LKS
MCID: LND001
MIFTS: 37

Landau-Kleffner Syndrome (LKS) malady

Neuronal category

Summaries for Landau-Kleffner Syndrome

Sources:
43NIH Rare Diseases, 44NINDS, 64Wikipedia, 47OMIM, 33MalaCards
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NIH Rare Diseases: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). 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: Landau-Kleffner Syndrome, also known as acquired epileptiform aphasia, is related to autistic disorder and brain disease. An important gene associated with Landau-Kleffner Syndrome is SRPX2 (sushi-repeat containing protein, X-linked 2), and among its related pathways is FSH signaling pathway. The compounds 3-nitropropionic acid and 1 methyl 4 phenylpyridinium have been mentioned in the context of this disorder. Affiliated tissues include brain, cortex and temporal lobe.

NINDS:44 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:64 Landau–Kleffner syndrome (LKS) — also called infantile acquired aphasia, acquired epileptic aphasia... more...

Description from OMIM:47 245570

Aliases & Classifications for Landau-Kleffner Syndrome

Sources:
8Disease Ontology, 43NIH Rare Diseases, 44NINDS, 10DISEASES, 61UMLS, 57SNOMED-CT, 40NCIt, 47OMIM, 35MeSH, 25ICD10
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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Anatomical: Neuronal


Aliases & Descriptions:

landau-kleffner syndrome 8 43 44 10 61
acquired epileptiform aphasia 43 44
acquired aphasia with convulsive disorder 43
acquired epileptic aphasia 8
lks 43


External Ids:

Disease Ontology8 DOID:2538
NCIt40 C84806
OMIM47 245570
MeSH35 D018887
ICD1025 F80.3

Related Diseases for Landau-Kleffner Syndrome

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17GeneCards, 18GeneDecks
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Graphical network of the top 20 diseases related to Landau-Kleffner Syndrome:



Diseases related to landau-kleffner syndrome

Clinical Features for Landau-Kleffner Syndrome

Sources:
47OMIM
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Clinical features from OMIM:

245570

Drugs & Therapeutics for Landau-Kleffner Syndrome

Sources:
5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials
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Approved drugs:

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Drug clinical trials:

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Genetic Tests for Landau-Kleffner Syndrome

Anatomical Context for Landau-Kleffner Syndrome

Sources:
33MalaCards
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MalaCards organs/tissues related to Landau-Kleffner Syndrome:

33
Brain, Cortex, Temporal lobe

Animal Models for Landau-Kleffner Syndrome or affiliated genes

Sources:
28inGenious Targeting Laboratory
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Publications for Landau-Kleffner Syndrome

Sources:
51PubMed
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Articles related to Landau-Kleffner Syndrome:

