LKS
MCID: LND001
MIFTS: 39

Landau-Kleffner Syndrome (LKS) malady

Neuronal diseases category

Summaries for Landau-Kleffner Syndrome

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42NIH Rare Diseases, 43NINDS, 63Wikipedia, 46OMIM, 32MalaCards
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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: Landau-Kleffner Syndrome, also known as acquired epileptiform aphasia, is related to epilepsy syndrome and autistic disorder. 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, temporal lobe and testes.

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

Description from OMIM:46 245570

Aliases & Classifications for Landau-Kleffner Syndrome

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8Disease Ontology, 42NIH Rare Diseases, 43NINDS, 10DISEASES, 60UMLS, 56SNOMED-CT, 39NCIt, 46OMIM, 34MeSH, 25ICD10
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Classifications:

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


Aliases & Descriptions:

landau-kleffner syndrome 8 42 43 10 60
acquired epileptiform aphasia 42 43
acquired aphasia with convulsive disorder 42
acquired epileptic aphasia 8
lks 42


External Ids:

Disease Ontology8 DOID:2538
NCIt39 C84806
OMIM46 245570
MeSH34 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

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46OMIM
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Clinical features from OMIM:

245570

Drugs & Therapeutics for Landau-Kleffner Syndrome

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5CenterWatch, 41NIH 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

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Anatomical Context for Landau-Kleffner Syndrome

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32MalaCards
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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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Publications for Landau-Kleffner Syndrome

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50PubMed
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Articles related to Landau-Kleffner Syndrome:

