MCID: LND001
MIFTS: 31

Landau-Kleffner Syndrome malady

Rare diseases, Neuronal diseases categories

Aliases & Classifications for Landau-Kleffner Syndrome

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

Aliases & Descriptions for Landau-Kleffner Syndrome:

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


Classifications:



Characteristics (Orphanet epidemiological data):

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


External Ids:

Disease Ontology10 DOID:2538
NCIt42 C84806
MeSH36 D018887
Orphanet51 98818
UMLS via Orphanet66 C0282512
MESH via Orphanet37 D018887

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 epileptiform aphasia, is related to aphasia and cerebritis. An important gene associated with Landau-Kleffner Syndrome is GRIN2A (Glutamate Receptor, Ionotropic, N-Methyl D-Aspartate 2A). Affiliated tissues include brain, temporal lobe and testes.

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.

Related Diseases for Landau-Kleffner Syndrome

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Diseases related to Landau-Kleffner Syndrome via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 44)
idRelated DiseaseScoreTop Affiliating Genes
1aphasia11.1
2cerebritis10.7
3epilepsy, focal, with speech disorder and with or without mental retardation10.6
4status epilepticus10.6
5auditory agnosia10.6
6agnosia10.6
7encephalopathy10.5
8epilepsy, nocturnal frontal lobe, 310.3
9celiac disease10.3
10centrotemporal epilepsy10.3
11child syndrome10.3
12speech and communication disorders10.3
13autistic disorder10.3
14temporal lobe epilepsy10.3
15cerebral arteritis10.3
16cysticercosis10.3
17frontal lobe epilepsy10.3
18benign epilepsy with centrotemporal spikes10.3
19verbal auditory agnosia10.3
20stuttering10.3
21specific language impairment10.3
22astrocytoma10.3
23demyelinating disease10.3
24encephalitis10.3
25epilepsy syndrome10.3
26focal epilepsy10.3
27pervasive developmental disorder10.3
28prion disease10.3
29sleep disorder10.3
30polymicrogyria10.3
31developmental dysphasia familial10.3
32kid syndrome10.3
33seizure disorder10.3
34continuous spikes and waves during sleep10.3
35vohwinkel syndrome with ichthyosis10.1
36dwarfism10.1
37kidney pelvis papillary carcinoma10.0ELP4, GRIN2A
38follicular adenoma9.9GRIN2A, TBC1D24
39alcohol dependence9.9GRIN2A, POMC
40infective endocarditis9.9POMC, TBC1D24
41spondylocostal dysostosis9.8POMC, TBC1D24
42kashin-beck disease9.7POMC, TBC1D24
43childhood infratentorial neoplasm9.6ELP4, POMC, TBC1D24
44nutritional optic neuropathy9.1ELP4, GRIN2A, ITSN2, NDUFS4, POMC, TBC1D24

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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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

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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, 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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Articles related to Landau-Kleffner Syndrome:

