landau kleffner syndrome

Summary

Summary: A syndrome characterized by the onset of isolated language dysfunction in otherwise normal children (age of onset 4-7 years) and epileptiform discharges on ELECTROENCEPHALOGRAPHY. Seizures, including atypical absence (EPILEPSY, ABSENCE), complex partial (EPILEPSY, COMPLEX PARTIAL), and other types may occur. The electroencephalographic abnormalities and seizures tend to resolve by puberty. The language disorder may also resolve although some individuals are left with severe language dysfunction, including APHASIA and auditory AGNOSIA. (From Menkes, Textbook of Child Neurology, 5th ed, pp749-50; J Child Neurol 1997 Nov;12(8):489-495)

Top Publications

  1. ncbi Epileptic syndromes in childhood: clinical features, outcomes, and treatment
    Peter Camfield
    Department of Pediatrics, Dalhousie University and the IWK Health Centre, Halifax, Nova Scotia, Canada
    Epilepsia 43:27-32. 2002
  2. ncbi Children with ESES: variability in the syndrome
    Michele Van Hirtum-Das
    Department of Pediatrics and Neurology, David Geffen School of Medicine at UCLA, Mattel Children s Hospital at UCLA, Los Angeles, CA, USA
    Epilepsy Res 70:S248-58. 2006
  3. ncbi The Landau-Kleffner syndrome
    Hanna Kolski
    The Hospital for Sick Children, Toronto, Ontario, Canada
    Adv Exp Med Biol 497:195-208. 2002
  4. ncbi The pathophysiological mechanisms of cognitive and behavioral disturbances in children with Landau-Kleffner syndrome or epilepsy with continuous spike-and-waves during slow-wave sleep
    Lotte Nieuwenhuis
    The Department of Neurology, Epilepsy Centre Kempenhaeghe, Heeze, The Netherlands
    Seizure 15:249-58. 2006
  5. ncbi Landau-Kleffner syndrome, electrical status epilepticus in slow wave sleep, and language regression in children
    Kathryn A McVicar
    Departments of Neurology, Pediatrics, The Comprehensive Epilepsy Management Center, Montefiore Medical Center, The Albert Einstein College of Medicine, Bronx, New York 10467, USA
    Ment Retard Dev Disabil Res Rev 10:144-9. 2004
  6. ncbi Electrical status epilepticus in sleep
    Katherine Nickels
    Department of Neurology, Mayo Clinic, Rochester, MN 55902, USA
    Semin Pediatr Neurol 15:50-60. 2008
  7. ncbi Clinical evidence that epilepsy is a progressive disorder with special emphasis on epilepsy syndromes that do progress
    Gregory L Holmes
    Neuroscience Center at Dartmouth, Dartmouth Medical School, Lebanon, New Hampshire, USA
    Adv Neurol 97:323-31. 2006
  8. ncbi Atypical presentations of benign childhood epilepsy with centrotemporal spikes: a review
    Uri Kramer
    Child Neurology Unit, Tel Aviv Sourasky Medical Center, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
    J Child Neurol 23:785-90. 2008
  9. ncbi Hemispheric integrative therapy in Landau-Kleffner syndrome: applications for rehabilitation sciences
    Victor M Pedro
    Department of Clinical Sciences, University of Bridgeport, CT, USA
    Int J Neurosci 115:1227-38. 2005
  10. ncbi Interactions between sleep and epilepsy
    M Mendez
    Department of Medicine, Division of Neurology, Duke University Medical Center, Durham, North Carolina 27707, USA
    J Clin Neurophysiol 18:106-27. 2001

Research Grants

Detail Information

Publications163 found, 100 shown here

  1. ncbi Epileptic syndromes in childhood: clinical features, outcomes, and treatment
    Peter Camfield
    Department of Pediatrics, Dalhousie University and the IWK Health Centre, Halifax, Nova Scotia, Canada
    Epilepsia 43:27-32. 2002
    ..Many of the epilepsies that begin in childhood are benign. Others interfere seriously with cognitive and social development...
  2. ncbi Children with ESES: variability in the syndrome
    Michele Van Hirtum-Das
    Department of Pediatrics and Neurology, David Geffen School of Medicine at UCLA, Mattel Children s Hospital at UCLA, Los Angeles, CA, USA
    Epilepsy Res 70:S248-58. 2006
    ..We undertook a retrospective study of children who present with significant activation of paroxysmal discharges during sleep to examine the clinical spectrum of disorders that present with such an EEG abnormality...
  3. ncbi The Landau-Kleffner syndrome
    Hanna Kolski
    The Hospital for Sick Children, Toronto, Ontario, Canada
    Adv Exp Med Biol 497:195-208. 2002
    ..Landau-Kleffner Syndrome is a rare childhood disorder which involves seizures and acquired aphasia. Anticonvulsants, or the passage of time, may control the seizures, but speech recovery is variable, and the aphasia may persist...
  4. ncbi The pathophysiological mechanisms of cognitive and behavioral disturbances in children with Landau-Kleffner syndrome or epilepsy with continuous spike-and-waves during slow-wave sleep
    Lotte Nieuwenhuis
    The Department of Neurology, Epilepsy Centre Kempenhaeghe, Heeze, The Netherlands
    Seizure 15:249-58. 2006
    ....
  5. ncbi Landau-Kleffner syndrome, electrical status epilepticus in slow wave sleep, and language regression in children
    Kathryn A McVicar
    Departments of Neurology, Pediatrics, The Comprehensive Epilepsy Management Center, Montefiore Medical Center, The Albert Einstein College of Medicine, Bronx, New York 10467, USA
    Ment Retard Dev Disabil Res Rev 10:144-9. 2004
    ..An understanding of the available data on clinical characteristics, EEG findings, pathology, prognosis, and treatment of these syndromes is essential for further progress in this area...
  6. ncbi Electrical status epilepticus in sleep
    Katherine Nickels
    Department of Neurology, Mayo Clinic, Rochester, MN 55902, USA
    Semin Pediatr Neurol 15:50-60. 2008
    ..Early recognition and effective therapy are necessary to improve long-term prognosis in this condition...
  7. ncbi Clinical evidence that epilepsy is a progressive disorder with special emphasis on epilepsy syndromes that do progress
    Gregory L Holmes
    Neuroscience Center at Dartmouth, Dartmouth Medical School, Lebanon, New Hampshire, USA
    Adv Neurol 97:323-31. 2006
  8. ncbi Atypical presentations of benign childhood epilepsy with centrotemporal spikes: a review
    Uri Kramer
    Child Neurology Unit, Tel Aviv Sourasky Medical Center, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
    J Child Neurol 23:785-90. 2008
    ..This review delineates the different forms with emphasis on the cognitive hazards of the more malignant types...
  9. ncbi Hemispheric integrative therapy in Landau-Kleffner syndrome: applications for rehabilitation sciences
    Victor M Pedro
    Department of Clinical Sciences, University of Bridgeport, CT, USA
    Int J Neurosci 115:1227-38. 2005
    ..At completion of the program, EEG was controlled, reading, language, and auditory processing improved and objective behavioral-social measures improved significantly...
  10. ncbi Interactions between sleep and epilepsy
    M Mendez
    Department of Medicine, Division of Neurology, Duke University Medical Center, Durham, North Carolina 27707, USA
    J Clin Neurophysiol 18:106-27. 2001
    ....
  11. ncbi Perisylvian polymicrogyria in Landau-Kleffner syndrome
    Peter Huppke
    Department of Pediatrics and Pediatric Neurology, Faculty of Medicine, Georg August University, Gottingen, Germany
    Neurology 64:1660. 2005
  12. ncbi Autistic epileptiform regression
    Roberto Canitano
    Division of Child and Adolescent Neuropsychiatry, General University Hospital of Siena, Italy
    Funct Neurol 21:97-101. 2006
    ....
  13. ncbi [Bifocal atypical rolandic epilepsy with speech dyspraxia]
    V A Karlov
    Zh Nevrol Psikhiatr Im S S Korsakova 104:4-9. 2004
    ..Significant clinical improvement and electrographical positive effect in EEG were achiered after prednisolone and sodium valproate treatment...
  14. ncbi A clinical and EEG study on idiopathic partial epilepsies with evolution into ESES spectrum disorders
    Sema Saltik
    SSK Goztepe Educational Hospital, Department of Pediatrics, Istanbul, Turkey
    Epilepsia 46:524-33. 2005
    ....
  15. ncbi [Landau kleffner syndrome]
    M J Pablo
    Servicio de Neurofisiologia, Hospital Universitario Miguel Servet, Zaragoza, 50009, Espana
    Rev Neurol 34:262-4. 2002
    The Landau Kleffner syndrome (SLK) is associated with paroxystic alterations of the electroencephalogram which are intensified during sleep, with acquired aphasia and epilepsy, in 75 83% of the cases...
  16. ncbi The relationship between sleep and epilepsy
    P Kotagal
    Section of Pediatric Epilepsy and Sleep Disorders, Cleveland Clinic Foundation, OH 44195, USA
    Semin Pediatr Neurol 8:241-50. 2001
    ..Sleep architecture and daytime alertness are influenced by seizures and antiepileptic medications. This review examines the clinical and basic science aspects of this relationship between sleep and epilepsy...
  17. ncbi Electroencephalography findings in autism: similarities and differences from Landau-Kleffner syndrome
    Paul M Levisohn
    University of Colorado Health Sciences Center, Denver, CO 80262, USA
    Semin Pediatr Neurol 11:218-24. 2004
  18. ncbi Seizures and epilepsy among children with language regression and autistic spectrum disorders
    Edwin Trevathan
    Pediatric Epilepsy Center, Departments of Neurology and Pediatrics, Washington University School of Medicine, Campus Box 8111, 660 South Euclid Avenue, St Louis, MO 63110 1093, USA
    J Child Neurol 19:S49-57. 2004
    ....
  19. ncbi Landau-Kleffner syndrome (acquired epileptic aphasia) in a Nigerian child
    O F Aina
    Department of Psychiatry, College of Medicine, University of Lagos, P M B 12003, Lagos, Nigeria
    Niger Postgrad Med J 9:29-31. 2002
    ..The clinical seizure is easily controlled by an antiepileptic; but speech recovery is variable, and this is usually poor in cases presenting one or two years after onset...
  20. ncbi [Electroclinical characteristics of Landau-Kleffner syndrome]
    K Iu Mukhin
    Zh Nevrol Psikhiatr Im S S Korsakova 103:16-27. 2003
    ..Clobasam in dosage 0.5-0.75 mg/kg daily was the most efficient in blockade of EEG diffuse epileptiform activity, reduction of aphasia symptoms and behavior improvement...
  21. ncbi Episodic receptive aphasia in a child with Landau-Kleffner Syndrome: PET correlates
    Aimee F Luat
    Carman and Ann Adams Department of Pediatrics, Children s Hospital of Michigan, Wayne State University, Detroit, MI, USA
    Brain Dev 28:592-6. 2006
    ..The clinical course of our patient with transient EEG and PET findings suggest that glucose metabolism in LKS cannot be attributed solely to interictal epileptiform activities on scalp EEG...
  22. ncbi Importance of early drug treatment in prognosis of the Landau-Kleffner syndrome
    N Sikic
    Hearing and Speech Rehabilitation Center, Polyclinic SUVAG, Zagreb, Croatia
    Coll Antropol 25:529-34. 2001
    ..Standard diagnostic criteria must be established to enable timely treatment...
  23. ncbi Outcome at adulthood of the continuous spike-waves during slow sleep and Landau-Kleffner syndromes
    Julien Praline
    Neurology Department, University Hospital, Tours Neuropediatric Department, Bicetre Hospital, Le Kremlin Bicetre, France
    Epilepsia 44:1434-40. 2003
    ....
  24. ncbi [Landau-Kleffner syndrome: relationship between aphasia and electroencepharographic changes]
    K Suwa
    Department of Pediatrics, Jichi Medical School, Tochigi
    No To Hattatsu 28:306-11. 1996
    ..The appearance of diffuse spike and wave complexes almost always preceded the exacerbation of aphasia. Prior to the improvement of aphasia, EEG abnormality localized into the temporal region, and subsequently disappeared...
  25. ncbi Safe and effective use of the ketogenic diet in children with epilepsy and mitochondrial respiratory chain complex defects
    Hoon Chul Kang
    Department of Pediatrics and Epilepsy Center, Sanggye Paik Hospital, Inje University College of Medicine, Seoul, Korea
    Epilepsia 48:82-8. 2007
    ..To evaluate the clinical efficacy and safety of the ketogenic diet (KD) for patients with intractable childhood epilepsy and mitochondrial respiratory chain (RC) complex defects...
  26. ncbi Linking autism, regression and Landau-Kleffner syndrome: integrative role of nerve growth factor
    Mariachiara Cortesi
    Med Hypotheses 68:1178-9. 2007
  27. ncbi Neuropsychological findings in Rolandic epilepsy and Landau-Kleffner syndrome
    Marie Noëlle Metz-Lutz
    CNRS UMR7004, Louis Pasteur University of Strasbourg School of Medicine, Strasbourg, France
    Epilepsia 47:71-5. 2006
    ....
  28. ncbi The spectrum from BCECTS to LKS: The Rolandic EEG trait-impact on cognition
    Ulrich Stephani
    University Children s Hospital, Department of Neuropediatrics, Kiel, Germany
    Epilepsia 47:67-70. 2006
    ..The neurobiology of RDs and their relationship to cognitive dysfunction and epilepsy requires further study...
  29. ncbi Efficacy of very high dose steroid treatment in a case of Landau-Kleffner syndrome
    Siobhan Gallagher
    Division of Neurology, Department of Paediatrics, The Hospital for Sick Children, University of Toronto, Canada
    Dev Med Child Neurol 48:766-9. 2006
    ..The patient demonstrated an excellent clinical response highlighting the importance of a multidisciplinary approach to management of LKS...
  30. ncbi Sleep and arousal mechanisms in experimental epilepsy: epileptic components of NREM and antiepileptic components of REM sleep
    M N Shouse
    Sleep Disturbance Research, VA Greater Los Angeles Health Care System, Department of Neurobiology, UCLA School of Medicine, Los Angeles, California, USA
    Ment Retard Dev Disabil Res Rev 10:117-21. 2004
    ....
  31. ncbi Landau-Kleffner syndrome beginning with stuttering: case report
    Sarenur Tutuncuoglu
    Department of Pediatrics, Division of Child Neurology, Ege University Faculty of Medicine, Izmir, Turkey
    J Child Neurol 17:785-8. 2002
    ..We suggest that if a child with normal language function starts to stutter, Landau-Kleffner syndrome must be considered in the differential diagnosis...
  32. ncbi Visual hallucinations associated with zonisamide
    Cigdem I Akman
    Division of Epilepsy and Clinical Neurophysiology, Department of Neurology, Children s Hospital, Harvard Medical School, Boston, Massachusetts, USA
    Pharmacotherapy 23:93-6. 2003
    ..Further observations and reports will help clarify this adverse effect. Until then, clinicians need to be aware of this possible complication associated with zonisamide...
  33. ncbi [Landau-Kleffner and autistic regression: the importance of differential diagnosis]
    Karla M N Ribeiro
    Serviço de Psiquiatria da Infãncia e da Adolescência, Instituto de Psiquiatria, Hospital das Clinicas, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, SP, Brasil
    Arq Neuropsiquiatr 60:835-9. 2002
    ..Language regression is observed LKS and AR. We stress the main differences between these two entities because misdiagnosis may postpone early intervention and consequent benefits, as observed in our case...
  34. ncbi Status epilepticus in idiopathic generalized epilepsy
    Simon Shorvon
    The Institute of Neurology, London, United Kingdom
    Epilepsia 46:73-9. 2005
    ..Similarly, cerebral damage seems not to occur in the forms of nonconvulsive SE in human IGE...
  35. ncbi The neurology of sign language
    Neil Gordon
    Huntlywood, 3 Styal Road, Wilmslow SK9 4AE, UK
    Brain Dev 26:146-50. 2004
    ..Other methods of treatment than signing, such as cochlear implants may be increasingly used in the future, but it seems likely that sign language will continue to be a dominant feature in the deaf culture...
  36. ncbi [Video electroencephalography in seizure disorders of children]
    M Kacinski
    Klinika Neurologii Dzieciecej Collegium Medicum Uniwersytetu Jagiellońskiego, Krakow
    Przegl Lek 58:25-31. 2001
    ..In approximately one third of patients, video-EEG evaluation was helpful in selecting appropriate therapy...
  37. ncbi Developmental regression in autism spectrum disorders
    Sally J Rogers
    University of California, Davis Medical Center, M I N D Institute, Davis, California 95817, USA
    Ment Retard Dev Disabil Res Rev 10:139-43. 2004
    ....
  38. ncbi [Non-verbal auditory agnosia with EEG abnormalities and epilepsy; an unusual case of Landau-Kleffner syndrome]
    T Koeda
    Department of Pediatrics, Kitakyushu City Sogo Ryoiku Center, Fukuoka
    No To Hattatsu 24:262-7. 1992
    ..Finally, she could speak a few words after the learning letters...
  39. ncbi [Antiepileptic drugs in the treatment of autistic regression syndromes]
    J J García-Peñas
    Hospital Universitario Nino Jesus, 28009 Madrid, Espana
    Rev Neurol 40:S173-6. 2005
    ....
  40. ncbi A pilot study transitioning children onto levetiracetam monotherapy to improve language dysfunction associated with benign rolandic epilepsy
    Eric H Kossoff
    The Johns Hopkins Hospital, Baltimore, MD, USA
    Epilepsy Behav 11:514-7. 2007
    ..08). Seizures improved in five, decreasing from 2.7 to 1.0 seizure per 6 months (P=0.11). Results from this pilot study suggest that levetiracetam may have a beneficial effect on language in children with BRE...
  41. ncbi Revisiting the role of magnetoencephalography in epilepsy
    Christoph Baumgartner
    Department of Clinical Neurology, Medical University of Vienna, Wahringer Gurtel 18 20, A 1090 Vienna, Austria
    Curr Opin Neurol 19:181-6. 2006
    ..This review considers the current role of magnetoencephalography in clinical epileptology...
  42. ncbi Cognitive and behavioral outcomes of epileptic syndromes: implications for education and clinical practice
    Frank M C Besag
    Twinwoods Health Resource Centre, Bedford, United Kingdom
    Epilepsia 47:119-25. 2006
    ....
  43. ncbi Clinical spectrum and medical treatment of children with electrical status epilepticus in sleep (ESES)
    Uri Kramer
    Pediatric Neurology Unit, Dana Children s Hospital, Tel Aviv Medical Center, Tel Aviv, Israel
    Epilepsia 50:1517-24. 2009
    ..To describe the clinical spectrum and to evaluate the efficacy of different therapeutic agents in children with electrical status epilepticus in sleep (ESES)...
  44. ncbi [Rolandic epilepsy: epidemiological and clinical characteristics and outcome]
    T Dura Trave
    Unidad de Neuropediatria, Hospital Materno Infantil Virgen del Camino, Pamplona, Espana
    An Pediatr (Barc) 68:466-73. 2008
    ..To analyse the epidemiological, clinical and developmental characteristics of Rolandic epilepsy as an aid to its suspected diagnosis in daily clinical practice...
  45. ncbi [Specific language disorders: their diagnosis, classification and study using magnetoencephalography]
    J A Munoz-Yunta
    Hospital Universitari del Mar, 08003 Barcelona, Espana
    Rev Neurol 40:S115-9. 2005
    ..After many studies and attempts to reach an agreement, specific language disorders have been broadly classified into two groups: specific expressive language disorder and specific receptive-expressive language disorder...
  46. ncbi [Diagnostic focus on the child with epilepsy and neuropsychological deterioration]
    A C Rodríguez-Barrionuevo
    Hospital Materno Infantil, Unidad de Neuropediatria, Carlos Haya, Malaga, Espana
    Rev Neurol 26:322-30. 1998
    ....
  47. ncbi Epileptic encephalopathies: a brief overview
    Rima Nabbout
    Department of Pediatrics, Hopital Necker Enfants Malades, 149 rue de Sevres, 75743 Paris Cedex 15, France
    J Clin Neurophysiol 20:393-7. 2003
    ..Treatment differs for all of these syndromes. It is important to avoid potential drug-induced worsening, and valproate is preferred when a definitive diagnosis is not reached in children and especially infants...
  48. ncbi [Cases of idiopathic and cryptogenic epilepsias in a regional referral neuropediatric unit]
    J Lopez-Pison
    Sección de Neuropediatría, Hospital Miguel Servet, Espana
    Rev Neurol 31:733-8. 2000
    ..In this study we analysed a series of cases of idiopathic and cryptogenic epilepsy attended by the Neuropaediatric Department of the Hospital Miguel Servet in Zaragoza (Spain)...
  49. ncbi Continuous focal spikes during REM sleep in a case of acquired aphasia (Landau-Kleffner syndrome)
    P Genton
    Centre Saint Paul, Marseille, France
    Sleep 15:454-60. 1992
    ..However, this profile was transient and all epileptiform changes disappeared during clinical recovery at 18 months of follow-up...
  50. ncbi [Neurocognitive dysfunction in electrical status epilepticus during slow-wave sleep syndrome: Can the natural course of the syndrome be modified with early pharmacological treatment?]
    J J García-Peñas
    Hospital Infantil Universitario Nino Jesus, Madrid, Espana
    Rev Neurol 50:S37-47. 2010
    ....
  51. ncbi Atypical evolutions of benign localization-related epilepsies in children: are they predictable?
    N Fejerman
    Department of Neurology, Pediatric Hospital Juan P Garrahan, Buenos Aires, Argentina
    Epilepsia 41:380-90. 2000
    ....
  52. ncbi Epilepsy-aphasia syndromes
    Ortrud K Steinlein
    Ludwig Maximilians University of Munich, School of Medicine, Institute of Human Genetics, Goethestrasse 29, 80336 Munich, Germany
    Expert Rev Neurother 9:825-33. 2009
    ..The monogenic etiology of this rare seizure disorder offers the possibility to study the molecular basis of epilepsy-aphasia syndromes...
  53. ncbi Epilepsy and language development: the continuous spike-waves during slow sleep syndrome
    Severine Debiais
    Department of Neurology, CHRU de Tours, France
    Epilepsia 48:1104-10. 2007
    ..Language can be affected but, to date, language disorders and their long-term outcome have been documented only rarely...
  54. ncbi Encephalopathy with electrical status epilepticus during slow sleep or ESES syndrome including the acquired aphasia
    C A Tassinari
    Department of Neurological Sciences, University of Bologna, Bellaria Hospital, Via Altura 3, 40139, Bologna, Italy
    Clin Neurophysiol 111:S94-S102. 2000
    ..Subpial transection is proposed in some instances as in non-regressive acquired aphasia. Recent data support the concept that ESES syndrome may include a large subset of developmental or acquired regressive conditions of infancy...
  55. ncbi Epilepsy in autism
    Roberto Tuchman
    Miami Children s Hospital, Dan Marino Center, Department of Neurology, Weston, FL 33331, USA
    Lancet Neurol 1:352-8. 2002
    ..There is no evidence-based treatment recommendation for individuals with autism, regression, and subclinical epilepsy. Double-blind studies with sufficient power to resolve this issue are urgently needed...
  56. ncbi Corticosteroids for the treatment of Landau-kleffner syndrome and continuous spike-wave discharge during sleep
    D Barry Sinclair
    Comprehensive Epilepsy Program, University of Alberta, Edmonton, Alberta, Canada
    Pediatr Neurol 32:300-6. 2005
    ..Side effects are few and reversible, and benefits appear long lasting. Corticosteroids should be considered as a treatment option in children with Landau-Kleffner syndrome and CSWS...
  57. ncbi The perisylvian epileptic network. A unifying concept
    Peter Halasz
    National Institute of Psychiatry and Neurology, Neurological Department, Budapest
    Ideggyogy Sz 58:21-31. 2005
    ....
  58. ncbi Neurophysiology of CSWS-associated cognitive dysfunction
    Stefano Seri
    Wellcome Laboratory for MEG Studies, School of Life and Health Sciences, Aston University, Birmingham, United Kingdom
    Epilepsia 50:33-6. 2009
    ..Differences in behavioral phenotypes could be explained in term of maturational and genetic differences, as well as by the functional specificity of the involved areas...
  59. ncbi Landau-Kleffner syndrome: a study of 29 patients
    Roberto Horacio Caraballo
    Department of Neurology, Hospital de Pediatria Prof Dr Juan P Garrahan, Buenos Aires, Argentina Electronic address
    Seizure 23:98-104. 2014
    ..The aim of the study was to retrospectively analyze the electroclinical features, etiology, treatment, and prognosis of 29 patients with Landau-Kleffner syndrome (LKS) with a long-term follow-up...
  60. ncbi [Children's aphasias and Landau-Kleffner syndrome in the light of brain plasticity]
    N Sh Aleksandrova
    Zh Nevrol Psikhiatr Im S S Korsakova 104:54-8. 2004
  61. ncbi Landau-Kleffner syndrome is not an eponymic badge of ignorance
    Edouard Hirsch
    Department of Neurology HUS, Strasbourg, France
    Epilepsy Res 70:S239-47. 2006
    ....
  62. ncbi Neurophysiological activity underlying altered brain metabolism in epileptic encephalopathies with CSWS
    Xavier De Tiège
    Laboratoire de Cartographie Fonctionnelle du Cerveau, UNI ULB Neuroscience Institute, Universite Libre de Bruxelles ULB, Brussels, Belgium
    Epilepsy Res 105:316-25. 2013
    ..These findings highlight the critical value of combining FDG-PET with time-sensitive functional neuroimaging approaches such as MSI to assess CSWS epileptic network when surgery is considered as a therapeutic approach...
  63. ncbi Encephalopathy with status epilepticus during slow sleep: "the Penelope syndrome"
    Carlo A Tassinari
    Department of Neurological Sciences, University of Bologna, Bologna, Italy
    Epilepsia 50:4-8. 2009
    ..As in the myth of Penelope, the wife of Odysseus, what is weaved during the day will be unraveled during the night...
  64. ncbi Landau-Kleffner syndrome and CSWS syndrome: treatment with intravenous immunoglobulins
    Willem F M Arts
    Department of Paediatric Neurology, Erasmus Medical Center, Rotterdam, The Netherlands
    Epilepsia 50:55-8. 2009
    ..It might also reflect the natural course of the disease. Although the temporal relation between IVIg treatment and clinical improvement cannot be denied in individual patients, its real value remains to be determined...
  65. ncbi Electrical status epilepticus in sleep: clinical presentation and pathophysiology
    Iván Sánchez Fernández
    Division of Epilepsy and Clinical Neurophysiology, Department of Neurology, Boston Children s Hospital, Harvard Medical School, Boston, Massachusetts, USA
    Pediatr Neurol 47:390-410. 2012
    ..These factors may dynamically alter normal processes of brain development, leading to an age-related pattern of electroclinical expression of electrical status epilepticus in sleep...
  66. ncbi Spike-induced interference in auditory sensory processing in Landau-Kleffner syndrome
    S Seri
    Department of Developmental Neuropsychiatry, University La Sapienza, Rome, Italy
    Electroencephalogr Clin Neurophysiol 108:506-10. 1998
    ....
  67. ncbi Impaired temporo-occipital blood flow in an atypical CLN1 case with late infantile onset and granular osmiophilic deposits
    M Philippart
    Mental Retardation Center, UCLA School of Medicine, Los Angeles, California, USA
    Eur J Paediatr Neurol 5:185-7. 2001
    ..The boy died shortly thereafter. Decreased cerebral blood flow is a new finding in CLN1 with delayed onset. Calcium-channel blockers improve cerebral blood flow and perhaps delay clinical regression...
  68. ncbi Neuropsychological characteristics of five children with the Landau-Kleffner syndrome: dissociation of auditory and phonological discrimination
    M Korkman
    Brain and Behavior Institute, University of Maastricht, The Netherlands
    J Int Neuropsychol Soc 4:566-75. 1998
    ..This suggests that the primary deficit of the receptive aphasia is an impairment of auditory phonological discrimination rather than a generalized auditory agnosia...
  69. ncbi EEG latency analysis for hemispheric lateralisation in Landau-Kleffner syndrome
    Maria del Carmen Martín Miguel
    Department of Clinical Neurophysiology, HGU Gregorio Marañon, UCM, Madrid, Spain
    Clin Neurophysiol 122:244-52. 2011
    ..To determine the reliability of latency analysis in lateralising the origin of epileptiform discharges in pre-surgical assessment of Landau-Kleffner syndrome (LKS)...
  70. ncbi Landau Kleffner syndrome
    S Bharani
    Department of Pediatrics, Medical College, Baroda 390 001, Gujarat, India
    Indian J Pediatr 68:567-9. 2001
    ..gradual loss of speech and generalized EEG (electroencephalography) abnormalities was diagnosed as Landau Kleffner Syndrome. He initially developed generalized convulsions which later changed to partial seizures during the course ..
  71. ncbi A review of the relationships between Landau-Kleffner syndrome, electrical status epilepticus during sleep, and continuous spike-waves during sleep
    John R Hughes
    Department of Neurology, University of Illinois Medical Center at Chicago, Chicago, IL 60612, USA
    Epilepsy Behav 20:247-53. 2011
    ..Memory disorders are common, because the nearly continuous generalized discharges in sleep do not allow for the memory consolidation that also occurs during sleep. Medications and possible etiologies are discussed...
  72. ncbi Continuous spike-and-wave activity during slow-wave sleep: syndrome or EEG pattern?
    P Veggiotti
    Child Neuropsychiatry Division, Neurological Institute Casimiro Mondino Foundation IRCCS, University of Pavia, Italy
    Epilepsia 40:1593-601. 1999
    ....
  73. ncbi Treatment in typical and atypical rolandic epilepsy
    D Rating
    Dept Pediatric Neurology, Children s Hospital, University of Heidelberg, Im Neuenheimer Feld 153, Heidelberg, Germany
    Epileptic Disord 2:S69-72. 2000
    ..Steroids seem the have the best efficacy in these cases...
  74. ncbi Electrophysiological evidence of persisting unilateral auditory cortex dysfunction in the late outcome of Landau and Kleffner syndrome
    N Wioland
    Service d Explorations Fonctionnelles du Système Nerveux, Clinique Neurologique, Hopitaux Universitaires de Strasbourg, 67091 cedex, Strasbourg, France
    Clin Neurophysiol 112:319-23. 2001
    ..Evoked potentials were used to check this hypothesis and to localize the level of the dysfunction along the auditory pathways...
  75. ncbi Epileptic encephalopathy with continuous spike-waves during slow-wave sleep including Landau-Kleffner syndrome
    Patrick Van Bogaert
    Department of Pediatric Neurology, ULB Hopital Erasme, Brussels, Belgium Electronic address
    Handb Clin Neurol 111:635-40. 2013
    ..Rehabilitation represents an important part of the treatment and visual forms of language should be encouraged in children with LKS...
  76. ncbi The effect of levetiracetam on focal nocturnal epileptiform activity during sleep--a placebo-controlled double-blind cross-over study
    Pål Gunnar Larsson
    Department of Neurosurgery, Oslo University Hospital, Norway
    Epilepsy Behav 24:44-8. 2012
    ..0002). To the best of our knowledge, this is the first placebo-controlled double-blind cross-over study for any AED in patients with ESES. The effect of LEV is comparable with its effect in treatment of epileptic seizures...
  77. ncbi Landau-Kleffner syndrome: sleep EEG characteristics at onset
    R Massa
    Unité d Explorations Fonctionnelles des Epilepsies, Hopitaux Universitaires de Strasbourg, Strasbourg, France
    Clin Neurophysiol 111:S87-93. 2000
    ..These cases raise the question of whether LKS and BECTS delineate fundamentally different conditions, or represent subclasses of a broad continuum...
  78. ncbi The spectrum of neuropsychiatric abnormalities associated with electrical status epilepticus in sleep
    A S Galanopoulou
    Department of Neurology, Albert Einstein College of Medicine, 1410 Pelham Parkway South, NY 10461, Bronx, USA
    Brain Dev 22:279-95. 2000
    ..Prolonged and if needed repetitive sleep electroencephalographs (EEGs) are warranted for proper diagnosis...
  79. ncbi Epileptic syndromes with focal seizures of childhood and adolescence
    Tobias Loddenkemper
    Pediatric Neurology Center, Department of Neurology, Cleveland Clinic, Cleveland, OH, USA Division of Epilepsy and Clinical Neurophysiology, Children s Hospital Boston, Boston, MA, USA
    Handb Clin Neurol 107:195-208. 2012
    ..Interactions between brain development and maturation processes as well as genetic influences may play a role in the development of various childhood epileptic syndromes associated with language and cognitive deficits...
  80. ncbi Successful use of intravenous immunoglobulin as initial monotherapy in Landau-Kleffner syndrome
    M A Mikati
    Adult and Pediatric Epilepsy Program and Department of Pediatrics, American University of Beirut, Beirut, Lebanon
    Epilepsia 41:880-6. 2000
    ..In this article we present the first case in which a patient with Landau-Kleffner syndrome was given intravenous immunoglobulin (IVIG) as his first and only therapy and responded to it...
  81. ncbi [Language and learning disorders in epilepsy with continuous spike-waves during slow sleep]
    C Billard-Daudu
    , 78, , , France
    Epileptic Disord 3:SI77-85. 2001
    ..Pathogenesis remains unknown and the relationship between EEG evolution and language improvement is not as clear as in Landau-Kleffner syndrome...
  82. ncbi Levetiracetam as add-on therapy in different subgroups of "benign" idiopathic focal epilepsies in childhood
    C von Stülpnagel
    Clinic for Neuropediatrics and Neurologic Rehabilitation, Epilepsy Centre for Children and Young People, Vogtareuth, Germany
    Epilepsy Behav 17:193-8. 2010
    ..1%) showed improvement in cognition and/or language functions and/or behavior. Surprisingly, LEV tended to be more helpful in atypical rolandic epilepsies and other variants...
  83. ncbi Nocturnal epileptiform EEG discharges, nocturnal epileptic seizures, and language impairments in children: review of the literature
    G M Overvliet
    Department of Neurology, Maastricht University Medical Center, Maastricht, The Netherlands
    Epilepsy Behav 19:550-8. 2010
    ..The diseased neuronal networks are less efficient compared with normal neuronal networks. This disorganization may cause language impairments...
  84. ncbi Acquired epileptiform aphasia: a dimensional view of Landau-Kleffner syndrome and the relation to regressive autistic spectrum disorders
    Gerry A Stefanatos
    Cognitive Neurophysiology Laboratory, Moss Rehabilitation Research Institute, Albert Einstein Medical Center, Philadelphia, PA 19141, USA
    Child Neuropsychol 8:195-228. 2002
    ..The implications of this viewpoint are discussed, with emphasis on parallels between the AEA variants and regressive autistic spectrum disorders...
  85. ncbi The effects of vagus nerve stimulation therapy on patients with intractable seizures and either Landau-Kleffner syndrome or autism
    Yong D Park
    Department of Neurology child, Medical College of Georgia, 1120 15th Street, Room BG 2000H, Augusta, GA 30912, USA
    Epilepsy Behav 4:286-90. 2003
    ....
  86. ncbi [Epileptic syndromes in children]
    Ann Sofie Eriksson
    Spesialsykehuset for epilepsi 1337 Sandvika
    Tidsskr Nor Laegeforen 123:1362-4. 2003
  87. ncbi Language dysfunction in epileptic conditions
    James W Wheless
    Department of Neurology, University of Texas Houston, 77030, USA
    Semin Pediatr Neurol 9:218-28. 2002
    ..We demonstrate how magnetoencephalography (MEG) offers the ability to analyze the relationship of language, EEG abnormalities, and epilepsy...
  88. ncbi Characteristics of the epilepsies
    David R Chabolla
    Department of Neurology, Mayo Clinic, Jacksonville, Fla, USA
    Mayo Clin Proc 77:981-90. 2002
    ..Understanding the characteristics of the epilepsy syndromes provides a powerful tool for the prognosis and treatment of individuals experiencing seizures. In this article, we discuss characteristic features of the epilepsies...
  89. ncbi [Epileptic syndromes which show continuous spike and wake complexes during slow wave sleep]
    M Nieto Barrera
    , Hospital Infantil Virgen del Rocio, Sevilla, Espania
    Rev Neurol 25:1045-51. 1997
    ..The neuropsychological manifestations depend probably on the localization of the original epileptic focus...
  90. ncbi Sleep electroencephalograms in young children with autism with and without regression
    Gillian Baird
    Newcomen Centre, Guy s Hospital, UK
    Dev Med Child Neurol 48:604-8. 2006
    ..There was no evidence that these cases of autism with and without regression were associated with epileptic encephalopathy. The significance of epileptiform discharges without epilepsy in the sleep EEG in autism remains unknown...
  91. ncbi Landau-Kleffner syndrome: study of four cases
    Lúcia H Coutinho dos Santos
    Departamento de Pediatria, Centro de Neuropediatria, Hospital de Clinicas, Universidade Federal do Parana, Curitiba, PR, Brasil
    Arq Neuropsiquiatr 60:239-41. 2002
    ..Anticonvulsants and prednisone were used for treatment with good control of seizures in all cases and a less effect response in acquired aphasia. Further studies are necessary to elucidate the causes and management of this syndrome...
  92. ncbi Speech, language, and cognitive dysfunction in children with focal epileptiform activity: A follow-up study
    Gunilla Rejnö-Habte Selassie
    Institute of Neuroscience and Physiology Speech and Language Pathology, Sahlgrenska Academy at the University of Gothenburg, Gothenburg, Sweden
    Epilepsy Behav 18:267-75. 2010
    ..Dysfunction of communication, oral motor ability, and stuttering were noted in a few. Family history of seizures and abundant epileptiform activity indicated a worse prognosis...
  93. ncbi Epidemiological study of Landau-Kleffner syndrome (LKS) in Japan
    Makiko Kaga
    National Institute of Mental Health, National Center of Neurology and Psychiatry, Japan Electronic address
    Brain Dev 36:284-6. 2014
    ..We aimed to determine the incidence and prevalence of LKS in Japanese children...
  94. ncbi Sign language in Landau-Kleffner syndrome
    Thierry Deonna
    Unité de Neurologie et de Neuroréhabilitation Pédiatrique, Département médico chirurgical de pédiatrie, CHUV, Lausanne, Switzerland
    Epilepsia 50:77-82. 2009
    ..On the contrary, SL may even facilitate this recovery by stimulating functionally connected core language networks and by helping speech therapy and auditory training...
  95. ncbi Serial auditory-evoked potentials in the diagnosis and monitoring of a child with Landau-Kleffner syndrome
    Erin Plyler
    Department of Audiology and Speech Pathology, University of Tennessee Health Science Center
    J Am Acad Audiol 24:564-71. 2013
    ..He saw multiple medical professionals across 2 yr. By almost 5 yr, his vocabulary diminished from 50 words to 4, and he was referred to our speech and hearing center...
  96. ncbi Landau-Kleffner syndrome
    Abdelkarim A Al-Qudah
    Pediatric Department, Jordan University Hospital, PO Box 13407, Amman, Jordan Tel 962 79566067 Fax 962 65353388 E mail
    Neurosciences (Riyadh) 9:80-3. 2004
    ..Antiepileptic drugs can easily control seizures, but language recovery is more difficult. Autoimmune etiology is considered likely and long-term prognosis remains variable...
  97. ncbi Sparse MEG source imaging in Landau-Kleffner syndrome
    Min Zhu
    School of Electrical and Computer Engineering, University of Oklahoma, Norman, OK 73019, USA
    Conf Proc IEEE Eng Med Biol Soc 2011:4909-12. 2011
    ..It can further be applied to non-invasively localize and/or lateralize eloquent cortex for language for epilepsy patients in general in the future...
  98. ncbi Cognitive disorders in pediatric epilepsy
    I Jambaque
    INSERM U663 Université Paris Descartes, Institut de Psychologie, Paris, France Fondation Rothschild, unité de neurochirurgie pédiatrique, Paris, France Electronic address
    Handb Clin Neurol 111:691-5. 2013
    ..These observations militate in favor of individual neuropsychological assessments as well as early interventions in order to provide the child with an optimal individualized treatment program...
  99. ncbi Functional imaging: PET
    Ajay Kumar
    Departments of Pediatrics and Neurology, School of Medicine, Wayne State University, and PET Center, Children s Hospital of Michigan, Detroit, MI, USA
    Handb Clin Neurol 111:767-76. 2013
    ..Finally, PET can also be used to evaluate various cognitive processes and their underlying neurological substrates and can help in addressing the issue of brain plasticity and reorganization, related to epilepsy...
  100. ncbi [Electroencephalography in status epilepticus in sleep (ESES) in various clinical pictures]
    Ljiljana Popović Miocinović
    Specijalna bolnica za zastitu djece s neurorazvojnim i motorickim smetnjama, Zagreb, Hrvatska
    Acta Med Croatica 59:69-74. 2005
    ..Clinical course could be modulated by different factors. Continuous following over a long period could be helpful in the classification of epilepsy...
  101. ncbi Sleep in patients with epilepsy
    Shang Yeong Kwan
    Neurological Institute, Taipei Veterans General Hospital, Taiwan National Yang Ming University School of Medicine, Taipei, Taiwan
    Acta Neurol Taiwan 20:229-131. 2011
    ..Indeed, the complex relationship between epilepsy and sleep disorder must be addressed in order to provide the best management of sleep disturbance in PWE...

Research Grants4

  1. Teaching Nonverbal Children with Autism Useful Speech
    Sally Rogers; Fiscal Year: 2003
    ..4) Finally, a short-term pilot study using random assignment of matched participants and single subject design will be conducted to examine efficacy of each approach. ..
  2. SEMANTICS, DISCOURSE MODELS, AND THE RIGHT HEMISPHERE
    Kathleen Baynes; Fiscal Year: 2005
    ..abstract_text> ..
  3. Definition and Development of the Phenotype of Autism
    Sally Rogers; Fiscal Year: 2006
    ....
  4. fMRI and DTI in Children with PVH/PVL
    Heidi M Feldman; Fiscal Year: 2010
    ..It will also contribute to understanding the impact of white matter injury and the development of cortical plasticity after early brain injuries. ..