral and educational intervention studies.

Neurosci Biobehav Rev 2018 Nov 24;96:56-71. Epub 2018 Nov 24.

Department of Child and Adolescent Psychiatry and Psychotherapy, University Medical Center Goettingen, Goettingen, Germany; iDN - Interdisciplinary Developmental Neuroscience, Division of Phoniatrics, Medical University of Graz, Graz, Austria. Electronic address:

Developmental regression is characteristic of Rett syndrome and it also occurs in a number of other developmental disabilities. To assist clinicians in identifying promising therapeutic approaches, we identified 38 studies that sought to improve adaptive behavior functioning in cases where developmental regression had either already occurred or was likely to occur. Studies were summarized in terms of (a) participants, (b) intervention, (c) dependent variables, (d) outcomes, (e) study design, and (f) certainty of evidence. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S01497634183071
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http://dx.doi.org/10.1016/j.neubiorev.2018.11.014DOI Listing
November 2018
3 Reads

The association of epileptic focus estimated by magnetoencephalography with cognitive function in non-lesional epilepsy with continuous spikes and waves during slow wave sleep (ECSWS) children.

Brain Dev 2018 Oct 17. Epub 2018 Oct 17.

Division of Child Neurology, Nishi-Niigata Chuo National Hospital, Japan.

Objective: Epilepsy with continuous spikes and waves during slow sleep (ECSWS) is associated with cognitive deficits. The underlying mechanism is thought to relate to disturbance of functions of the foci by the persistent epileptic activity. However, the relationship between epileptic foci and cognitive deficits remains largely unknown, except for in Landau-Kleffner syndrome. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S03877604183046
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http://dx.doi.org/10.1016/j.braindev.2018.09.005DOI Listing
October 2018
3 Reads

[Speech and language neurodevelopmental disorders in epilepsy: pathophysiologic mechanisms and therapeutic approaches].

Zh Nevrol Psikhiatr Im S S Korsakova 2018 ;118(8):118-125

Pirogov Russian National Research Medical University, Moscow, Russia.

Speech and language development may be impaired in all forms of epilepsy involving specialized functional areas in the dominant cerebral hemisphere and their connections. The concept of epilepsy-aphasia clinical spectrum was recently proposed, but the notion of aphasia is quite conditional here as many of these patients demonstrate disorders of speech and language development from their infancy. Those forms of epilepsy are considered as continuum from the most severe Landau-Kleffner syndrome (LKS) and epilepsy with continuous spike-and-wave during sleep (CSWS) (also indicating as electrical status epilepticus during sleep - ESES) to intermediate epilepsy-aphasia disorders (with incomplete correspondence to diagnostic criteria of LKS and epilepsy with CSWS). Read More

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http://www.mediasphera.ru/issues/zhurnal-nevrologii-i-psikhi
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http://dx.doi.org/10.17116/jnevro2018118081118DOI Listing
January 2018
12 Reads

Benign epilepsy with centrotemporal spikes - Current concepts of diagnosis and treatment.

Neurol Neurochir Pol 2018 Nov - Dec;52(6):677-689. Epub 2018 Sep 7.

Pediatric Neurology and Pediatrics Clinic, Medical University of Warsaw, Warsaw, Poland.

Benign epilepsy with centrotemporal spikes (BECTS) is the most common focal epilepsy of the childhood and also one of the best known. It has a proclivity to start at a particular age and remit spontaneously before adolescence. Majority of patients may avoid long-term treatment, because of the mild course and very good outcome. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00283843183024
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http://dx.doi.org/10.1016/j.pjnns.2018.08.010DOI Listing
September 2018
7 Reads

[Study of GRIN2A mutation in epilepsy-aphasia spectrum disorders].

Zhonghua Yi Xue Yi Chuan Xue Za Zhi 2018 Jun;35(3):314-318

Department of Pediatrics, Peking University First Hospital, Beijing 100034, China.

Objective: To detect potential mutations of the glutamate receptor subunit (GRIN2A) gene and delineate the clinical-genetic characteristics of patients with epilepsy-aphasia spectrum (EAS) disorders.

Methods: One hundred twenty two patients with Landau-Kleffner syndrome (LKS), epileptic encephalopathy with continuous spike-and-wave during sleep (CSWS), benign childhood epilepsy with centrotemporal spikes (BECT) and BECT variants were recruited. Potential mutations of the GRIN2A gene were screened with Sanger sequencing. Read More

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http://dx.doi.org/10.3760/cma.j.issn.1003-9406.2018.03.002DOI Listing
June 2018
29 Reads

Amantadine: A new treatment for refractory electrical status epilepticus in sleep.

Epilepsy Behav 2018 Jul 10;84:74-78. Epub 2018 May 10.

Department of Pediatrics, Division of Neurology, David Geffen School of Medicine, UCLA Mattel Children's Hospital, Los Angeles, CA, United States. Electronic address:

Purpose: Electrical status epilepticus in sleep (ESES) is an electrographic abnormality linked to language abnormalities and cognitive dysfunction and specifically associated with Landau-Kleffner syndrome (LKS), the syndrome of continuous spike and wave in slow-wave sleep (CSWS), and autistic regression with epileptiform EEG (AREE). As first-line therapies for treatment of ESES display inadequate efficacy and confer substantial risk, we set out to describe our center's experience with amantadine in the treatment of ESES.

Methods: Patients with video-EEG-confirmed ESES who received amantadine were retrospectively identified in a clinical EEG database. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S15255050183032
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http://dx.doi.org/10.1016/j.yebeh.2018.04.018DOI Listing
July 2018
44 Reads

The inhibitory effect of functional lesions on eloquent brain areas: from research bench to operating bed.

Int J Neurosci 2018 Apr 23:1-8. Epub 2018 Apr 23.

a Clinical Neurology Research Center , Shiraz University of Medical Sciences , Shiraz , Iran.

Objectives: Functioning, but injured cerebral connections are hypothesized to inhibit cortical plasticity. Study of neural networks can validate this hypothesis, and provide further practical clues for clinical and surgical options to restore function in eloquent brain areas.

Material And Methods: Cortical lesions in eloquent areas were simulated by means of artificial neural networks. Read More

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https://www.tandfonline.com/doi/full/10.1080/00207454.2018.1
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http://dx.doi.org/10.1080/00207454.2018.1458726DOI Listing
April 2018
4 Reads

Epilepsy in patients with autism: links, risks and treatment challenges.

Authors:
Frank Mc Besag

Neuropsychiatr Dis Treat 2018 18;14:1-10. Epub 2017 Dec 18.

Neurodevelopmental Team, East London Foundation NHS Trust, Family Consultation Clinic, Bedford, UK.

Autism is more common in people with epilepsy, approximately 20%, and epilepsy is more common in people with autism with reported rates of approximately 20%. However, these figures are likely to be affected by the current broader criteria for autism spectrum disorder (ASD), which have contributed to an increased prevalence of autism, with the result that the rate for ASD in epilepsy is likely to be higher and the figure for epilepsy in ASD is likely to be lower. Some evidence suggests that there are two peaks of epilepsy onset in autism, in infancy and adolescence. Read More

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http://dx.doi.org/10.2147/NDT.S120509DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5739118PMC
December 2017
5 Reads

Language Dysfunction in Pediatric Epilepsy.

J Pediatr 2018 Mar 11;194:13-21. Epub 2017 Dec 11.

Department of Neurology, Division of Child Neurology, Stanford University School of Medicine, Palo Alto, CA.

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http://dx.doi.org/10.1016/j.jpeds.2017.10.031DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5826845PMC
March 2018
34 Reads

Treatment of electrical status epilepticus in sleep: Clinical and EEG characteristics and response to 147 treatments in 47 patients.

Eur J Paediatr Neurol 2018 Jan 26;22(1):64-71. Epub 2017 Oct 26.

Department of Pediatric Neurology, Brain Center Rudolf Magnus, University Medical Center Utrecht, Utrecht, The Netherlands.

Objective: Electrical status epilepticus in sleep (ESES) syndrome is characterized by near-continuous sleep-induced epileptiform activity and acquired cognitive deficits. Treatment is assumed mandatory to improve cognitive outcome. We aimed to compare EEG characteristics, subjective evaluation and objective neuropsychological assessment as measures to evaluate treatment efficacy, and to analyze possible predictors. Read More

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http://dx.doi.org/10.1016/j.ejpn.2017.08.006DOI Listing
January 2018
35 Reads

GRIN2A mutations in epilepsy-aphasia spectrum disorders.

Brain Dev 2018 Mar 19;40(3):205-210. Epub 2017 Oct 19.