(show top 50)    (show all 161)
idTitleAuthorsYear
1
Epileptic encephalopathy with continuous spike-waves during slow-wave sleep including Landau-Kleffner syndrome. (23622211)
2013
2
Acquired auditory agnosia in childhood and normal sleep electroencephalography subsequently diagnosed as Landau-Kleffner syndrome: a report of three cases. (23121226)
2013
3
Sparse MEG source imaging in Landau-Kleffner syndrome. (22255439)
2011
4
Early-onset acquired epileptic aphasia (Landau-Kleffner syndrome, LKS) and regressive autistic disorders with epileptic EEG abnormalities: the continuing debate. (20637551)
2010
5
General anesthesia for a child with Landau-Kleffner syndrome, a case report. (20843226)
2010
6
Magnetoencephalography in Landau-Kleffner syndrome. (19682052)
2009
7
Landau-Kleffner syndrome and CSWS syndrome: treatment with intravenous immunoglobulins. (19682053)
2009
8
Linking autism, regression and Landau-Kleffner syndrome: integrative role of nerve growth factor. (17157996)
2007
9
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
10
Corticosteroids for the treatment of Landau-kleffner syndrome and continuous spike-wave discharge during sleep. (15866429)
2005
11
Landau-Kleffner syndrome, electrical status epilepticus in slow wave sleep, and language regression in children. (15362173)
2004
12
Electroencephalography findings in autism: similarities and differences from Landau-Kleffner syndrome. (15575417)
2004
13
The effects of vagus nerve stimulation therapy on patients with intractable seizures and either Landau-Kleffner syndrome or autism. (12791330)
2003
14
Landau-Kleffner syndrome. (12873863)
2003
15
Sign language in childhood epileptic aphasia (Landau-Kleffner syndrome). (11730147)
2001
16
Accessibility of spoken, written, and sign language in Landau-Kleffner syndrome: a linguistic and functional MRI study. (11431170)
2001
17
Importance of early drug treatment in prognosis of the Landau-Kleffner syndrome. (11811283)
2001
18
Pathophysiological aspects of Landau-Kleffner syndrome: from the active epileptic phase to recovery. (11130914)
2001
19
Landau Kleffner syndrome. (11450391)
2001
20
Autistic regression and Landau-Kleffner syndrome: progress or confusion? (10855658)
2000
21
Successful use of intravenous immunoglobulin as initial monotherapy in Landau-Kleffner syndrome. (10897161)
2000
22
Landau-Kleffner syndrome with onset at 18 months and an initial diagnosis of pervasive developmental disorder. (10817490)
2000
23
Language disorders in Landau-Kleffner syndrome. (10073434)
1999
24
Prosodic preservation in Landau-Kleffner syndrome: a case report. (10053236)
1999
25
Landau-Kleffner syndrome (LKS): long-term follow-up and links with electrical status epilepticus during sleep (ESES). (10206525)
1999
26
Landau Kleffner syndrome. (10216604)
1998
27
IVIG in Landau-Kleffner syndrome. (9880151)
1998
28
Landau-Kleffner syndrome: consistent response to repeated intravenous gamma-globulin doses: a case report. (9118856)
1997
29
Discrimination of speech sounds in a boy with Landau-Kleffner syndrome: an intraoperative event-related potential study. (8892373)
1996
30
Brain single photon emission computed tomography imaging in Landau-Kleffner syndrome. (8603627)
1996
31
Linguistic development in a patient with Landau-Kleffner syndrome: a nine-year follow-up. (7791945)
1995
32
Landau-Kleffner syndrome. Treatment with subpial intracortical transection. (8595482)
1995
33
Landau-Kleffner syndrome with continuous spikes and waves during slow-wave sleep. (7782603)
1995
34
Sounds triggers spikes in the Landau-Kleffner syndrome. (7519634)
1994
35
Acquired aphasia without deafness in childhood--the Landau-Kleffner syndrome. (15125283)
1993
36
What differentiates Landau-Kleffner syndrome from the syndrome of continuous spikes and waves during slow sleep? (7692838)
1993
37
Landau-Kleffner syndrome: some suggestions. (8363441)
1993
38
Landau-Kleffner syndrome. An eponymic badge of ignorance. (1558513)
1992
39
A role of autoimmunity in the etiopathogenesis of Landau-Kleffner syndrome? (1280911)
1992
40
Diagnostic and management considerations of acquired epileptic aphasia or Landau-Kleffner syndrome. (1715675)
1991
41
A case of Landau-Kleffner syndrome: speechaudiometry and clinical efficacy of treatment. (1722261)
1991
42
Cerebral glucose utilization during sleep in Landau-Kleffner syndrome: a PET study. (1700954)
1990
43
Landau-Kleffner syndrome. EEG topographic studies. (2466418)
1989
44
Landau-Kleffner syndrome: a case study with a fourteen-year follow-up. (2468500)
1989
45
Landau-Kleffner syndrome with unilateral EEG abnormalities--two cases from Beijing, China. (2482676)
1989
46
The Landau-Kleffner syndrome--case report and theoretical considerations. (2779747)
1989
47
Ear disease in Landau-Kleffner syndrome (LKS) and in adult psychiatry. (2466546)
1989
48
Acquired aphasia with epilepsy--Landau-Kleffner syndrome. (2453356)
1988
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

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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

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Pathways for genes affiliated with Landau-Kleffner Syndrome

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37NCBI 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

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44Novoseek, 11DrugBank, 59Tocris Bioscience, 28IUPHAR, 24HMDB
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Compounds related to Landau-Kleffner Syndrome according to GeneCards/GeneDecks:

(show all 14)
idCompoundScoreTop Affiliating Genes
13-nitropropionic acid449.5NDUFS4, BDNF
21 methyl 4 phenylpyridinium449.5NDUFS4, BDNF
3glatiramer acetate44 1110.4MBP, BDNF
46-hydroxydopamine449.4NDUFS4, BDNF
5mptp449.4NDUFS4, BDNF
6tetrodotoxin44 59 2811.2MBP, BDNF
7melatonin44 59 28 11 2413.2NDUFS4, BDNF
8silver449.2MBP, BDNF
9levodopa44 1110.1BDNF, NDUFS4
10n acetylcysteine448.9BDNF, NDUFS4, MBP
11norepinephrine44 11 2410.9MBP, NDUFS4, BDNF
12corticosterone44 59 2410.8MBP, BDNF
13lactate448.8BDNF, NDUFS4, MBP
14h2o2448.7MBP, NDUFS4, BDNF

GO Terms for genes affiliated with Landau-Kleffner Syndrome

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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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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
50PubMed
51QIAGEN
57SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet