513 results match your criteria Landau-Kleffner Syndrome


Exome sequencing in 57 patients with self-limited focal epilepsies of childhood with typical or atypical presentations suggests novel candidate genes.

Eur J Paediatr Neurol 2020 May 29. Epub 2020 May 29.

Department of Medical Genetics, Member of the ERN EpiCARE, University Hospital of Lyon, Lyon, France; Institut NeuroMyoGène, CNRS UMR 5310 - INSERM U1217, Lyon, France; Université de Lyon - Université Claude Bernard Lyon 1, Lyon, France. Electronic address:

Objective: Self-limited focal epilepsies of childhood (SFEC) are amongst the best defined and most frequent epilepsy syndromes affecting children with usually normal developmental milestones. They include core syndromes such as Rolandic epilepsy or "Benign" epilepsy with Centro-Temporal Spikes and the benign occipital epilepsies, the early onset Panayiotopoulos syndrome and the late-onset Gastaut type. Atypical forms exist for all of them. Read More

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http://dx.doi.org/10.1016/j.ejpn.2020.05.003DOI Listing

Music processing deficits in Landau-Kleffner syndrome: Four case studies in adulthood.

Cortex 2020 Apr 15;129:99-111. Epub 2020 Apr 15.

Lyon Neuroscience Research Center, Inserm, CNRS, Lyon 1 University, Lyon, France.

Verbal-auditory agnosia and aphasia are the most prominent symptoms in Landau-Kleffner syndrome (LKS), a childhood epilepsy that can have sustained long-term effects on language processing. The present study provides the first objective investigation of music perception skills in four adult patients with a diagnosis of LKS during childhood, covering the spectrum of severity of the syndrome from mild to severe. Pitch discrimination, short-term memory for melodic, rhythmic and verbal information, as well as emotion recognition in music and speech prosody were assessed with listening tests, and subjective attitude to music with a questionnaire. Read More

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http://dx.doi.org/10.1016/j.cortex.2020.03.025DOI Listing

Immunotherapy in GRIN2A-negative Landau-Kleffner Syndrome.

Minerva Pediatr 2020 Apr;72(2):139-141

Unit of Pediatrics, San Paolo Hospital, Savona, Italy.

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http://dx.doi.org/10.23736/S0026-4946.19.05419-7DOI Listing

Methylprednisolone pulse therapy in 31 patients with refractory epilepsy: A single-center retrospective analysis.

Epilepsy Behav 2020 Aug 6;109:107116. Epub 2020 May 6.

National Epilepsy Center, NHO, Shizuoka Institute of Epilepsy and Neurological Disorders, Japan.

Purpose: We investigated the efficacy of methylprednisolone pulse therapy (MP) and responder characteristics in patients with refractory epilepsy.

Methods: We reviewed medical records of our center to identify patients with refractory epilepsy treated with MP other than continuous spikes and waves during slow sleep (CSWS), Landau-Kleffner syndrome (LKS), or Rasmussen's syndrome (RS) between 2004 and 2015. A course of MP consisted of intravenous methylprednisolone (30 mg/kg/day) on three consecutive days. Read More

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http://dx.doi.org/10.1016/j.yebeh.2020.107116DOI Listing

[New applications of conventional EEG analysis].

Ideggyogy Sz 2020 Mar;73(3-4):99-110

Debreceni Egyetem, Kenézy Gyula Egyetemi Kórház, Neurológiai Osztály, Debrecen.

Neurophysiological research suggests that the so-called "standard" EEG analysis has been confronted with new diagnostic challenges. The findings mainly concern the occurrence, the neurophysiological and clinical significance of epileptiform EEG discharges in several neurological and psychiatric disorders. In addition to well-known interictal and ictal discharges, a growing number of recently recognized epileptiform phenomena have been described. Read More

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http://dx.doi.org/10.18071/isz.73.0099DOI Listing

Immunotherapy for GRIN2A and GRIN2D-related epileptic encephalopathy.

Epilepsy Res 2020 Jul 2;163:106325. Epub 2020 Apr 2.

Pediatric Neurology Institute, Dana-Dwek Children's Hospital, Tel Aviv Sourasky Medical Center, 6 Weizmann Street, Tel Aviv, Israel; Sackler Faculty of Medicine, Tel Aviv University, P.O.B 39040, Ramat Aviv, Tel Aviv, Israel.

Background: GRIN-related developmental-epileptic encephalopathies are associated with a spectrum of neurodevelopmental disorders, including intellectual disability, epilepsy including continuous spike-and-wave during sleep syndrome (CSWS), or epilepsy-aphasia spectrum phenotypes such as in Landau-Kleffner syndrome. Efficacy of IVIG treatment was recently reported in a patient with LKS related to GRIN2A mutation.

Aim And Methods: We describe the efficacy of Immunotherapy in 5 consecutive patients (4 males, age range 6 months-13 years) with molecularly confirmed GRIN-related epileptic encephalopathy (4 with GRIN2A- related epilepsy-aphasia spectrum/epileptic encephalopathy with CSWS, accompanied by verbal, communicative and behavioural regression, and one patient with GRIN2D - related infantile developmental-epileptic encephalopathy). Read More

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http://dx.doi.org/10.1016/j.eplepsyres.2020.106325DOI Listing

Landau-Kleffner Syndrome: A Diagnostic Challenge.

Cureus 2020 Mar 5;12(3):e7182. Epub 2020 Mar 5.

Obstetrics and Gynecology, St. John's University, New York, USA.

The Landau-Kleffner syndrome (LKS), formerly known as acquired epileptic aphasia, is a rare syndrome that typically presents in early childhood with language regression and seizures. We report a case of LKS in an 7-year-old boy who presented with aggressive behavior, difficulty in maintaining posture, and language regression. Systemic examination, including neurological evaluation, was normal. Read More

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http://dx.doi.org/10.7759/cureus.7182DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7124882PMC

Surgical management of pediatric patients with encephalopathy due to electrical status epilepticus during sleep (ESES).

Epileptic Disord 2020 Feb;22(1):39-54

Division of Pediatric Neurology, Nicklaus Children's Hospital, Miami, USA.

ESES is a developmental epileptic disorder directly responsible for progressive encephalopathy and neurocognitive regression. The natural history, indications for surgical intervention, and predictors for favorable seizure and neuropsychological outcome remain unclear. We performed a retrospective review of children who underwent resective or disconnective surgery for ESES between January 2009 and July 2016 at a large tertiary pediatric center. Read More

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http://dx.doi.org/10.1684/epd.2020.1129DOI Listing
February 2020
0.899 Impact Factor

Epilepsy syndromes of childhood with sleep activation: Insights from functional imaging.

Eur J Paediatr Neurol 2020 Jan 17;24:58-60. Epub 2019 Dec 17.

Department of Pediatric Neurology, CHU d'Angers and Laboratoire Angevin de Recherche en Ingénierie des Systèmes (LARIS), Université d'Angers, France. Electronic address:

In epilepsy syndromes of childhood with sleep activation, defined as the spectrum of epileptic conditions going from classical benign childhood epilepsy with centrotemporal spikes (BECTS) to epileptic encephalopathy (EE) with continuous spike and waves during slow-wave sleep (CSWS) including Landau-Kleffner syndrome (LKS), a lot of functional imaging studies have been performed so far, leading to results that are not always consistent, related to the technique of neuroimaging performed and to the variability of the clinical phenotype. Most consistent findings are, depending of the method used, activations or increased regional glucose metabolism in the epileptogenic regions, and deactivations, hypometabolism or decreased functional connectivity in cortical regions that belong to the default mode network. Functional changes are either transitory, temporally related to the occurrence of interictal epileptiform discharges (IED), or permanent, persisting across IED-free periods. Read More

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http://dx.doi.org/10.1016/j.ejpn.2019.12.006DOI Listing
January 2020

[Genetically determined epileptic encephalopathies].

Medicina (B Aires) 2019 ;79 Suppl 3:42-47

Servicio de Neurología Pediátrica, Departamento de Pediatría, Hospital Italiano de Buenos Aires, Argentina.

Epileptic encephalopathies is a group of epileptic syndromes characterized by progressive cognitive impairment beyond the expected for the epilepsy activity. They are characterized by severe pharmaco-resistant epilepsy, severely abnormal electroencephalograms, early-age onset, neurocognitve impairment, variable phenotype and usually normal brain MRI. These syndromes are usually genetically determined. Read More

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October 2019
1 Read

Development of Ontology for Self-limited Epilepsy with Centrotemporal Spikes and Application of Data Mining Algorithms to Identify New Subtypes.

Isr Med Assoc J 2019 Jul;21(7):503

Department of Pediatric Neurology, Dana-Dwek Hospital, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel.

Background: Benign rolandic epilepsy or benign childhood epilepsy with centrotemporal spikes (BCECTS) is a common childhood epileptic syndrome. The syndrome resolves in adolescence, but 1-7% of patients have an atypical presentation, some of which require aggressive medical treatment. Early treatment may prevent complications and neurocognitive deterioration. Read More

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July 2019
6 Reads

An Uncommon Presentation of Mucopolysaccharidosis Type IIIb.

Iran J Child Neurol 2019 ;13(3):105-111

School of Medicine, Shahid Beheshti University of Medical Sciences, Tehran, Iran.

Mucopolysaccharidosis type III (MPS III; Sanfilippo syndrome) is a metabolic disorder characterized by a lysosomal enzyme deficiency in the catabolic pathway of heparan sulfate. The patients with mucopolysaccharidosis type III usually present with declined neurocognitive functions such as speech and hearing loss. Subtle somatic features of patients with mucopolysaccharidosis type III can lead to diagnostic delay and consequently, a greater neurocognitive deterioration may happen. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6586455PMC
January 2019
2 Reads

Idiopathic encephalopathy related to status epilepticus during slow sleep (ESES) as a "pure" model of epileptic encephalopathy. An electroclinical, genetic, and follow-up study.

Epilepsy Behav 2019 08 26;97:244-252. Epub 2019 Jun 26.

Danish Epilepsy Centre - Filadelfia, Dianalund, Denmark; Department of Regional Health Research, University of Southern Denmark, Odense, Denmark.

Objective: The objective of the study was to investigate electroclinical and neuropsychological features, genetic background, and evolution of children with idiopathic encephalopathy with status epilepticus during slow sleep (ESES), including Landau-Kleffner syndrome (LKS).

Material And Methods: All children diagnosed with idiopathic ESES at the Danish Epilepsy Centre between March 2003 and December 2014 were retrospectively reviewed. Repeated 24-hour electroencephalography (24-h EEG) recordings, neuropsychological assessments, and clinical-neurological evaluation were performed throughout the follow-up in all patients. Read More

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http://dx.doi.org/10.1016/j.yebeh.2019.05.030DOI Listing
August 2019
10 Reads

Social cognition and psychopathology in childhood and adolescence.

Epilepsy Behav 2019 11 10;100(Pt B):106210. Epub 2019 Jun 10.

East London Foundation NHS Trust, 5-7 Rush Court, Bedford MK40 3JT, UK; University College, London, UK; King's College, London, UK. Electronic address:

There is a substantial body of research on social cognition in adults with epilepsy, and in broad categories such as focal and generalized epilepsies, but much less has been written about social cognition in children with epilepsy (CWE), and in childhood-onset epilepsy syndromes specifically. In several of these syndromes, autism spectrum disorder (ASD) and attention-deficit hyperactivity disorder (ADHD), two disorders with social cognitive impairments, are reported. There is strong evidence for social cognitive deficits in juvenile myoclonic epilepsy (JME). Read More

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http://dx.doi.org/10.1016/j.yebeh.2019.03.015DOI Listing
November 2019
8 Reads

Update on the genetics of the epilepsy-aphasia spectrum and role of GRIN2A mutations.

Epileptic Disord 2019 Jun;21(S1):41-47

Aix-Marseille University, INSERM UMR1249, INMED, Marseille, France.

Formerly idiopathic, focal epilepsies (IFE) are self-limiting, "age-related" diseases that mainly occur during critical developmental periods. Childhood epilepsy with centrotemporal spikes, or Rolandic epilepsy (RE), is the most frequent form of IFE. Together with the Landau-Kleffner syndrome and the epileptic Encephalopathy related to Status Epilepticus during slow Sleep syndrome (ESES), RE is part of a single and continuous spectrum of childhood epilepsies and epileptic encephalopathies with acquired cognitive, behavioral and speech and/or language impairment, known as the epilepsy-aphasia spectrum (EAS). Read More

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http://dx.doi.org/10.1684/epd.2019.1056DOI Listing
June 2019
44 Reads

Encephalopathy related to Status Epilepticus during slow Sleep: from concepts to terminology.

Epileptic Disord 2019 Jun;21(S1):5-12

Paediatric Neurosciences Research Group, Royal Hospital for Children & University of Glasgow, Member of the European Reference Network EpiCARE, Glasgow, UK.

Five pediatric and adult neurologists with clinical and research interests in Encephalopathy related to Status Epilepticus during slow Sleep (ESES) express their opinions on definition, diagnostic assessment and terminology that may be considered for this condition. The aim of this "debate" is to identify aspects in which there is a shared opinion and areas where there are still controversies in the classification and suggest areas which demand further studies and research. Read More

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http://dx.doi.org/10.1684/epd.2019.1051DOI Listing
June 2019
16 Reads

Epilepsy and Autism Severity: A Study of 6,975 Children.

Autism Res 2019 08 24;12(8):1251-1259. Epub 2019 May 24.

Department of Medical Informatics, Interactive Autism Network at Kennedy Krieger, Baltimore, Maryland.

Epilepsy is known to occur in a higher-than-expected proportion of individuals with autism spectrum disorders (ASDs). Prior studies of this heterogeneous disorder have suggested that intelligence quotient (IQ) may drive this relationship. Because intellectual disability (ID) is, independently of ASD, a risk factor for epilepsy, current literature calls into question the long-understood unique relationship between ASD and epilepsy. Read More

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https://onlinelibrary.wiley.com/doi/abs/10.1002/aur.2132
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http://dx.doi.org/10.1002/aur.2132DOI Listing
August 2019
14 Reads

[Continuous spike-waves during slow-wave sleep: Experience during 20 years].

An Pediatr (Barc) 2019 Sep 14;91(3):180-188. Epub 2019 Feb 14.

Sección de Neuropediatría, Hospital Materno Infantil Gregorio Marañón, Madrid, España.

Introduction: Continuous spikes and waves during slow sleep (CSWS) is an EEG pattern that appears during childhood, and is often associated with cognitive impairment. It can appear in the course of epileptic syndromes, as well as in benign epilepsy. The aim of this study is to analyse epidemiological and clinical characteristic of patients with CSWS, in order to describe possible predictive factors in their outcome. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S16954033193002
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http://dx.doi.org/10.1016/j.anpedi.2018.12.016DOI Listing
September 2019
37 Reads

Perisylvian epileptic network revisited.

Seizure 2019 Feb 21;65:31-41. Epub 2018 Dec 21.

National Institute of Clinical Neuroscience, Budapest, Hungary.

We overview here the new data about the epileptic spectrum disorders within the frame of perisylvian epileptic network since our first trial to synthetize knowledge about this system epilepsy (Halász et al., 2005). We found evidences for a continual features relating together syndromes constituting this spectrum disorder in several fields: in sharing genetic origin, in common perisylvian human communication circuitry, in NREM sleep related potentiation of interictal epileptiform discharges of the centro-temporal spike phenomenon and in the discharge related cognitive impairment, reflecting functional deficits in human communication abilities. Read More

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http://dx.doi.org/10.1016/j.seizure.2018.12.003DOI Listing
February 2019
15 Reads
2.059 Impact Factor

Regression in children with epilepsy.

Neurosci Biobehav Rev 2019 01 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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https://linkinghub.elsevier.com/retrieve/pii/S01497634183068
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http://dx.doi.org/10.1016/j.neubiorev.2018.12.008DOI Listing
January 2019
45 Reads

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

Neurosci Biobehav Rev 2019 01 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
January 2019
35 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 2019 Feb 17;41(2):163-172. 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
February 2019
11 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
March 2019
23 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
June 2019
29 Reads

Transient microstructural brain anomalies and epileptiform discharges in mice defective for epilepsy and language-related NMDA receptor subunit gene Grin2a.

Epilepsia 2018 10 26;59(10):1919-1930. Epub 2018 Aug 26.

INSERM, UMR1249, INMED, Aix-Marseille University, Marseille, France.

Objective: The epilepsy-aphasia spectrum (EAS) is a heterogeneous group of age-dependent childhood disorders characterized by sleep-activated discharges associated with infrequent seizures and language, cognitive, and behavioral deficits. Defects in the GRIN2A gene, encoding a subunit of glutamate-gated N-methyl-d-aspartate (NMDA) receptors, represent the most important cause of EAS identified so far. Neocortical or thalamic lesions were detected in a subset of severe EAS disorders, and more subtle anomalies were reported in patients with so-called "benign" phenotypes. Read More

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http://doi.wiley.com/10.1111/epi.14543
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http://dx.doi.org/10.1111/epi.14543DOI Listing
October 2018
45 Reads

Toxicological evaluation of convulsant and anticonvulsant drugs in human induced pluripotent stem cell-derived cortical neuronal networks using an MEA system.

Sci Rep 2018 Jul 10;8(1):10416. Epub 2018 Jul 10.

Department of Electronics, Graduate School of Engineering, Tohoku Institute of Technology, 35-1 Yagiyama Kasumicho, Taihaku-ku, Sendai, Miyagi, 982-8577, Japan.

Functional evaluation assays using human induced pluripotent stem cell (hiPSC)-derived neurons can predict the convulsion toxicity of new drugs and the neurological effects of antiepileptic drugs. However, differences in responsiveness depending on convulsant type and antiepileptic drugs, and an evaluation index capable of comparing in vitro responses with in vivo responses are not well known. We observed the difference in synchronized burst patterns in the epileptiform activities induced by pentylentetrazole (PTZ) and 4-aminopryridine (4-AP) with different action mechanisms using multi-electrode arrays (MEAs); we also observed that 100 µM of the antiepileptic drug phenytoin suppressed epileptiform activities induced by PTZ, but increased those induced by 4-AP. Read More

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http://dx.doi.org/10.1038/s41598-018-28835-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6039442PMC
July 2018
34 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
85 Reads

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

Epilepsy Behav 2018 07 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
112 Reads

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

Int J Neurosci 2018 Nov 23;128(11):1022-1029. Epub 2018 Apr 23.

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

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. 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
November 2018
13 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
43 Reads

Language Dysfunction in Pediatric Epilepsy.

J Pediatr 2018 03 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
56 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
70 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
54 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
19 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
73 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
29 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
22 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
21 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
20 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
214 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
66 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
27 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
26 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
71 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
34 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
78 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
23 Reads