LKS
MCID: LND001
MIFTS: 44

Landau-Kleffner Syndrome (LKS) malady

Rare diseases, Mental diseases, Neuronal diseases categories
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Summaries for Landau-Kleffner Syndrome

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NIH Rare Diseases:42 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 5 and 7. 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, mental retardation, 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 early. some affected children may have a permanent severe language disorder, while others may regain much of their language abilities (although it may take months or years). last updated: 12/8/2008

MalaCards based summary: Landau-Kleffner Syndrome, also known as acquired epileptiform aphasia, is related to autistic disorder and aphasia. An important gene associated with Landau-Kleffner Syndrome is UBIAD1 (UbiA prenyltransferase domain containing 1), and among its related pathways are FSH signaling pathway and Spinal Cord Injury. The compounds 3-nitropropionic acid and glatiramer acetate have been mentioned in the context of this disorder. Affiliated tissues include brain, temporal lobe and testes, and related mouse phenotypes are cellular and normal.

NINDS:43 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, mental retardation, childhood schizophrenia, or emotional/behavioral problems.

Wikipedia:65 Landau?Kleffner syndrome (LKS) ? also called infantile acquired aphasia, acquired epileptic aphasia or... more...

Description from OMIM:46 245570

Aliases & Classifications for Landau-Kleffner Syndrome

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Landau-Kleffner Syndrome, Aliases & Descriptions:

Name: Landau-Kleffner Syndrome 8 42 43 10 62
Acquired Epileptiform Aphasia 42 43 62
Acquired Aphasia with Convulsive Disorder 42 62
 
Aphasia, Acquired Epileptic 62
Acquired Epileptic Aphasia 8
Lks 42


Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Rare diseases
Anatomical: Mental diseases, Neuronal diseases


External Ids:

Disease Ontology8 DOID:2538
MeSH34 D018887
OMIM46 245570
NCIt39 C84806
ICD1025 F80.3

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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Clinical features from OMIM:

245570

Drugs & Therapeutics for Landau-Kleffner Syndrome

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Drug clinical trials:

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Search NIH Clinical Center for 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:

32
Brain, Temporal lobe, Testes, Cortex

Animal Models for Landau-Kleffner Syndrome or affiliated genes

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MGI Mouse Phenotypes related to Landau-Kleffner Syndrome:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053848.4BDNF, NDUFS4, MBP, RPS6KB2
2MP:00028738.1BDNF, NDUFS4, MBP, RPS6KB2

Publications for Landau-Kleffner Syndrome

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Articles related to Landau-Kleffner Syndrome:

(show top 50)    (show all 165)
idTitleAuthorsYear
1
Temporoparietal resection in a patient with Landau-Kleffner syndrome. (25149936)
2014
2
Epidemiological study of Landau-Kleffner syndrome (LKS) in Japan. (23706409)
2013
3
Epileptic encephalopathy with continuous spike-waves during slow-wave sleep including Landau-Kleffner syndrome. (23622211)
2013
4
Landau-Kleffner syndrome in Norway: long-term prognosis and experiences with the health services and educational systems. (21514895)
2011
5
Landau-Kleffner syndrome: a rare auditory processing disorder series of cases and review of the literature. (21074868)
2011
6
Landau-Kleffner syndrome: long-term follow-up. (18930363)
2009
7
Acquired epileptic aphasia: Landau-Kleffner syndrome. (21887181)
2009
8
Landau-Kleffner syndrome: 50 years after. (19682039)
2009
9
Sign language in Landau-Kleffner syndrome. (19682058)
2009
10
When epilepsy interferes with word comprehension: findings in Landau-Kleffner syndrome. (18184944)
2008
11
Possible involvement of the tip of temporal lobe in Landau-Kleffner syndrome. (17383838)
2007
12
Neuropsychological findings in Rolandic epilepsy and Landau-Kleffner syndrome. (17105467)
2006
13
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
14
Landau-Kleffner syndrome, electrical status epilepticus in slow wave sleep, and language regression in children. (15362173)
2004
15
Electroencephalography findings in autism: similarities and differences from Landau-Kleffner syndrome. (15575417)
2004
16
Landau-Kleffner syndrome. (23377356)
2004
17
Landau-Kleffner syndrome responsive to levetiracetam. (14527502)
2003
18
The Landau-Kleffner syndrome. (11993733)
2002
19
Efficacy of intravenous immunoglobulin in Landau-Kleffner syndrome. (11992758)
2002
20
Landau-Kleffner Syndrome. (15309185)
2001
21
The Landau-Kleffner Syndrome. (15309183)
2001
22
Landau-Kleffner Syndrome: Localization of Epileptogenic Lesion Using Wavelet- Cross-Correlation Analysis. (12609372)
2001
23
Sign language in childhood epileptic aphasia (Landau-Kleffner syndrome). (11730147)
2001
24
Theory and testing in Landau-Kleffner Syndrome. (11844720)
2001
25
Pathophysiological accounts of language impairments in Landau-Kleffner syndrome. (11679310)
2001
26
Effects of high-dose intravenous corticosteroid therapy in Landau-Kleffner syndrome. (10738922)
2000
27
Magnetoencephalography in children with Landau-Kleffner syndrome and acquired epileptic aphasia. (10696012)
2000
28
Spindles-inducing mechanism modulates sleep activation of interictal epileptiform discharges in the Landau-Kleffner syndrome. (10691117)
2000
29
Landau-Kleffner syndrome: relation of clinical, EEG and Tc-99m-HMPAO brain SPECT findings and improvement in EEG after treatment. (10656276)
1999
30
Language outcome following multiple subpial transection for Landau-Kleffner syndrome. (10094262)
1999
31
Language disorders in Landau-Kleffner syndrome. (10073434)
1999
32
Landau Kleffner syndrome. (10216604)
1998
33
Chronic auditory agnosia following Landau-Kleffner syndrome: a 23 year outcome study. (9672766)
1998
34
Landau-Kleffner syndrome: consistent response to repeated intravenous gamma-globulin doses: a case report. (9118856)
1997
35
Discrimination of speech sounds in a boy with Landau-Kleffner syndrome: an intraoperative event-related potential study. (8892373)
1996
36
Multiple subpial transection for Landau-Kleffner syndrome. (8660018)
1996
37
Brain single photon emission computed tomography imaging in Landau-Kleffner syndrome. (8603627)
1996
38
Linguistic development in a patient with Landau-Kleffner syndrome: a nine-year follow-up. (7791945)
1995
39
Sounds triggers spikes in the Landau-Kleffner syndrome. (7519634)
1994
40
Landau-Kleffner syndrome: acquired epileptic aphasia in children. (7514057)
1994
41
Frequency modulation analysis in children with Landau-Kleffner syndrome. (8323151)
1993
42
Developmental dysphasias, epilepsy, and Landau-Kleffner syndrome. (7504408)
1993
43
Landau-Kleffner syndrome: epileptic activity in the auditory cortex. (1716495)
1991
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
Acquired childhood aphasia with convulsive disorder (Landau-Kleffner syndrome). A case report. (1689510)
1990
47
Landau-Kleffner syndrome. EEG topographic studies. (2466418)
1989
48
Landau-Kleffner syndrome: a case study with a fourteen-year follow-up. (2468500)
1989
49
Landau-Kleffner syndrome--epileptic aphasia in children--possible role of toxoplasma gondii infection. (2479399)
1988-1989
50
The Landau-Kleffner syndrome of acquired epileptic aphasia: unusual clinical outcome, surgical experience, and absence of encephalitis. (2447519)
1988

Variations for Landau-Kleffner Syndrome

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Expression for genes affiliated with Landau-Kleffner Syndrome

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Expression patterns in normal tissues for genes affiliated with Landau-Kleffner Syndrome

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

Pathways for genes affiliated with Landau-Kleffner Syndrome

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Pathways related to Landau-Kleffner Syndrome according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.4BDNF, RPS6KB2
29.3BDNF, MBP

Compounds for genes affiliated with Landau-Kleffner Syndrome

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Sources:
44Novoseek, 11DrugBank, 61Tocris Bioscience, 28IUPHAR, 24HMDB
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Compounds related to Landau-Kleffner Syndrome according to GeneCards/GeneDecks:

(show all 21)
idCompoundScoreTop Affiliating Genes
13-nitropropionic acid449.7BDNF, NDUFS4
2glatiramer acetate44 1110.7BDNF, MBP
31 methyl 4 phenylpyridinium449.7BDNF, NDUFS4
46-hydroxydopamine449.6BDNF, NDUFS4
5mptp449.6BDNF, NDUFS4
6tetrodotoxin44 61 2811.6BDNF, MBP
7melatonin44 28 61 24 1113.6BDNF, NDUFS4
8silver449.5BDNF, MBP
9levodopa44 1110.5NDUFS4, BDNF
10corticosterone44 61 2411.5BDNF, MBP
11kainate44 2810.4BDNF, MBP
12choline44 24 1111.3BDNF, MBP
13cocaine44 1110.2MBP, BDNF
14n acetylcysteine449.1MBP, NDUFS4, BDNF
15norepinephrine44 24 1111.1BDNF, NDUFS4, MBP
16lactate449.1MBP, NDUFS4, BDNF
17h2o2449.0MBP, NDUFS4, BDNF
18threonine449.0RPS6KB2, MBP, NDUFS4
19inositol449.0BDNF, MBP
20glutamate448.8BDNF, NDUFS4, MBP
21serine448.6RPS6KB2, MBP, NDUFS4, BDNF

GO Terms for genes affiliated with Landau-Kleffner Syndrome

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Biological processes related to Landau-Kleffner Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1mitochondrial electron transport, NADH to ubiquinoneGO:0061209.3BDNF, NDUFS4

Products for genes affiliated with Landau-Kleffner Syndrome

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  • Antibodies
  • Proteins
  • Lysates

Sources for Landau-Kleffner Syndrome

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet