Landau-Kleffner Syndrome

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)

Also Known As:

Networked: 69 relevant articles (6 outcomes, 8 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Epilepsy (Aura)
2.	Malformations of Cortical Development
3.	Tuberous Sclerosis (Bourneville's Disease)
4.	Sturge-Weber Syndrome (Sturge-Kalischer-Weber Syndrome)
5.	Infantile Spasms (West Syndrome)

Experts

1.	Kim, Heung Dong: 3 articles (04/2008 - 08/2006)
2.	Catsman-Berrevoets, Coriene E: 2 articles (09/2015 - 08/2009)
3.	Devinsky, Orrin: 2 articles (10/2014 - 01/2008)
4.	Van Bogaert, Patrick: 2 articles (08/2013 - 08/2009)
5.	Dulac, Olivier: 2 articles (08/2009 - 11/2003)
6.	Slama, Abdelhamid: 2 articles (04/2008 - 01/2007)
7.	Lee, Joon Soo: 2 articles (04/2008 - 01/2007)
8.	Kang, Hoon-Chul: 2 articles (01/2007 - 08/2006)
9.	Neuteboom, Rinze F: 1 article (09/2015)
10.	Bakker, Dewi P: 1 article (09/2015)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Landau-Kleffner Syndrome:

1.	Immunoglobulins (Immunoglobulin)IBA 04/01/2002 - "Efficacy of intravenous immunoglobulin in Landau-Kleffner syndrome." 08/01/2009 - "This study reports results of therapy with immunoglobulin in children with Landau-Kleffner syndrome (LKS) or the syndrome of continuous spikes and waves during sleep (CSWS syndrome). " 07/01/2000 - "Successful use of intravenous immunoglobulin as initial monotherapy in Landau-Kleffner syndrome." 07/01/2000 - "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. "
2.	Adrenocorticotropic Hormone (ACTH)FDA Link 11/30/2008 - "On the basis of our data ACTH is an effective treatment for Landau-Kleffner syndrome. " 11/30/2008 - "In our study we aimed to investigate the efficacy of ACTH therapy in Landau-Kleffner syndrome. " 11/30/2008 - "We have analysed retrospectively the documentation of five children treated by ACTH, who suffered from Landau-Kleffner syndrome. " 12/01/2014 - "ESES was resolved by sodium valproate, clonazepam, and acetazolamide in Patient 1, and by adrenocorticotropic hormone, sodium valproate, and clorazepate in Patient 2. The present cases had the unique cognitive dysfunction of Kanji dysgraphia, which is distinct from that of Landau-Kleffner syndrome and continuous spikes and waves during slow-wave sleep. "
3.	Lennox-Gastaut type Epileptic encephalopathyIBA 07/01/2008 - "Of 41 patients, 32 had Lennox-Gastaut syndrome, 4 had Doose syndrome, 1 had Otahara syndrome, 2 had Landau-Kleffner syndrome, and 2 had other unspecified generalized epilepsies. " 12/01/1995 - "The last three syndromes discussed (West syndrome, Lennox-Gastaut syndrome, and acquired epileptic aphasia) are devastating catastrophic epileptic syndromes. " 12/01/2014 - "Responder rates were 11 of 15 (73%) in patients with continuous spike-waves in slow sleep (CSWS) or Landau-Kleffner syndrome, 2 of 6 in patients with myoclonic astatic epilepsy or Lennox-Gastaut syndrome, and 17 of 31 (55%) in patients with unclassified epilepsies. " 11/01/2008 - "This includes syndromes with multiple etiologies, including Lennox-Gastaut syndrome and infantile spasms; developmental syndromes of unknown etiology, such as Landau-Kleffner syndrome; and idiopathic epilepsies, such as myoclonic-astatic (Doose) epilepsy. " 01/01/2012 - "These syndromes include early myoclonic encephalopathy and Ohtahara syndrome in the neonatal period, West syndrome and Dravet syndrome in infancy, myoclonic status in nonprogressive encephalopathies, and Lennox-Gastaut syndrome, Landau-Kleffner syndrome, and epilepsy with continuous spike waves during slow wave sleep in childhood and adolescences. "
4.	Intravenous Immunoglobulins (IVIG)FDA Link 04/01/2002 - "This finding suggests that IVIG has at least some efficacy for the therapy of Landau-Kleffner syndrome." 08/01/2009 - "Landau-Kleffner syndrome and CSWS syndrome: treatment with intravenous immunoglobulins." 07/01/2000 - "Repeated, immediate, and remarkable clinical and EEG responses of this patient suggest that IVIG was helpful as first-line therapy in the treatment of Landau-Kleffner syndrome. " 11/01/1998 - "IVIG in Landau-Kleffner syndrome." 02/01/1998 - "Successful use of intravenous immunoglobulins in Landau-Kleffner syndrome."
5.	Adrenal Cortex Hormones (Corticosteroids)IBA 05/01/2005 - "Corticosteroids are a safe and effective treatment for patients with Landau-Kleffner syndrome and CSWS. " 05/01/2005 - "Corticosteroids should be considered as a treatment option in children with Landau-Kleffner syndrome and CSWS." 05/01/2005 - "We describe our experience in treating patients with Landau-Kleffner syndrome and CSWS with corticosteroids. " 05/01/2005 - "Corticosteroids for the treatment of Landau-kleffner syndrome and continuous spike-wave discharge during sleep." 10/01/2014 - "Episodic epileptic verbal auditory agnosia in Landau Kleffner syndrome treated with combination diazepam and corticosteroids."
6.	BenzodiazepinesIBA 10/01/2014 - "These observations suggest that pulse combination high-dose corticosteroid and benzodiazepine therapy may be especially effective in Landau-Kleffner syndrome. " 11/16/1999 - "Early acquired epileptic aphasia treatment is advisable with valproic acid, benzodiazepines or ethosuximide alone or in combinations, supplementary with corticoids for at least six months in the absence of clinical response and/or EEG improvement. "
7.	Glucose (Dextrose)FDA LinkGeneric 03/01/1995 - "In order to investigate differences in glucose metabolic patterns between awake and sleep states in two children with Landau-Kleffner syndrome and continuous spike-waves during slow-wave sleep, fluorodeoxyglucose positron-emission tomographic (PET) studies were performed in each state. " 11/01/1990 - "Cerebral glucose utilization during sleep in Landau-Kleffner syndrome: a PET study." 11/01/1997 - "We have evaluated 17 children (aged 2.4 to 10.6 yr) with Landau-Kleffner syndrome using positron emission tomography (PET) with 2-deoxy-2-[18F]fluoro-D-glucose (FDG) in order to determine whether there are metabolic abnormalities common to this syndrome. " 11/01/1990 - "Our data demonstrate that in Landau-Kleffner syndrome, cerebral glucose utilization is not normal during sleep. " 03/01/1994 - "Regional brain glucose utilisation was investigated with PET and fluorodeoxyglucose (FDG) in a case of epileptic aphasia (Landau-Kleffner syndrome) associated with a left sylvian arachnoid cyst. "
8.	SteroidsIBA 08/01/2009 - "Hence, 20 patients (45.4%) were long-term responders after a single but prolonged trial of steroids, including all four cases of Landau-Kleffner syndrome. " 01/01/1996 - "While steroids are accepted as an effective therapy for infantile spasms, their role in the treatment of the Landau-Kleffner syndrome is far less clear. " 01/01/2015 - "However, EEG was abnormal and the boy was diagnosed as a case of Landau-Kleffner syndrome (LKS) and treated with sodium valproate, levetiracetam and steroids. "
9.	Prednisolone (Predate)FDA LinkGeneric 09/01/2015 - "In this retrospective observational study, 26 children diagnosed with epilepsy with refractory seizures other than West syndrome and Landau-Kleffner syndrome were eligible for a treatment regimen consisting of three days intravenous methylprednisolone (20 mg per kilogram per day) followed by twelve weeks oral prednisolone (0.5 mg per kilogram on alternate days), concluded with a taper phase. "
10.	MethylprednisoloneFDA LinkGeneric 09/01/2015 - "In this retrospective observational study, 26 children diagnosed with epilepsy with refractory seizures other than West syndrome and Landau-Kleffner syndrome were eligible for a treatment regimen consisting of three days intravenous methylprednisolone (20 mg per kilogram per day) followed by twelve weeks oral prednisolone (0.5 mg per kilogram on alternate days), concluded with a taper phase. "