(show top 50)    (show all 162)
idTitleAuthorsYear
1
Landau-Kleffner syndrome: A study of 29 patients. (24315829)
2014
2
Rehabilitation issues in Landau-Kleffner syndrome. (23030303)
2012
3
Lead poisoning in two children with Landau-Kleffner syndrome. (22578276)
2012
4
Sparse MEG source imaging in Landau-Kleffner syndrome. (22255439)
2011
5
Acquired epileptic aphasia: Landau-Kleffner syndrome. (21887181)
2009
6
Landau-Kleffner syndrome: 50 years after. (19682039)
2009
7
The Landau-Kleffner syndrome. (19682040)
2009
8
Fifty years of Landau-Kleffner syndrome. Proceedings of an international symposium. November 2-4, 2007. Alden-Biesen, Belgium. (19746560)
2009
9
When epilepsy interferes with word comprehension: findings in Landau-Kleffner syndrome. (18184944)
2008
10
Possible involvement of the tip of temporal lobe in Landau-Kleffner syndrome. (17383838)
2007
11
Linking autism, regression and Landau-Kleffner syndrome: integrative role of nerve growth factor. (17157996)
2007
12
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
13
Landau-Kleffner syndrome with mitochondrial respiratory chain-complex I deficiency. (16876018)
2006
14
Converging evidences on language impairment in Landau-Kleffner Syndrome revealed by behavioral and brain activity measures: a case study. (16386952)
2006
15
Perisylvian polymicrogyria in Landau-Kleffner syndrome. (15883344)
2005
16
Hemispheric integrative therapy in Landau-Kleffner syndrome: applications for rehabilitation sciences. (16040364)
2005
17
Landau-Kleffner syndrome, electrical status epilepticus in slow wave sleep, and language regression in children. (15362173)
2004
18
Epileptic encephalopathy of late childhood: Landau-Kleffner syndrome and the syndrome of continuous spikes and waves during slow-wave sleep. (14734935)
2003
19
Landau-Kleffner syndrome beginning with stuttering: case report. (12546439)
2002
20
Treatment strategies in Landau-Kleffner syndrome and paraictal psychiatric and cognitive disturbances. (12609317)
2002
21
Landau-Kleffner syndrome: a case report. (12134193)
2002
22
Landau Kleffner syndrome: electroclinical and etiopathogenic heterogeneity. (12577088)
2002
23
Landau-Kleffner Syndrome. (15309185)
2001
24
Landau-Kleffner Syndrome: Localization of Epileptogenic Lesion Using Wavelet- Cross-Correlation Analysis. (12609372)
2001
25
Sign language in childhood epileptic aphasia (Landau-Kleffner syndrome). (11730147)
2001
26
Pathophysiological aspects of Landau-Kleffner syndrome: from the active epileptic phase to recovery. (11130914)
2001
27
Landau-Kleffner syndrome: relation of clinical, EEG and Tc-99m-HMPAO brain SPECT findings and improvement in EEG after treatment. (10656276)
1999
28
SPECT abnormalities in Landau-Kleffner syndrome. (10833563)
1999
29
Landau-Kleffner syndrome and atypical benign partial epilepsy of childhood: the effectiveness of acetazolamide. (10480215)
1999
30
Chronic auditory agnosia following Landau-Kleffner syndrome: a 23 year outcome study. (9672766)
1998
31
Successful use of intravenous immunoglobulins in Landau-Kleffner syndrome. (9535304)
1998
32
Landau-Kleffner syndrome and sulthiame. (9208418)
1997
33
Landau-Kleffner syndrome: much more than aphasia and epilepsy. (9126414)
1997
34
Landau kleffner syndrome. (21584071)
1997
35
Discrimination of speech sounds in a boy with Landau-Kleffner syndrome: an intraoperative event-related potential study. (8892373)
1996
36
Brain single photon emission computed tomography imaging in Landau-Kleffner syndrome. (8603627)
1996
37
Linguistic development in a patient with Landau-Kleffner syndrome: a nine-year follow-up. (7791945)
1995
38
Landau-Kleffner syndrome. Treatment with subpial intracortical transection. (8595482)
1995
39
Frequency modulation analysis in children with Landau-Kleffner syndrome. (8323151)
1993
40
Vigabatrin in the Landau-Kleffner syndrome. (7684348)
1993
41
A case of Landau-Kleffner syndrome secondary to inflammatory demyelinating disease. (7684984)
1993
42
Is cerebral arteritis the cause of the Landau-Kleffner syndrome? Four cases in childhood with angiographic study. (1373338)
1992
43
Continuous focal spikes during REM sleep in a case of acquired aphasia (Landau-Kleffner syndrome). (1280854)
1992
44
Regional cerebral perfusion in Landau-Kleffner syndrome and related childhood aphasias. (1383478)
1992
45
Acquired epileptiform aphasia in children (Landau-Kleffner syndrome). (1918334)
1991
46
A case of Landau-Kleffner syndrome: speechaudiometry and clinical efficacy of treatment. (1722261)
1991
47
Acquired aphasia in childhood: the Landau-Kleffner syndrome. (1690154)
1990
48
Landau-Kleffner syndrome with unilateral EEG abnormalities--two cases from Beijing, China. (2482676)
1989
49
Low erythrocyte zinc content in acquired aphasia with convulsive disorder (Landau-Kleffner syndrome). (2442240)
1987
50
Acquired aphasia of childhood with epilepsy: the Landau-Kleffner syndrome. (2478319)
1987

Genetic Variations for Landau-Kleffner Syndrome

Expression for genes affiliated with Landau-Kleffner Syndrome

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1BioGPS, 15Gene Expression Omnibus DataSets
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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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38NCBI BioSystems Database
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Pathways related to Landau-Kleffner Syndrome according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.1BDNF, RPS6KB2

Compounds for genes affiliated with Landau-Kleffner Syndrome

Sources:
45Novoseek, 11DrugBank, 60Tocris Bioscience, 29IUPHAR, 24HMDB
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Compounds related to Landau-Kleffner Syndrome according to GeneCards/GeneDecks:

(show all 14)
idCompoundScoreTop Affiliating Genes
13-nitropropionic acid459.5NDUFS4, BDNF
21 methyl 4 phenylpyridinium459.5NDUFS4, BDNF
3glatiramer acetate45 1110.4MBP, BDNF
46-hydroxydopamine459.4NDUFS4, BDNF
5mptp459.4NDUFS4, BDNF
6tetrodotoxin45 60 2911.2MBP, BDNF
7melatonin45 60 29 11 2413.2NDUFS4, BDNF
8silver459.2MBP, BDNF
9levodopa45 1110.1BDNF, NDUFS4
10n acetylcysteine458.9BDNF, NDUFS4, MBP
11norepinephrine45 11 2410.9MBP, NDUFS4, BDNF
12corticosterone45 60 2410.8MBP, BDNF
13lactate458.8BDNF, NDUFS4, MBP
14h2o2458.7MBP, NDUFS4, BDNF

GO Terms for genes affiliated with Landau-Kleffner Syndrome

Sources:
16Gene Ontology
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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:0061209.2NDUFS4, BDNF

Products for genes affiliated with Landau-Kleffner Syndrome

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  • Lysates
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Sources for Landau-Kleffner Syndrome

3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet