DLB
MCID: LWY001
MIFTS: 61

Lewy Body Dementia (DLB) malady

Genetic diseases, Rare diseases, Neuronal diseases, Mental diseases categories

Summaries for Lewy Body Dementia

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44NIH Rare Diseases, 35MedlinePlus, 45NINDS, 66Wikipedia, 48OMIM, 34MalaCards
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MedlinePlus:35 Lewy body disease is one of the most common causes of dementia in the elderly. dementia is the loss of mental functions severe enough to affect normal activities and relationships. lewy body disease happens when abnormal structures, called lewy bodies, build up in areas of the brain. the disease may cause a wide range of symptoms, including changes in alertness and attention hallucinations problems with movement and posture muscle stiffness confusion loss of memory lewy body disease can be hard to diagnose, because parkinson's disease and alzheimer's disease cause similar symptoms. scientists think that lewy body disease might be related to these diseases, or that they sometimes happen together. lewy body disease usually begins between the ages of 50 and 85. the disease gets worse over time. there is no cure. treatment focuses on drugs to help symptoms. nih: national institute of neurological disorders and stroke

MalaCards: Lewy Body Dementia, also known as lewy body disease, is related to dementia and alzheimer's disease. An important gene associated with Lewy Body Dementia is GBA (glucosidase, beta, acid), and among its related pathways are Neurotransmitter Release Cycle and Alzheimers Disease Pathway. The compounds thioflavin and thioflavine s have been mentioned in the context of this disorder. Affiliated tissues include brain, testes and eye, and related mouse phenotypes are cardiovascular system and normal.

NIH Rare Diseases:44 Lewy bodies dementia is one of the most common types of dementia. the central feature of this condition is progressive cognitive decline, combined with three additional defining features: (1) fluctuations in alertness and attention, such as frequent drowsiness, lethargy, lengthy periods of time spent staring into space, or disorganized speech; (2) recurrent visual hallucinations, and (3) parkinsonian motor symptoms, such as rigidity and the loss of spontaneous movement. although the exact cause of this condition is unknown, symptoms result from the build-up of abnormal structures, called lewy bodies, inside the brain. this condition usually occurs sporadically, in people with no known family history of the disease. however, rare familial cases have been reported. last updated: 1/12/2012

NINDS:45 Is one of the most common types of progressive dementia. The central features of DLB include progressive cognitive decline, ?fluctuations? in alertness and attention, visual hallucinations, and parkinsonian motor symptoms, such as slowness of movement, difficulty walking, or rigidity.

Wikipedia:66 Dementia with Lewy bodies (DLB), also known under a variety of other names including Lewy body dementia,... more...

Description from OMIM:48 127750

Aliases & Classifications for Lewy Body Dementia

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9Disease Ontology, 44NIH Rare Diseases, 45NINDS, 48OMIM, 11DISEASES, 46Novoseek, 35MedlinePlus, 63UMLS, 10diseasecard, 23GTR, 59SNOMED-CT, 28ICD9CM, 41NCIt, 36MeSH
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Classifications:

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


Aliases & Descriptions:

lewy body dementia 9 44 45 48 11
lewy body disease 9 44 46 35 63
dementia, lewy body 10 23 48
autosomal dominant diffuse lewy body disease 44 63
dementia with lewy bodies 9 45
diffuse lewy body disease 9 44
senile dementia of the lewy body type 9
dlb 44


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Disease Ontology9 DOID:12217
ICD9CM28 331.82
NCIt41 C84826
OMIM48 127750
MeSH36 D020961

Related Diseases for Lewy Body Dementia

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18GeneCards, 19GeneDecks
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Diseases related to Lewy Body Dementia via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 71)
idRelated DiseaseScoreTop Affiliating Genes
1dementia31.8SNCB, SNCA, PSEN2, GBA, APP, CHAT
2alzheimer's disease31.3APP, CHAT, PSEN2, SNCA, SNCB
3parkinson's disease31.0APP, SNCB, SNCA, SLC18A2, GBA, CHAT
4multiple system atrophy30.7CHAT, TPPP, SNCA, SNCB
5frontotemporal dementia30.7APP, PSEN2, SNCA
6progressive supranuclear palsy30.7APP, CHAT, SNCA
7vascular dementia30.6SNCA, CHAT, APP
8huntington's disease30.5CHAT, SNCA, PSEN2, APP
9pick's disease30.4SNCA, APP, CHAT
10memory impairment30.4CHAT, APP, SNCA
11amyotrophic lateral sclerosis30.4SNCA, APP, CHAT
12tauopathy30.4APP, SNCA
13schizophrenia30.2SLC18A2, CHAT, SNCA
14corticobasal degeneration10.6
15autonomic dysfunction10.6
16neuroleptic malignant syndrome10.5
17capgras syndrome10.4
18cerebral atrophy10.4
19posterior cortical atrophy10.4
20neuronitis10.4
21lateral sclerosis10.3
22relapsing polychondritis10.3
23cerebral amyloid angiopathy10.3
24schizoaffective disorder10.3
25cortical blindness10.3
26striatonigral degeneration10.3
27aphasia10.3
28charles bonnet syndrome10.3
29logopenic progressive aphasia10.3
30pure autonomic failure10.3
31ataxia10.3
32dysautonomia10.3
33myoclonus10.3
34tremor10.3
35blindness10.3
36argyrophilic grain disease10.3
37parametritis10.2
38bruxism10.2
39hyperhomocysteinemia10.2
40cerebritis10.2
41rem sleep behavior disorder10.2
42cerebrovascular disease10.2
43tuberculosis10.2
44vascular disease10.2
45tardive dyskinesia10.2
46dementia - subcortical10.2
47dysphagia10.2
48parkinson disease 110.1SNCA
49parkinson disease 410.1SNCA
50neuroaxonal dystrophy10.1

Graphical network of the top 20 diseases related to Lewy Body Dementia:



Diseases related to lewy body dementia

Symptoms for Lewy Body Dementia

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48OMIM
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Symptoms by clinical synopsis from OMIM:

127750

Clinical features from OMIM:

127750

Drugs & Therapeutics for Lewy Body Dementia

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Sources:
6CenterWatch, 43NIH Clinical Center, 7ClinicalTrials, 63UMLS, 42NDF-RT
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Approved drugs:

Search CenterWatch for Lewy Body Dementia

Drug clinical trials:

Search ClinicalTrials for Lewy Body Dementia

Search NIH Clinical Center for Lewy Body Dementia

Search CenterWatch for Lewy Body Dementia

Genetic Tests for Lewy Body Dementia

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23GTR
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Genetic tests related to Lewy Body Dementia:

id Genetic test Affiliating Genes
1 Lewy Body Dementia23

Anatomical Context for Lewy Body Dementia

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34MalaCards
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MalaCards organs/tissues related to Lewy Body Dementia:

34
Brain, Testes, Eye, Heart, Skin, T cells, Temporal lobe

Animal Models for Lewy Body Dementia or affiliated genes

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38MGI
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MGI Mouse Phenotypes related to Lewy Body Dementia:

38
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053858.0SLC18A2, PSEN2, CHAT, GBA, SNCA
2MP:00028737.7APP, SNCB, EIF4G1, PSEN2, CHAT, GBA
3MP:00036317.4APP, GBA, SNCB, SLC18A2, SNCA, PSEN2
4MP:00053787.4APP, GBA, CHAT, PSEN2, SNCA, SNCB
5MP:00053867.3GBA, APP, CHAT, SNCA, SLC18A2, PSEN2
6MP:00053767.3PSEN2, GBA, APP, CHAT, SNCA, SNCB
7MP:00107687.2GBA, CHAT, APP, PSEN2, SNCA, SNCB

Publications for Lewy Body Dementia

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Sources:
53PubMed
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Articles related to Lewy Body Dementia:

