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
Epidemiological study of Landau-Kleffner syndrome (LKS) in Japan. (23706409)
2013
2
BECTS evolving to Landau-Kleffner Syndrome and back by subsequent recovery: a longitudinal language reorganization case study using fMRI, source EEG, and neuropsychological testing. (23399945)
2013
3
Serial auditory-evoked potentials in the diagnosis and monitoring of a child with Landau-Kleffner syndrome. (24047944)
2013
4
Changing perspectives on Landau-Kleffner syndrome. (21955111)
2011
5
A child with refractory complex partial seizures, right temporal ganglioglioma, contralateral continuous electrical status epilepticus, and a secondary Landau-Kleffner autistic syndrome. (18602026)
2009
6
Sign language in Landau-Kleffner syndrome. (19682058)
2009
7
Rational treatment options with AEDs and ketogenic diet in Landau-Kleffner syndrome: still waiting after all these years. (19682054)
2009
8
Some facts about sleep relevant for Landau-Kleffner syndrome. (19682050)
2009
9
Specific language impairment versus Landau-Kleffner syndrome. (19682045)
2009
10
The surgical treatment of Landau-Kleffner syndrome. (19682055)
2009
11
Age of onset and outcome in Landau-Kleffner syndrome (1985). (18834381)
2008
12
An in computo investigation of the Landau-Kleffner syndrome. (18002559)
2007
13
Brain-derived neurotrophic factor and autoantibodies to neural antigens in sera of children with autistic spectrum disorders, Landau-Kleffner syndrome, and epilepsy. (16181614)
2006
14
Episodic receptive aphasia in a child with Landau-Kleffner Syndrome: PET correlates. (16632288)
2006
15
Landau-Kleffner syndrome is not an eponymic badge of ignorance. (16806832)
2006
16
Management of Landau-Kleffner syndrome. (16356025)
2005
17
Landau-Kleffner syndrome. (23377356)
2004
18
Landau-Kleffner syndrome and temporal cortical volume reduction: cause or effect? (15477529)
2004
19
Efficacy of intravenous immunoglobulin in Landau-Kleffner syndrome. (11992758)
2002
20
Landau-Kleffner syndrome (acquired epileptic aphasia) in a Nigerian child. (11932757)
2002
21
Landau-Kleffner syndrome: study of four cases. (12068352)
2002
22
The Landau-Kleffner Syndrome. (15309183)
2001
23
Landau-Kleffner syndrome: course and correlates with outcome. (11305401)
2001
24
Multiple subpial transection in Landau-Kleffner syndrome. (11305402)
2001
25
Effects of high-dose intravenous corticosteroid therapy in Landau-Kleffner syndrome. (10738922)
2000
26
Spindles-inducing mechanism modulates sleep activation of interictal epileptiform discharges in the Landau-Kleffner syndrome. (10691117)
2000
27
Landau-Kleffner syndrome: sleep EEG characteristics at onset. (10996560)
2000
28
Language outcome following multiple subpial transection for Landau-Kleffner syndrome. (10094262)
1999
29
Magnetoencephalography in presurgical evaluation of children with the Landau-Kleffner syndrome. (10080514)
1999
30
Spike-induced interference in auditory sensory processing in Landau-Kleffner syndrome. (9780021)
1998
31
Neuropsychological characteristics of five children with the Landau-Kleffner syndrome: dissociation of auditory and phonological discrimination. (10050361)
1998
32
Landau-Kleffner syndrome: metabolic abnormalities in temporal lobe are a common feature. (9430313)
1997
33
Correlation between CSWS and aphasia in Landau-Kleffner syndrome: a study of three cases. (8836500)
1996
34
Acquired epileptiform opercular syndrome: a second case report, review of the literature, and comparison to the Landau-Kleffner syndrome. (7555956)
1995
35
The Landau-Kleffner Syndrome: a review. (8608387)
1995
36
Landau-Kleffner syndrome: acquired epileptic aphasia in children. (7514057)
1994
37
Landau-Kleffner syndrome in cerebral cysticercosis. (7533144)
1994
38
Developmental dysphasias, epilepsy, and Landau-Kleffner syndrome. (7504408)
1993
39
Intracranial EEG monitoring in Landau-Kleffner syndrome associated with left temporal lobe astrocytoma. (7684985)
1993
40
Landau-Kleffner syndrome: six patients including discordant monozygotic twins. (7680862)
1993
41
Landau-Kleffner syndrome: temporal lobe tumor resection results in good outcome. (7692865)
1993
42
The Landau-Kleffner syndrome or 'acquired aphasia with convulsive disorder'. Long-term follow-up of six children and a review of the recent literature. (1558514)
1992
43
Landau-Kleffner syndrome: epileptic activity in the auditory cortex. (1716495)
1991
44
Effect of early corticosteroid therapy for Landau-Kleffner syndrome. (1709123)
1991
45
A case of pure word deafness associated with Landau-Kleffner syndrome: a long-term study of auditory disturbance. (1799334)
1991
46
Landau-Kleffner syndrome: a clinical and EEG study of five cases. (1700952)
1990
47
Acquired childhood aphasia with convulsive disorder (Landau-Kleffner syndrome). A case report. (1689510)
1990
48
Landau-Kleffner syndrome: a pharmacologic study of five cases. (1700953)
1990
49
Acquired epileptic aphasia (the Landau-Kleffner syndrome) due to neurocysticercosis. (2477238)
1989
50
Landau-Kleffner syndrome--epileptic aphasia in children--possible role of toxoplasma gondii infection. (2479399)
1988-1989

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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  • Antibodies
  • Proteins
  • Lysates
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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