MCID: LND001
MIFTS: 36

Landau-Kleffner Syndrome malady

Categories: Rare diseases, Neuronal diseases

Aliases & Classifications for Landau-Kleffner Syndrome

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Sources:
10Disease Ontology, 45NIH Rare Diseases, 46NINDS, 12DISEASES, 51Orphanet, 36MeSH, 65UMLS, 27ICD10, 42NCIt, 59SNOMED-CT, 37MESH via Orphanet, 66UMLS via Orphanet
See all MalaCards sources

Aliases & Descriptions for Landau-Kleffner Syndrome:

Name: Landau-Kleffner Syndrome 10 45 46 12 51 36 65
Acquired Epileptic Aphasia 10 45 51
Acquired Epileptiform Aphasia 45 46
 
Lks 45 51
Acquired Aphasia with Convulsive Disorder 45

Characteristics:

Orphanet epidemiological data:

51
landau-kleffner syndrome:
Inheritance: Autosomal dominant; Age of onset: Childhood; Age of death: any age

Classifications:



External Ids:

Disease Ontology10 DOID:2538
ICD1027 G40.8
MeSH36 D018887
NCIt42 C84806
Orphanet51 98818
MESH via Orphanet37 D018887
UMLS via Orphanet66 C0282512
UMLS65 C0282512

Summaries for Landau-Kleffner Syndrome

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NIH Rare Diseases:45 Landau-kleffner syndrome (lks) is a rare, childhood neurological disorder characterized by the sudden or gradual development of aphasia (the inability to understand or express language) and an abnormal electro-encephalogram (eeg). the disorder usually occurs in children between age 2 and 8. typically, children with lks develop normally but then lose their language skills for no apparent reason. while many of the affected individuals have seizures, some do not. the disorder is difficult to diagnose and may be misdiagnosed as autism, pervasive developmental disorder, hearing impairment, learning disability, auditory/verbal processing disorder, attention deficit disorder, intellectual disability, childhood schizophrenia, or emotional/behavioral problems. treatment for lks usually consists of medications, such as anticonvulsants and corticosteroids, and speech therapy, which should be started promptly. the prognosis varies. some children may have a permanent language disorder, while others may regain much of their language abilities (although it may take months or years). last updated: 4/13/2015

MalaCards based summary: Landau-Kleffner Syndrome, also known as acquired epileptic aphasia, is related to epilepsy, focal, with speech disorder and with or without mental retardation and aphasia, and has symptoms including convulsion in childhood, seizures, focal and myoclonus, eyelid. An important gene associated with Landau-Kleffner Syndrome is GRIN2A (Glutamate Ionotropic Receptor NMDA Type Subunit 2A). Affiliated tissues include brain, endothelial and thyroid, and related mouse phenotype integument.

NINDS:46 Landau-Kleffner syndrome (LKS) is a rare, childhood neurological disorder characterized by the sudden or gradual development of aphasia (the inability to understand or express language) and an abnormal electro-encephalogram (EEG). LKS affects the parts of the brain that control comprehension and speech. The disorder usually occurs in children between the ages of 5 and 7 years. Typically, children with LKS develop normally but then lose their language skills for no apparent reason. While many of the affected individuals have seizures, some do not. The disorder is difficult to diagnose and may be misdiagnosed as autism, pervasive developmental disorder, hearing impairment, learning disability, auditory/verbal processing disorder, attention deficit disorder, childhood schizophrenia, or emotional/behavioral problems.

Wikipedia:68 Landau–Kleffner syndrome (LKS)—also called infantile acquired aphasia, acquired epileptic aphasia or... more...

Related Diseases for Landau-Kleffner Syndrome

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Graphical network of the top 20 diseases related to Landau-Kleffner Syndrome:



Diseases related to landau-kleffner syndrome

Symptoms for Landau-Kleffner Syndrome

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UMLS symptoms related to Landau-Kleffner Syndrome:


convulsion in childhood, seizures, focal, myoclonus, eyelid, specific learning problem, seizures, tonic, epileptic aura, seizures

Drugs & Therapeutics for Landau-Kleffner Syndrome

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Interventional clinical trials:

idNameStatusNCT IDPhase
1The Rolandic Epilepsy/ESES/Landau-Kleffner Syndrome and Correlation With Language Impairment StudyCompletedNCT01335425
2Genetic Basis of Idiopathic Focal Epilepsies With Cognitif DeficitsNot yet recruitingNCT00851331

Search NIH Clinical Center for Landau-Kleffner Syndrome


Cochrane evidence based reviews: landau-kleffner syndrome

Genetic Tests for Landau-Kleffner Syndrome

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Anatomical Context for Landau-Kleffner Syndrome

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MalaCards organs/tissues related to Landau-Kleffner Syndrome:

33
Brain, Endothelial, Thyroid, Breast, Prostate, Testes, Pituitary

Animal Models for Landau-Kleffner Syndrome or affiliated genes

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MGI Mouse Phenotypes related to Landau-Kleffner Syndrome:

38
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00107718.7GRIN2A, ITSN2, NDUFS4, POMC

Publications for Landau-Kleffner Syndrome

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Articles related to Landau-Kleffner Syndrome:

(show top 50)    (show all 177)
idTitleAuthorsYear
1
In response: Comment on outcome following multiple subpial transection in Landau-Kleffner syndrome and related regression. (27041136)
2016
2
Response to immunotherapy in a patient with Landau-Kleffner syndrome and GRIN2A mutation. (26806548)
2016
3
Comment on outcome following multiple subpial transection in Landau-Kleffner syndrome and related regression. (27041135)
2016
4
Landau-Kleffner syndrome: an uncommon dealt with case in Southeast Asia. (26420702)
2015
5
Evidence for normal letter-sound integration, but altered language pathways in a case of recovered Landau-Kleffner Syndrome. (26232266)
2015
6
Landau-Kleffner syndrome. (26627294)
2015
7
Temporoparietal resection in a patient with Landau-Kleffner syndrome. (25149936)
2014
8
Epidemiological study of Landau-Kleffner syndrome (LKS) in Japan. (23706409)
2013
9
Epileptic encephalopathy with continuous spike-waves during slow-wave sleep including Landau-Kleffner syndrome. (23622211)
2013
10
Landau-Kleffner syndrome in Norway: long-term prognosis and experiences with the health services and educational systems. (21514895)
2011
11
Landau-Kleffner syndrome: a rare auditory processing disorder series of cases and review of the literature. (21074868)
2011
12
Landau-Kleffner syndrome: long-term follow-up. (18930363)
2009
13
Acquired epileptic aphasia: Landau-Kleffner syndrome. (21887181)
2009
14
Landau-Kleffner syndrome: 50 years after. (19682039)
2009
15
When epilepsy interferes with word comprehension: findings in Landau-Kleffner syndrome. (18184944)
2008
16
Possible involvement of the tip of temporal lobe in Landau-Kleffner syndrome. (17383838)
2007
17
Neuropsychological findings in Rolandic epilepsy and Landau-Kleffner syndrome. (17105467)
2006
18
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. (16617025)
2006
19
Landau-Kleffner syndrome, electrical status epilepticus in slow wave sleep, and language regression in children. (15362173)
2004
20
Electroencephalography findings in autism: similarities and differences from Landau-Kleffner syndrome. (15575417)
2004
21
Landau-Kleffner syndrome responsive to levetiracetam. (14527502)
2003
22
The Landau-Kleffner syndrome. (11993733)
2002
23
Efficacy of intravenous immunoglobulin in Landau-Kleffner syndrome. (11992758)
2002
24
Landau-Kleffner Syndrome. (15309185)
2001
25
The Landau-Kleffner Syndrome. (15309183)
2001
26
Landau-Kleffner Syndrome: Localization of Epileptogenic Lesion Using Wavelet- Cross-Correlation Analysis. (12609372)
2001
27
Sign language in childhood epileptic aphasia (Landau-Kleffner syndrome). (11730147)
2001
28
Effects of high-dose intravenous corticosteroid therapy in Landau-Kleffner syndrome. (10738922)
2000
29
Magnetoencephalography in children with Landau-Kleffner syndrome and acquired epileptic aphasia. (10696012)
2000
30
Landau-Kleffner syndrome: relation of clinical, EEG and Tc-99m-HMPAO brain SPECT findings and improvement in EEG after treatment. (10656276)
1999
31
Language outcome following multiple subpial transection for Landau-Kleffner syndrome. (10094262)
1999
32
Language disorders in Landau-Kleffner syndrome. (10073434)
1999
33
Landau Kleffner syndrome. (10216604)
1998
34
Chronic auditory agnosia following Landau-Kleffner syndrome: a 23 year outcome study. (9672766)
1998
35
Landau-Kleffner syndrome: consistent response to repeated intravenous gamma-globulin doses: a case report. (9118856)
1997
36
Discrimination of speech sounds in a boy with Landau-Kleffner syndrome: an intraoperative event-related potential study. (8892373)
1996
37
Multiple subpial transection for Landau-Kleffner syndrome. (8660018)
1996
38
Brain single photon emission computed tomography imaging in Landau-Kleffner syndrome. (8603627)
1996
39
Linguistic development in a patient with Landau-Kleffner syndrome: a nine-year follow-up. (7791945)
1995
40
Sounds triggers spikes in the Landau-Kleffner syndrome. (7519634)
1994
41
Landau-Kleffner syndrome: acquired epileptic aphasia in children. (7514057)
1994
42
Frequency modulation analysis in children with Landau-Kleffner syndrome. (8323151)
1993
43
Developmental dysphasias, epilepsy, and Landau-Kleffner syndrome. (7504408)
1993
44
Landau-Kleffner syndrome: a clinical and EEG study of five cases. (1700952)
1990
45
Cerebral glucose utilization during sleep in Landau-Kleffner syndrome: a PET study. (1700954)
1990
46
Landau-Kleffner syndrome. EEG topographic studies. (2466418)
1989
47
Landau-Kleffner syndrome: a case study with a fourteen-year follow-up. (2468500)
1989
48
Landau-Kleffner syndrome--epileptic aphasia in children--possible role of toxoplasma gondii infection. (2479399)
1988-1989
49
The Landau-Kleffner syndrome of acquired epileptic aphasia: unusual clinical outcome, surgical experience, and absence of encephalitis. (2447519)
1988
50

Variations for Landau-Kleffner Syndrome

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Expression for genes affiliated with Landau-Kleffner Syndrome

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Search GEO for disease gene expression data for Landau-Kleffner Syndrome.

Pathways for genes affiliated with Landau-Kleffner Syndrome

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GO Terms for genes affiliated with Landau-Kleffner Syndrome

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Sources for Landau-Kleffner Syndrome

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2CDC
14ExPASy
15FDA
16FMA
24GTR
25HGMD
26HMDB
27ICD10
28ICD10 via Orphanet
29ICD9CM
30IUPHAR
31KEGG
34MedGen
36MeSH
37MESH via Orphanet
38MGI
41NCI
42NCIt
43NDF-RT
46NINDS
47Novoseek
49OMIM
50OMIM via Orphanet
54PubMed
55QIAGEN
60SNOMED-CT via Orphanet
64Tumor Gene Family of Databases
65UMLS
66UMLS via Orphanet