(show top 50)    (show all 124)
idTitleAuthorsYear
1
Intrusion of rapid eye movement (REM) behavior disorder episodes into NREM sleep in a case of Lewy body dementia. (23063303)
2013
2
Visual hallucinations treated with the reinitiation of memantine in a patient with Lewy body dementia. (23386073)
2013
3
Prevalence of cerebrovascular lesions in patients with Lewy body dementia: a neuropathological study. (23237637)
2013
4
Dysphagia due to isoniazid therapy for tuberculosis in a patient with Lewy body dementia. (22608343)
2013
5
Neuropsychological Correlates of Behavioral Symptoms in Alzheimer's Disease, Frontal Variant of Frontotemporal, Subcortical Vascular, and Lewy Body Dementias: A Comparative Study. (24254701)
2013
6
Amyloid-specific T-cells differentiate Alzheimer's disease from Lewy body dementia. (22330173)
2012
7
Finding hope in the midst of despair: my decade with Parkinson's disease and Lewy body dementia. (22890610)
2012
8
Cognitive and structural magnetic resonance imaging features of Lewy body dementia and Alzheimer's disease. (22546353)
2012
9
Neuropathological correlates of volumetric MRI in autopsy-confirmed Lewy body dementia. (21353336)
2012
10
The high frequency of periodic limb movements in patients with Lewy body dementia. (22835911)
2012
11
Depressive symptoms in Alzheimer's disease and lewy body dementia: a one-year follow-up study. (21986003)
2011
12
Presenilin-2 gene mutation presenting as Lewy body dementia? (21409510)
2011
13
Accuracy of fluoropropyl-2beta-carbomethoxy-3beta-(4- iodophenyl) nortropane datscan for the diagnosis of lewy body dementia. (20122075)
2010
14
Lewy body dementia: the caregiver experience of clinical care. (20434939)
2010
15
Motor worsening and tardive dyskinesia with aripiprazole in Lewy body dementia. (21686897)
2009
16
BuChE-K and APOE epsilon4 allele frequencies in Lewy body dementias, and influence of genotype and hyperhomocysteinemia on cognitive decline. (19006190)
2009
17
Twin pairs discordant for neuropathologically confirmed Lewy body dementia. (19372291)
2009
18
Significance of brain lesions in Parkinson disease dementia and Lewy body dementia. (19182469)
2009
19
Defining "prodromal" Alzheimer's disease, frontotemporal dementia, and Lewy body dementia: are we there yet? (18327642)
2008
20
Amyloid load in Parkinson's disease dementia and Lewy body dementia measured with [11C]PIB positron emission tomography. (18653550)
2008
21
Memantine-induced decompensation in a patient with lewy body dementia. (17767347)
2007
22
Patients with Lewy body dementia use more resources than those with Alzheimer's disease. (17195278)
2007
23
A predominance of category deficits for living things in Alzheimer's disease and Lewy body dementia. (17445288)
2007
24
Detailed electroencephalographic long-term follow-up study in Lewy body dementia with periodic sharp wave complexes. (17345046)
2007
25
A friend of mine has been losing her memory and suffering from confusion. She's been diagnosed with a condition called Lewy body dementia. What exactly is this disorder and how does it affect people? (16832887)
2006
26
Rapid loss of striatal VMAT2 binding associated with onset of Lewy body dementia. (16258980)
2006
27
Lewy body dementia and creativity: case report. (16930639)
2006
28
The contribution of statistical parametric mapping in the assessment of precuneal and medial temporal lobe perfusion by 99mTc-HMPAO SPECT in mild Alzheimer's and Lewy body dementia. (16264357)
2005
29
Correlation of entorhinal amyloid with memory in Alzheimer's and vascular but not Lewy body dementia. (15572872)
2005
30
Imaging the dopaminergic system in suspected parkinsonism, drug induced movement disorders, and Lewy body dementia. (15657499)
2005
31
The new mutation, E46K, of alpha-synuclein causes Parkinson and Lewy body dementia. (14755719)
2004
32
Severe worsening of parkinsonism in Lewy body dementia due to donepezil. (14638981)
2003
33
Differentiating between lewy body dementia and Alzheimer's disease: a retrospective brain bank study. (12812570)
2001
34
The pathology of REM sleep behavior disorder with comorbid Lewy body dementia. (11113232)
2000
35
Clinical Lewy body dementia and the impact of vascular components. (10637403)
2000
36
Fatal attractions: abnormal protein aggregation and neuron death in Parkinson's disease and Lewy body dementia. (10203692)
1998
37
Combination of risperidone and donepezil in Lewy body dementia. (9598283)
1998
38
Distinguishing Lewy body dementia. (9484298)
1998
39
Risperidol and withdrawal bruxism in Lewy body dementia. (9489584)
1998
40
Lewy body dementia--clinical, pathological and neurochemical interconnections. (9470131)
1997
41
Neuropsychological performance in Lewy body dementia and Alzheimer's disease. (9093505)
1997
42
Comparison of proline endopeptidase activity in brain tissue from normal cases and cases with Alzheimer's disease, Lewy body dementia, Parkinson's disease and Huntington's disease. (8737597)
1996
43
Alteration in nicotine binding sites in Parkinson's disease, Lewy body dementia and Alzheimer's disease: possible index of early neuropathology. (7700528)
1995
44
Neuroleptic sensitivity to risperidone in Lewy body dementia. (7544860)
1995
45
Amyloid precursor protein accumulation in Lewy body dementia and Alzheimer's disease. (7606281)
1995
46
Monoaminergic activities in Lewy body dementia: relation to hallucinosis and extrapyramidal features. (8123190)
1993
47
Cholinergic transmitter and neurotrophic activities in Lewy body dementia: similarity to Parkinson's and distinction from Alzheimer disease. (8347330)
1993
48
Alzheimer's disease and Lewy body dementia. (8155132)
1993
49
Evidence of a monoaminergic-cholinergic imbalance related to visual hallucinations in Lewy body dementia. (1697897)
1990
50
Lewy body dementia without Alzheimer changes. (3024794)
1986