Department of Pediatrics, Peking University First Hospital, Beijing, China. Electronic address:

Objective: Epilepsy-aphasia spectrum (EAS) are a group of epilepsy syndromes denoting an association between epilepsy, speech disorders and the EEG signature of centrotemporal spikes. Mutations in the GRIN2A gene, encoding the NMDA glutamate receptor α2 subunit were reported in focal epilepsy with speech disorder. We aimed to explore the role of GRIN2A mutations in patients with centrotemporal spikes related epileptic syndromes in a Chinese cohort. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S03877604173026
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http://dx.doi.org/10.1016/j.braindev.2017.09.007DOI Listing
March 2018
17 Reads

The Clinical Spectrum of Benign Epilepsy with Centro-Temporal Spikes: a Challenge in Categorization and Predictability.

J Epilepsy Res 2017 Jun 30;7(1):1-6. Epub 2017 Jun 30.

Department of Pediatric Neurology, Kyungpook National University Children's Hospital, Kyungpook National University School of Medicine, Daegu, Korea.

Benign epilepsy with centro-temporal spikes (BECTS) is the most common type of focal epilepsy in children; it is age-dependent and presumably genetic. Traditionally, children with BECTS have a very good prognosis, even without medical treatment, and are thought to show no neurological symptoms or cognitive deficits. However, many previous studies have shown that BECTS can present with various clinical and electroencephalographic characteristics that are commonly associated with neuropsychological deficits, including linguistic, cognitive, and behavioral impairment. Read More

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http://dx.doi.org/10.14581/jer.17001DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5540684PMC
June 2017
13 Reads

A de novo loss-of-function GRIN2A mutation associated with childhood focal epilepsy and acquired epileptic aphasia.

PLoS One 2017 9;12(2):e0170818. Epub 2017 Feb 9.

Department of Pediatrics and Pediatric Epilepsy Center, Peking University First Hospital, Beijing, China.

Objective: N-methyl-D-aspartate receptors (NMDAR) subunit GRIN2A/GluN2A mutations have been identified in patients with various neurological diseases, such as epilepsy and intellectual disability / developmental delay (ID/DD). In this study, we investigated the phenotype and underlying molecular mechanism of a GRIN2A missense mutation identified by next generation sequencing on idiopathic focal epilepsy using in vitro electrophysiology.

Methods: Genomic DNA of patients with epilepsy and ID/DD were sequenced by targeted next-generation sequencing within 300 genes related to epilepsy and ID/DD. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0170818PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5300259PMC
August 2017
32 Reads

Is ketogenic diet treatment hepatotoxic for children with intractable epilepsy?

Seizure 2016 Dec 13;43:32-38. Epub 2016 Nov 13.

Behçet Uz Children Hospital, Division of Pediatric Neurology, Izmir, Turkey.

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. Read More

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http://dx.doi.org/10.1016/j.seizure.2016.10.024DOI Listing
December 2016
9 Reads

Current and Emerging Therapies of Severe Epileptic Encephalopathies.

Semin Pediatr Neurol 2016 05 3;23(2):180-6. Epub 2016 Jun 3.

Division of Pediatric Neurology, Children's Health Center, Duke University Medical Center, Durham, NC. Electronic address:

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. Read More

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http://dx.doi.org/10.1016/j.spen.2016.06.001DOI Listing
May 2016
3 Reads

The Expanding Clinical Spectrum of Genetic Pediatric Epileptic Encephalopathies.

Semin Pediatr Neurol 2016 05 2;23(2):134-42. Epub 2016 Jun 2.

Division of Pediatric Neurology, Children's Health Center, Duke University Medical Center, Durham, NC. Electronic address:

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. Read More

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http://dx.doi.org/10.1016/j.spen.2016.06.002DOI Listing
May 2016
6 Reads

Pediatric Epileptic Encephalopathies: Pathophysiology and Animal Models.

Semin Pediatr Neurol 2016 05 24;23(2):98-107. Epub 2016 May 24.

Division of Pediatric Neurology, Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD. Electronic address:

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. Read More

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http://dx.doi.org/10.1016/j.spen.2016.05.004DOI Listing
May 2016
5 Reads

Idiopathic focal epilepsies: the "lost tribe".

Epileptic Disord 2016 Sep;18(3):252-88

GSTT, Clin Neurophysiology and Epilepsies, Lambeth Wing, St Thomas' Hospital, London, UK.

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. Read More

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http://dx.doi.org/10.1684/epd.2016.0839DOI Listing
September 2016
68 Reads

Neurocognitive and behavioral profiles of children with Landau-Kleffner syndrome.

Appl Neuropsychol Child 2017 Oct-Dec;6(4):345-354. Epub 2016 Jun 29.

b Department of Neurology, Medical College of Georgia at Georgia Regents University , Augusta , Georgia.

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. Read More

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http://dx.doi.org/10.1080/21622965.2016.1197127DOI Listing
May 2018
21 Reads

Psychiatric and Behavioural Disorders in Children with Epilepsy (ILAE Task Force Report): Behavioural effects of epilepsy surgery.

Epileptic Disord 2016 May 16. Epub 2016 May 16.

MD PhD, Professor Dr. emer., Senior Research Scientist, Depts. Public Health and Child Neurology, University of Turku, Turku, Finland.

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. Read More

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http://dx.doi.org/10.1684/epd.2016.0818DOI Listing
May 2016
7 Reads

Psychiatric and Behavioural Disorders in Children with Epilepsy (ILAE Task Force Report): Subtle behavioural and cognitive manifestations of epilepsy in children.

Epileptic Disord 2016 May 16. Epub 2016 May 16.

MD PhD, Professor Dr. emer., Senior Research Scientist, Depts. Public Health and Child Neurology, University of Turku, Turku, Finland.

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. Read More

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http://dx.doi.org/10.1684/epd.2016.0816DOI Listing
May 2016
7 Reads

Psychiatric and Behavioural Disorders in Children with Epilepsy (ILAE Task Force Report): Behavioural and psychiatric disorders associated with childhood epilepsy syndromes.

Epileptic Disord 2016 May 16. Epub 2016 May 16.

MD PhD, Professor Dr. emer., Senior Research Scientist, Depts. Public Health and Child Neurology, University of Turku, Turku, Finland.

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. Read More

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http://dx.doi.org/10.1684/epd.2016.0815DOI Listing
May 2016
27 Reads

Psychiatric and Behavioural Disorders in Children with Epilepsy (ILAE Task Force Report): Epilepsy and autism.

Epileptic Disord 2016 May 16. Epub 2016 May 16.

MD PhD, Professor Dr. emer., Senior Research Scientist, Depts. Public Health and Child Neurology, University of Turku, Turku, Finland.

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. Read More

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http://dx.doi.org/10.1684/epd.2016.0812DOI Listing
May 2016
14 Reads

The Impact of 3:1 Ketogenic Diet on Cardiac Repolarization Changes in Children with Refractory Seizures: A Prospective Follow-Up Study.

Neuropediatrics 2016 Jun 4;47(3):157-61. Epub 2016 Apr 4.

Department of Pediatric Cardiology, Izmir Dr. Behcet Uz Children's Hospital, Izmir, Turkey.

Background The association between ketogenic diet (KD) and prolonged QT interval, life-threatening ventricular arrhythmias, and sudden death is controversial. Aim We aimed to prospectively evaluate the effect of KD on electrocardiography (ECG) measures in children with refractory epilepsy. Method A total of 70 children with drug-resistant epilepsy who received a KD for at least 12 months were included in the study. Read More

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http://dx.doi.org/10.1055/s-0036-1582139DOI Listing
June 2016
32 Reads

Comment on outcome following multiple subpial transection in Landau-Kleffner syndrome and related regression.

Epilepsia 2016 Apr;57(4):674

Epilepsy Center, Cleveland Clinic, Cleveland, Ohio, U.S.A.

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http://dx.doi.org/10.1111/epi.13336DOI Listing
April 2016
7 Reads

Current understanding and neurobiology of epileptic encephalopathies.

Neurobiol Dis 2016 08 16;92(Pt A):72-89. Epub 2016 Mar 16.

Department of Neuroscience, IRCCS-"Mario Negri" Institute for Pharmacological Research, Milano, Italy.

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. Read More

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http://dx.doi.org/10.1016/j.nbd.2016.03.007DOI Listing
August 2016
6 Reads

Response to immunotherapy in a patient with Landau-Kleffner syndrome and GRIN2A mutation.

Epileptic Disord 2016 Mar;18(1):97-100

Duke University Medical Center, Durham.

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. Read More

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http://dx.doi.org/10.1684/epd.2016.0791DOI Listing
March 2016
11 Reads

Syndrome of Electrical Status Epilepticus During Sleep: Epileptic Encephalopathy Related to Brain Development.

Pediatr Neurol 2016 Mar 19;56:35-41. Epub 2015 Dec 19.

Department of Pediatrics, Chinese PLA General Hospital, Beijing, China; Center of Epilepsy, Beijing Institute for Brain Disorders, Beijing, China. Electronic address:

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. Read More

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http://dx.doi.org/10.1016/j.pediatrneurol.2015.12.006DOI Listing
March 2016
10 Reads

[Neurodevelopmental disorders in children with epilepsy].

Zh Nevrol Psikhiatr Im S S Korsakova 2016;116(3):88-95

Pirogov Russian National Research Medical University, Ministry of Heath of Russian Federation, Moscow.

Neurodevelopmental disorders, including intellectual disability, autistic-spectrum disorders, speech disorders, attention deficit hyperactivity disorder (ADHD), learning disabilities, are more prevalent in children with epilepsy compared with the general population. Marked developmental delay and regression of acquired skills are characteristic of epileptic encephalopathies. Conditions, in which neurodevelopmental disorders are associated with the marked epileptiform EEG activity, while clinical epileptic seizures are absent, represent a serious problem. Read More

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http://dx.doi.org/10.17116/jnevro20161163188-95DOI Listing
December 2018
7 Reads

Cortical interneuron dysfunction in epilepsy associated with autism spectrum disorders.

Authors:
John Jacob

Epilepsia 2016 Feb 19;57(2):182-93. Epub 2015 Dec 19.

Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, Oxford, United Kingdom.

Autism and epilepsy are two associated disorders that are highly prevalent, share common developmental origins, and demonstrate substantial heritability. In this review, cross-disciplinary data in a rapidly evolving field that bridges neurology and psychiatry are synthesized to identify shared biologic mechanisms. The relationship between these debilitating, lifelong conditions is examined at the clinical, genetic, and neurophysiologic levels in humans and in animal models. Read More

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http://dx.doi.org/10.1111/epi.13272DOI Listing
February 2016
2 Reads

Serum inflammatory mediators correlate with disease activity in electrical status epilepticus in sleep (ESES) syndrome.

Epilepsia 2016 Feb 14;57(2):e45-50. Epub 2015 Dec 14.

Department of Pediatric Neurology, Brain Center Rudolf Magnus, University Medical Center Utrecht, Utrecht, The Netherlands.

We aimed to study serum cytokine levels in 11 electrical status epilepticus in sleep (ESES) patients and 20 healthy control children. Patients showed significantly higher levels of interleukin (IL)-1α, IL-6, IL-10, chemokine (C-C motif) ligand (CCL)2 and chemokine (C-X-C motif) ligand (CXCL)8/IL-8 than controls, while macrophage migration inhibitory factor (MIF) and CCL3 were significantly lower. Follow-up analyses in five patients revealed a significant decrease of IL-6 levels after immunomodulating treatment. Read More

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http://dx.doi.org/10.1111/epi.13274DOI Listing
February 2016
11 Reads

Landau-Kleffner syndrome.

Tidsskr Nor Laegeforen 2015 Dec 1;135(22):2061-4. Epub 2015 Dec 1.

Avdeling for kompleks epilepsi Oslo universitetssykehus.

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http://dx.doi.org/10.4045/tidsskr.15.0162DOI Listing
December 2015
4 Reads

Landau-Kleffner syndrome: an uncommon dealt with case in Southeast Asia.

BMJ Case Rep 2015 Sep 29;2015. Epub 2015 Sep 29.

Department of Paediatric Surgery, CM Hospital, Bhilainagar, Chhattisgarh, India.

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. Read More

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http://dx.doi.org/10.1136/bcr-2015-212333DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4593242PMC
September 2015
5 Reads

Outcome following multiple subpial transection in Landau-Kleffner syndrome and related regression.

Epilepsia 2015 Nov 4;56(11):1760-6. Epub 2015 Sep 4.

UCL Institute of Child Health, London, United Kingdom.

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. Read More

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http://dx.doi.org/10.1111/epi.13132DOI Listing
November 2015
9 Reads

Treatment of electrical status epilepticus in sleep: A pooled analysis of 575 cases.

Epilepsia 2015 Nov 4;56(11):1738-46. Epub 2015 Sep 4.

Department of Pediatric Neurology, Brain Center Rudolf Magnus, University Medical Center Utrecht, Utrecht, The Netherlands.

Objective: Epileptic encephalopathy with electrical status epilepticus in sleep (ESES) is a pediatric epilepsy syndrome with sleep-induced epileptic discharges and acquired impairment of cognition or behavior. Treatment of ESES is assumed to improve cognitive outcome. The aim of this study is to create an overview of the current evidence for different treatment regimens in children with ESES syndrome. Read More

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http://dx.doi.org/10.1111/epi.13128DOI Listing
November 2015
23 Reads

Evidence for normal letter-sound integration, but altered language pathways in a case of recovered Landau-Kleffner Syndrome.

Brain Cogn 2015 Oct 29;99:32-45. Epub 2015 Jul 29.

Maastricht University, Maastricht Brain Imaging Center, Faculty of Psychology and Neuroscience, Oxfordlaan 55, 6229 EV Maastricht, Netherlands; Brain Innovation BV, Research & Development, Oxfordlaan 55, 6229 EV Maastricht, Netherlands.

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. Read More

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http://dx.doi.org/10.1016/j.bandc.2015.07.003DOI Listing
October 2015
4 Reads

Acetazolamide for electrical status epilepticus in slow-wave sleep.

Epilepsia 2015 Sep 31;56(9):e134-8. Epub 2015 Jul 31.

Child and Adolescent Neurology, Department of Neurology, Mayo Clinic, Rochester, Minnesota, U.S.A.

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. Read More

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http://dx.doi.org/10.1111/epi.13101DOI Listing
September 2015
18 Reads

Landau-Kleffner Syndrome: An Acquired Epileptic Aphasia.

Acta Neurol Taiwan 2015 Mar;24(1):34-5

Department of Child Neurology, Puli Christian Hospital.

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March 2015
13 Reads

[The atypical developments of rolandic epilepsy are predictable complications].

Rev Neurol 2015 Aug;61(3):106-13

UAM. Universidad Autonoma de Madrid. Hospital Universitario La Paz, 28046 Madrid, Espana.

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). Read More

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August 2015
8 Reads

Seizures and epilepsy: an overview for neuroscientists.

Cold Spring Harb Perspect Med 2015 Jun 1;5(6). Epub 2015 Jun 1.

Division of Neurology, Department of Pediatrics, Sainte-Justine Hospital, Universite Montreal, Montreal, Quebec H3T 1C5, Canada.

Epilepsy is one of the most common and disabling neurologic conditions, yet we have an incomplete understanding of the detailed pathophysiology and, thus, treatment rationale for much of epilepsy. This article reviews the clinical aspects of seizures and epilepsy with the goal of providing neuroscientists an introduction to aspects that might be amenable to scientific investigation. Seizures and epilepsy are defined, diagnostic methods are reviewed, various clinical syndromes are discussed, and aspects of differential diagnosis, treatment, and prognosis are considered to enable neuroscientists to formulate basic and translational research questions. Read More

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http://dx.doi.org/10.1101/cshperspect.a022426DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4448698PMC
June 2015
5 Reads

Effectiveness of a hybrid corticosteroid treatment regimen on refractory childhood seizures and a review of other corticosteroid treatments.

Eur J Paediatr Neurol 2015 Sep 29;19(5):553-60. Epub 2015 Apr 29.

Erasmus University Hospital-Sophia Children's Hospital, The Netherlands. Electronic address:

Background: Many different corticosteroid treatment schedules have been used in order to treat refractory epileptic seizures with encouraging effects on seizure reduction in many epileptic syndromes.

Objective: The objective is to report our experience with a hybrid treatment regimen for refractory seizures in children with epilepsies other than West and Landau-Kleffner syndrome. We hypothesized that a pulse of corticosteroids effectively reduces seizures while low-dosage maintenance treatment reduces side effects. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S10903798150008
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http://dx.doi.org/10.1016/j.ejpn.2015.04.006DOI Listing
September 2015
6 Reads

Sleep and epilepsy syndromes.

Authors:
Bernhard Schmitt

Neuropediatrics 2015 Jun 12;46(3):171-80. Epub 2015 May 12.

Division of Clinical Neurophysiology and Epilepsy, University Children's Hospital, Zurich, Switzerland.

Sleep and epilepsy have a close relationship. About 20% of patients suffer seizures only during the night, approximately 40% only during the day and approximately 35% during the day and night. In certain epilepsy syndromes, the occurrence of seizures is strongly related to sleep or awakening. Read More

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http://dx.doi.org/10.1055/s-0035-1551574DOI Listing
June 2015
10 Reads

New genes for focal epilepsies with speech and language disorders.

Curr Neurol Neurosci Rep 2015 Jun;15(6):35

Department of Paediatrics, The University of Melbourne, The Royal Children's Hospital, Parkville, Australia,

The last 2 years have seen exciting advances in the genetics of Landau-Kleffner syndrome and related disorders, encompassed within the epilepsy-aphasia spectrum (EAS). The striking finding of mutations in the N-methyl-D-aspartate (NMDA) receptor subunit gene GRIN2A as the first monogenic cause in up to 20% of patients with EAS suggests that excitatory glutamate receptors play a key role in these disorders. Patients with GRIN2A mutations have a recognizable speech and language phenotype that may assist with diagnosis. Read More

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http://dx.doi.org/10.1007/s11910-015-0554-0DOI Listing
June 2015
4 Reads

Rare variants in γ-aminobutyric acid type A receptor genes in rolandic epilepsy and related syndromes.

Ann Neurol 2015 Jun 28;77(6):972-86. Epub 2015 Mar 28.

Department of Neurology, Medical University of Vienna, Vienna, Austria.

Objective: To test whether mutations in γ-aminobutyric acid type A receptor (GABAA -R) subunit genes contribute to the etiology of rolandic epilepsy (RE) or its atypical variants (ARE).

Methods: We performed exome sequencing to compare the frequency of variants in 18 GABAA -R genes in 204 European patients with RE/ARE versus 728 platform-matched controls. Identified GABRG2 variants were functionally assessed for protein stability, trafficking, postsynaptic clustering, and receptor function. Read More

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http://doi.wiley.com/10.1002/ana.24395
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http://dx.doi.org/10.1002/ana.24395DOI Listing
June 2015
10 Reads

Antiepileptic treatment in a child with Landau Kleffner syndrome: a case report.

Turk Psikiyatri Derg 2014 ;25(4):282-6

Introduction: Landau-Kleffner Syndrome is a rare childhood disorder characterized by acquired aphasia and epileptiform EEG abnormalities. Hyperactivity and attention problems are common in LKS, and symptoms of Autism Spectrum Disorders (ASDs) may also coexist. Many different treatments have been used for LKS, with varying rates of success. Read More

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November 2015
6 Reads

[Efficacy of methylprednisolone therapy for electrical status epilepticus during sleep in children].

Zhonghua Er Ke Za Zhi 2014 Sep;52(9):678-82

The first author is now in Department of Neurology, Nanjing Children's Hospital Affiliated to Nanjing Medical University, Nanjing 210008, China.

Objective: To evaluate the therapeutic effect of methylprednisolone for electrical status epilepticus during sleep (ESES) in children.

Method: The clinical and EEG data of 82 epilepsy patients with ESES, which included benign childhood epilepsy with centro temporal spikes (BECT) variants, epilepsy with continuous spikes and waves during slow sleep (CSWS) , Landau-Kleffner syndrome (LKS) collected from department of pediatrics, Peking University First Hospital were analyzed from July 2007 to September 2012. During ESES period, all patients received methylprednisolone treatment for three courses, which included methylprednisolone intravenous infusion for three days, followed by oral prednisone for four days every time. Read More

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September 2014
21 Reads

Epileptic encephalopathy with continuous spikes and waves in the occipito-temporal region during slow-wave sleep in two patients with acquired Kanji dysgraphia.

Epileptic Disord 2014 Dec;16(4):540-5

Department of Pediatric Neurology, Child Medical Center, Osaka City General Hospital, Osaka, Japan.

We encountered two patients with acquired Kanji dysgraphia in whom continuous spikes and waves, dominant in the occipito-temporal region, were recorded during slow-wave sleep. Electrical status epileptics during sleep (ESES) was demonstrated on overnight electroencephalography, and dipoles clustered in and around the posterior inferior temporal cortex on magnetoencephalography. Functional neuroimaging suggested dysfunction in the left posterior temporal lobe, including the posterior inferior temporal cortex. Read More

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http://dx.doi.org/10.1684/epd.2014.0698DOI Listing
December 2014
9 Reads