536 results match your criteria Landau-Kleffner Syndrome


International League Against Epilepsy classification and definition of epilepsy syndromes with onset in childhood: Position paper by the ILAE Task Force on Nosology and Definitions.

Epilepsia 2022 06 3;63(6):1398-1442. Epub 2022 May 3.

Robert Debré Hospital, Public Hospital Network of Paris, NeuroDiderot, National Institute of Health and Medical Research, Department Medico-Universitaire Innovation Robert-Debré, Pediatric Neurology, University of Paris, Paris, France.

The 2017 International League Against Epilepsy classification has defined a three-tier system with epilepsy syndrome identification at the third level. Although a syndrome cannot be determined in all children with epilepsy, identification of a specific syndrome provides guidance on management and prognosis. In this paper, we describe the childhood onset epilepsy syndromes, most of which have both mandatory seizure type(s) and interictal electroencephalographic (EEG) features. Read More

View Article and Full-Text PDF

Long-term outcome of developmental and epileptic encephalopathies.

Authors:
P Van Bogaert

Rev Neurol (Paris) 2022 Apr 27. Epub 2022 Apr 27.

Department of Pediatric Neurology, CHU d'Angers, and Laboratoire Angevin de Recherche en Ingénierie des Systèmes (LARIS), Université d'Angers, 4, rue Larrey, 49000 Angers, France. Electronic address:

Developmental and epileptic encephalopathies are conditions where there is developmental impairment related to both the underlying etiology independent of epileptiform activity and the epileptic encephalopathy. Usually they have multiple etiologies. Therefore, long-term outcome is related to both etiology-related factors and epilepsy-related factors-age at onset of epilepsy, type(s) of seizure(s), type of electroencephalographic abnormalities, duration of the epileptic disorder. Read More

View Article and Full-Text PDF

Mild neurological phenotype in a family carrying a novel N-terminal null GRIN2A variant.

Eur J Med Genet 2022 May 1;65(5):104500. Epub 2022 Apr 1.

Department of Translational Medicine, Federico II University, Naples, Italy. Electronic address:

GRIN2A encodes for the 2A subunit of N-methyl-D-aspartate receptors. Pathogenic variants in GRIN2A have been associated with a wide spectrum of neurodevelopmental disorders ranging from speech disorders and/or self-limiting epilepsy (childhood epilepsy with centrotemporal spikes) to severe and disabling phenotypes (atypical childhood epilepsy with centrotemporal spikes, epileptic encephalopathy with continuous spike-wave during sleep, Landau-Kleffner syndrome and infantile-onset epileptic encephalopathy). Here we describe a family with two affected sisters with atypical childhood epilepsy with centrotemporal spikes and their mildly affected mother carrying a novel N-terminal null variant in GRIN2A gene. Read More

View Article and Full-Text PDF

The role of vagus nerve stimulation in genetic etiologies of drug-resistant epilepsy: a meta-analysis.

J Neurosurg Pediatr 2022 Mar 18:1-14. Epub 2022 Mar 18.

3Department of Neurosurgery, Division of Pediatric Neurosurgery, Northwestern University, Lurie Children's Hospital, Chicago, Illinois.

Objective: Drug-resistant epilepsy (DRE) affects many children. Vagus nerve stimulation (VNS) may improve seizure control; however, its role in children with genetic etiologies of epilepsy is not well described. The authors systematically reviewed the literature to examine the effectiveness of VNS in this cohort. Read More

View Article and Full-Text PDF

De novo GRIN2A variants associated with epilepsy and autism and literature review.

Epilepsy Behav 2022 04 23;129:108604. Epub 2022 Feb 23.

Department of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialities "G. D'Alessandro", University of Palermo, Palermo, Italy. Electronic address:

N-methyl-D-aspartate receptors (NMDAR) are di- or tri-heterotetrameric ligand-gated ion channels composed of two obligate glycine-binding GluN1 subunits and two glutamate-binding GluN2 or GluN3 subunits, encoded by GRIN1, GRIN2A-D, and GRIN3A-B receptor genes respectively. Each NMDA receptor subtype has different temporal and spatial expression patterns in the brain and varies in the cell types and subcellular localization resulting in different functions. They play a crucial role in mediating the excitatory neurotransmission, but are also involved in neuronal development and synaptic plasticity, essential for learning, memory, and high cognitive functions. Read More

View Article and Full-Text PDF

What is the effect of pharmacological treatment for continuous spike-wave during slow wave sleep syndrome and Landau-Kleffner syndrome? A Cochrane Review summary with commentary.

Dev Med Child Neurol 2022 04 22;64(4):411-412. Epub 2022 Jan 22.

Erasmus University Medical Center, Department of Rehabilitation Medicine, Rotterdam, the Netherlands.

View Article and Full-Text PDF

Cerebral dominance in an unusual case of Landau-Kleffner syndrome.

BMJ Case Rep 2021 Dec 9;14(12). Epub 2021 Dec 9.

Institute of Neuroscience, Medanta, Gurgaon, Haryana, India.

Landau-Kleffner syndrome (LKS) is described by the International Classification of Epileptic Syndromes since 1985 as a constellation of clinical and electrographic signs, including acquired aphasia, regression of language milestones and seizures, along with sleep-activated paroxysms on electroencephalogram which can progress to electrographic status epilepticus of sleep. In this case, a 7-year-old boy presented with an atypical history of new-onset aphasia and regression of language milestones with rare seizures. However, there was an electrographic mismatch in the form of right-sided epileptiform activity and continuous spike and wave of sleep pattern. Read More

View Article and Full-Text PDF
December 2021

Aphasia and a Dual-Stream Language Model in a 4-Year-Old Female with Landau-Kleffner Syndrome.

Neuropediatrics 2021 Dec 1. Epub 2021 Dec 1.

Division of Neurology, National Center for Child Health and Development, Tokyo, Japan.

Landau-Kleffner syndrome (LKS) is a rare neurological disorder characterized by acquired aphasia. LKS presents with distinctive electroencephalography (EEG) findings, including diffuse continuous spike and wave complexes (CSW), particularly during sleep. There has been little research on the mechanisms of aphasia and its origin within the brain and how it recovers. Read More

View Article and Full-Text PDF
December 2021

Cnksr2 Loss in Mice Leads to Increased Neural Activity and Behavioral Phenotypes of Epilepsy-Aphasia Syndrome.

J Neurosci 2021 11 27;41(46):9633-9649. Epub 2021 Sep 27.

Department of Cell Biology, Duke University Medical School, Durham, North Carolina 27710

Epilepsy Aphasia Syndromes (EAS) are a spectrum of childhood epileptic, cognitive, and language disorders of unknown etiology. is a strong X-linked candidate gene implicated in EAS; however, there have been no studies of genetic models to dissect how its absence may lead to EAS. Here we develop a novel KO mouse line and show that male mice exhibit increased neural activity and have spontaneous electrographic seizures. Read More

View Article and Full-Text PDF
November 2021

Genomic analysis of "microphenotypes" in epilepsy.

Am J Med Genet A 2022 01 27;188(1):138-146. Epub 2021 Sep 27.

Department of Neurology, Beaumont Hospital, School of Pharmacy and Biomedical Sciences, and FutureNeuro Research Centre, Royal College of Surgeons in Ireland, Dublin, Ireland.

Large international consortia examining the genomic architecture of the epilepsies focus on large diagnostic subgroupings such as "all focal epilepsy" and "all genetic generalized epilepsy". In addition, phenotypic data are generally entered into these large discovery databases in a unidirectional manner at one point in time only. However, there are many smaller phenotypic subgroupings in epilepsy, many of which may have unique genomic risk factors. Read More

View Article and Full-Text PDF
January 2022

More than one self-limited epilepsy of childhood in the same patient: A multicenter study.

Epilepsy Res 2021 Nov 17;177:106768. Epub 2021 Sep 17.

Hospital de Pediatría Prof. Dr. Juan P. Garrahan, Buenos Aires, Argentina. Electronic address:

Objective: We describe the evolution of the electroclinical picture of patients with different types of self-limited epilepsy of childhood (SLEC) occurring at the same or at different times with or without atypical evolutions as well as patients with SLEC associated with childhood absence epilepsy (CAE).

Material And Methods: A multicenter, retrospective, descriptive study was conducted evaluating patients with SLEC who had focal seizures of different types of SLEC including atypical evolutions as well as SLEC associated with absence epilepsy seen at eight Argentinian centers between April 2000 and April 2019. Of 7705 patients with SLEC, aged between 2 and 14 years (mean, 7. Read More

View Article and Full-Text PDF
November 2021

Cognitive linguistic Treatment in Landau Kleffner Syndrome: Improvement in Daily Life Communication.

Child Neurol Open 2021 Jan-Dec;8:2329048X211022196. Epub 2021 Jun 2.

Erasmus MC, University Medical Center Rotterdam, Rotterdam, The Netherlands.

We report a case study of cognitive linguistic treatment in a teenager with chronic severe Landau Kleffner Syndrome. The effect of speech and language therapy in LKS is rarely examined and our case is unique in that we use an effective approach in adult aphasia to treat language deficits in aphasia in LKS. The results show successful acquisition of a considerable amount of new words as well as improved communication in daily life. Read More

View Article and Full-Text PDF

Clinical profile of patients with rolandic epilepsy at a clinic in rural Maharashtra.

J Family Med Prim Care 2021 Mar 8;10(3):1263-1266. Epub 2021 Apr 8.

Centre for Medical Genetics, Office No 250 and 251, Ecstasy Business Park, JSD Road, Mulund West, Mumbai, Maharashtra, India Centre at which study carried out: Dr Sunil Sable Children's Hospital and Saikrishna Child Neurology Centre, Shirdi -423109, Maharashtra, India.

Purpose: To describe the seizure pattern, treatment strategies and outcome in a series of children with Rolandic seizures or childhood epilepsy with centrotemporal spikes.

Materials And Methods: Patients were defined as Rolandic epilepsy if on electroencephalographic studies high voltage spike and waves were seen in centrotemporal areas, could be followed by slow waves, often activated on sleep and could shift from one side to other or be secondarily generalized. Typical (TRS) or benign were those with normal intellect. Read More

View Article and Full-Text PDF

A follow-up study in children with status epilepticus during sleep: From clinical spectrum to outcome.

Epilepsy Behav 2021 04 25;117:107843. Epub 2021 Feb 25.

Department of Neurology, Guangzhou Women and Children's Medical Center, Guangzhou Medical University, China. Electronic address:

Purpose: To evaluate the correlation between clinical spectrum and therapeutic outcomes and neuropsychological deficits in children with status epilepticus during sleep (SES).

Methods: The clinical spectrum of patients with SES was defined as follows: status epilepticus of benign childhood epilepsy with centro-temporal spikes (SEBECTs), atypical benign focal epilepsy during childhood (ABFEC), non-idiopathic focal epilepsy (NIFE), and Landau-Kleffner syndrome (LKS). SES cases were divided into 4 groups according to neuropsychological findings before treatment: developmental delay/intellectual disability (DD/ID), cognitive impairment (CI), attention deficit and/or hyperactivity behaviors (AHD), and normal group (NG). Read More

View Article and Full-Text PDF

Treatment Practices and Outcomes in Continuous Spike and Wave during Slow Wave Sleep: A Multicenter Collaboration.

J Pediatr 2021 05 20;232:220-228.e3. Epub 2021 Jan 20.

Department of Pediatrics & Neurology, University of Colorado, Aurora, CO.

Objectives: To determine how continuous spike and wave during slow wave sleep (CSWS) is currently managed and to compare the effectiveness of current treatment strategies using a database from 11 pediatric epilepsy centers in the US.

Study Design: This retrospective study gathered information on baseline clinical characteristics, CSWS etiology, and treatment(s) in consecutive patients seen between 2014 and 2016 at 11 epilepsy referral centers. Treatments were categorized as benzodiazepines, steroids, other antiseizure medications (ASMs), or other therapies. Read More

View Article and Full-Text PDF

Clinical Forms and Genotype of Severe End of Epileptic-Aphasia Spectrum Disorder.

Front Pediatr 2020 6;8:574803. Epub 2020 Nov 6.

Department of Neurology, Children's Hospital of Chongqing Medical University, Chongqing, China.

This study aims to analyze the electroclinical characteristics and gene test results of children on the severe end of the epilepsy aphasia spectrum (EAS) and also the correlation of EAS-related GRIN2A genes to explore the genotype-phenotype relationships, as well as potential pathogenic mechanism of EAS. A retrospective study was conducted on the participants diagnosed with Landau-Kleffner syndrome (LKS), epileptic encephalopathy with continuous spike-and-wave during sleep (CSWS), and atypical benign partial epilepsy (ABPE) at the Children's Hospital of Chongqing Medical University from January 2013 to June 2019. Whole-exome sequencing was performed in six patients, and epileptic panel was carried out in two. Read More

View Article and Full-Text PDF
November 2020

Pharmacological treatment for continuous spike-wave during slow wave sleep syndrome and Landau-Kleffner Syndrome.

Cochrane Database Syst Rev 2020 11 6;11:CD013132. Epub 2020 Nov 6.

Child Neuropsychiatry Unit, IRCCS Istituto Giannina Gaslini, Genoa, Italy.

Background: Continuous spike-wave during slow wave sleep syndrome (CSWS) and Landau-Kleffner syndrome (LKS) are two epileptic encephalopathies that present with neurocognitive regression, aphasia, and clinical seizures, typically presenting in children around five years of age. The pathophysiology of these conditions is not completely understood. Some studies suggest a common origin for both. Read More

View Article and Full-Text PDF
November 2020

Acquired epileptiform aphasia: 44 years after diagnosis.

Epilepsy Behav Rep 2020 31;14:100388. Epub 2020 Aug 31.

Section on Neurology, Ontario Medical Association, Toronto, Ontario, Canada.

We report a case of a 48-year-old woman who was diagnosed with Landau-Kleffner syndrome (LKS) at the age of 4 and reassessed by the same neurologist four decades later. While her seizures abated by the time she was 12 years old, she was left with chronic aphasia, despite receiving optimal care. Although she graduated from high school, started her own family, and was gainfully employed, she was vulnerable in situations that required clear communication. Read More

View Article and Full-Text PDF

Sleep and Epilepsy Link by Plasticity.

Front Neurol 2020 28;11:911. Epub 2020 Aug 28.

Institute of Behavioral Sciences, Semmelweis University, Budapest, Hungary.

We aimed to explore the link between NREM sleep and epilepsy. Based on human and experimental data we propose that a sleep-related epileptic transformation of normal neurological networks underlies epileptogenesis. Major childhood epilepsies as medial temporal lobe epilepsy (MTLE), absence epilepsy (AE) and human perisylvian network (PN) epilepsies - made us good models to study. Read More

View Article and Full-Text PDF

Early prediction of encephalopathic transformation in children with benign epilepsy with centro-temporal spikes.

Brain Dev 2021 Feb 8;43(2):268-279. Epub 2020 Sep 8.

Sackler Faculty of Medicine, Tel-Aviv University, Israel; Pediatric Neurology Institute, Dana-Dwek Children's Hospital, Tel Aviv Sourasky Medical Center, Israel.

Background: Most children with Benign epilepsy with centro-temporal spikes (BECTS) undergo remission during late adolescence and do not require treatment. In a small group of patients, the condition may evolve to encephalopathic syndromes including epileptic encephalopathy with continuous spike-and-wave during sleep (ECSWS), or Landau-Kleffner Syndrome (LKS). Development of prediction models for early identification of at-risk children is of utmost importance. Read More

View Article and Full-Text PDF
February 2021

Differential Diagnosis of Landau-Kleffner Syndrome Versus Post Encephalitis Syndrome in a 13-year-old Boy With Autism Spectrum Disorder.

Cureus 2020 Jul 25;12(7):e9385. Epub 2020 Jul 25.

Psychiatry, State University of New York Upstate Medical University, Syracuse, USA.

Landau-Kleffner syndrome (LKS) is a rare childhood neurological condition that causes developmental regression, loss of language skills and abnormal electroencephalogram (EEG) patterns. Its etiology is unknown. This report describes a case of a 13-year-old boy who at 3. Read More

View Article and Full-Text PDF

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 Jul 29;27:104-110. 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

View Article and Full-Text PDF

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

Cortex 2020 08 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

View Article and Full-Text PDF

Immunotherapy in GRIN2A-negative Landau-Kleffner Syndrome.

Minerva Pediatr 2020 04;72(2):139-141

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

View Article and Full-Text PDF

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

Epilepsy Behav 2020 08 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

View Article and Full-Text PDF

[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

View Article and Full-Text PDF

Immunotherapy for GRIN2A and GRIN2D-related epileptic encephalopathy.

Epilepsy Res 2020 07 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

View Article and Full-Text PDF

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

View Article and Full-Text PDF

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

View Article and Full-Text PDF
February 2020

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

View Article and Full-Text PDF
January 2020