(show top 50)    (show all 170)
idTitleAuthorsYear
1
Landau-Kleffner syndrome: an uncommon dealt with case in Southeast Asia. (26420702)
2015
2
Evidence for normal letter-sound integration, but altered language pathways in a case of recovered Landau-Kleffner Syndrome. (26232266)
2015
3
Temporoparietal resection in a patient with Landau-Kleffner syndrome. (25149936)
2014
4
Epidemiological study of Landau-Kleffner syndrome (LKS) in Japan. (23706409)
2013
5
Epileptic encephalopathy with continuous spike-waves during slow-wave sleep including Landau-Kleffner syndrome. (23622211)
2013
6
Landau-Kleffner syndrome: a rare auditory processing disorder series of cases and review of the literature. (21074868)
2011
7
Landau-Kleffner syndrome: long-term follow-up. (18930363)
2009
8
Acquired epileptic aphasia: Landau-Kleffner syndrome. (21887181)
2009
9
Landau-Kleffner syndrome: 50 years after. (19682039)
2009
10
Sign language in Landau-Kleffner syndrome. (19682058)
2009
11
When epilepsy interferes with word comprehension: findings in Landau-Kleffner syndrome. (18184944)
2008
12
Possible involvement of the tip of temporal lobe in Landau-Kleffner syndrome. (17383838)
2007
13
Neuropsychological findings in Rolandic epilepsy and Landau-Kleffner syndrome. (17105467)
2006
14
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
15
Landau-Kleffner syndrome, electrical status epilepticus in slow wave sleep, and language regression in children. (15362173)
2004
16
Electroencephalography findings in autism: similarities and differences from Landau-Kleffner syndrome. (15575417)
2004
17
Landau-Kleffner syndrome. (23377356)
2004
18
Landau-Kleffner syndrome responsive to levetiracetam. (14527502)
2003
19
The Landau-Kleffner syndrome. (11993733)
2002
20
Efficacy of intravenous immunoglobulin in Landau-Kleffner syndrome. (11992758)
2002
21
Landau-Kleffner Syndrome. (15309185)
2001
22
The Landau-Kleffner Syndrome. (15309183)
2001
23
Landau-Kleffner Syndrome: Localization of Epileptogenic Lesion Using Wavelet- Cross-Correlation Analysis. (12609372)
2001
24
Sign language in childhood epileptic aphasia (Landau-Kleffner syndrome). (11730147)
2001
25
Theory and testing in Landau-Kleffner Syndrome. (11844720)
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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Clinvar genetic disease variations for Landau-Kleffner Syndrome:

5 (show all 13)
id Gene Variation Type Significance SNP ID Assembly Location
1GRIN2ANM_000833.4(GRIN2A): c.314A> G (p.Asp105Gly)single nucleotide variantLikely pathogenicrs797045015GRCh38Chr 16, 10180098: 10180098
2GRIN2ANM_000833.4(GRIN2A): c.652C> T (p.Gln218Ter)single nucleotide variantPathogenicrs387906637GRCh37Chr 16, 10032171: 10032171
3GRIN2ANM_000833.4(GRIN2A): c.1845C> A (p.Asn615Lys)single nucleotide variantPathogenicrs397518447GRCh37Chr 16, 9923442: 9923442
4GRIN2ANM_000833.4(GRIN2A): c.1945C> G (p.Leu649Val)single nucleotide variantPathogenicrs397514557GRCh37Chr 16, 9923342: 9923342
5GRIN2ANM_000833.4(GRIN2A): c.1655C> G (p.Pro552Arg)single nucleotide variantPathogenicrs397518450GRCh37Chr 16, 9928084: 9928084
6GRIN2ANM_000833.4(GRIN2A): c.1007+1G> Asingle nucleotide variantPathogenicrs397518465GRCh37Chr 16, 10031815: 10031815
7GRIN2ANM_000833.4(GRIN2A): c.2T> C (p.Met1Thr)single nucleotide variantPathogenicrs397518466GRCh37Chr 16, 10274267: 10274267
8GRIN2ANM_000833.4(GRIN2A): c.2829C> G (p.Tyr943Ter)single nucleotide variantPathogenicrs397518467GRCh37Chr 16, 9858572: 9858572
9GRIN2ANM_000833.4(GRIN2A): c.1592C> T (p.Thr531Met)single nucleotide variantPathogenicrs397518468GRCh37Chr 16, 9934563: 9934563
10GRIN2ANM_000833.4(GRIN2A): c.1123-2A> Gsingle nucleotide variantPathogenicrs397518469GRCh37Chr 16, 9943820: 9943820
11GRIN2ANM_000833.4(GRIN2A): c.1553G> A (p.Arg518His)single nucleotide variantPathogenicrs397518470GRCh37Chr 16, 9934602: 9934602
12GRIN2ANM_000833.4(GRIN2A): c.1954T> G (p.Phe652Val)single nucleotide variantPathogenicrs397518471GRCh37Chr 16, 9923333: 9923333
13GRIN2ANM_000833.4(GRIN2A): c.2041C> T (p.Arg681Ter)single nucleotide variantPathogenicrs397518472GRCh37Chr 16, 9916248: 9916248

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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Cellular components related to Landau-Kleffner Syndrome according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1terminal boutonGO:00431959.5GRIN2A, TBC1D24

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