Therapies and Procedures

1.	Aftercare (After-Treatment) 12/01/1999 - "Landau-Kleffner syndrome: relation of clinical, EEG and Tc-99m-HMPAO brain SPECT findings and improvement in EEG after treatment."
2.	Sign Language 06/01/2001 - "Accessibility of spoken, written, and sign language in Landau-Kleffner syndrome: a linguistic and functional MRI study." 08/01/2009 - "Sign language in Landau-Kleffner syndrome." 08/01/2009 - "This article reviews the history of sign language (SL) and the rationale for its use in children with profound auditory agnosia due to Landau-Kleffner syndrome (LKS), illustrated by studies of children and adults followed for many years and rare cases from the literature. " 04/01/2004 - "The use of sign language in the treatment of patients with verbal aphasia is considered, especially of children with the Landau-Kleffner syndrome, but therapy of this kind can be used in children with delayed language development, and in other types of acquired aphasia at any age. " 11/01/2001 - "Sign language in childhood epileptic aphasia (Landau-Kleffner syndrome)."
3.	Ketogenic Diet 08/01/2009 - "Rational treatment options with AEDs and ketogenic diet in Landau-Kleffner syndrome: still waiting after all these years." 08/01/2006 - "Landau-Kleffner syndrome with mitochondrial respiratory chain-complex I deficiency was controlled in this case by ketogenic diet and supplementary mitochondrial cocktail therapy." 11/01/1999 - "This is the first reported series of patients with acquired epileptic aphasia successfully treated with the ketogenic diet, and suggests a new therapeutic alternative for patients with this often difficult-to-treat disorder." 11/01/1999 - "We describe three patients with acquired epileptic aphasia refractory to traditional treatments who were successfully treated with the ketogenic diet. " 11/01/1999 - "Treatment of acquired epileptic aphasia with the ketogenic diet."
4.	Vagus Nerve Stimulation 06/01/2003 - "The effects of vagus nerve stimulation therapy on patients with intractable seizures and either Landau-Kleffner syndrome or autism."
5.	General Anesthesia 01/01/2010 - "General anesthesia for a child with Landau-Kleffner syndrome, a case report."