Variations for Lewy Body Dementia

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Sources:
65UniProtKB/Swiss-Prot, 1 National Center for Biotechnology Information (Clinvar)
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UniProtKB/Swiss-Prot genetic disease variations for Lewy Body Dementia:

65
id Symbol AA change Variation ID SNP ID
1SNCAp.Glu46LysVAR_022703
2SNCAp.Glu46LysVAR_022703

Clinvar genetic disease variations for Lewy Body Dementia:

1
id Gene Name Type Significance SNP ID Assembly Location
1SNCANM_000345.3(SNCA): c.136G> A (p.Glu46Lys)single nucleotide variantPathogenicrs104893875GRCh37Chr 4, 90749321: 90749321
2GBANM_001005741.2(GBA): c.1448T> C (p.Leu483Pro)single nucleotide variantPathogenic, risk factorrs421016GRCh37Chr 1, 155205043: 155205043
3GBANM_001005741.2(GBA): c.1226A> G (p.Asn409Ser)single nucleotide variantPathogenic, risk factorrs76763715GRCh37Chr 1, 155205634: 155205634
4GBANM_001005741.2(GBA): c.1448T> C (p.Leu483Pro)single nucleotide variantPathogenic, risk factorrs421016GRCh37Chr 1, 155205043: 155205043
5SNCBNM_001001502.1(SNCB): c.208G> A (p.Val70Met)single nucleotide variantPathogenicrs104893936GRCh37Chr 5, 176053473: 176053473
6SNCBNM_001001502.1(SNCB): c.368C> A (p.Pro123His)single nucleotide variantPathogenicrs104893937GRCh37Chr 5, 176048219: 176048219

Expression for genes affiliated with Lewy Body Dementia

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2BioGPS, 16Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Lewy Body Dementia

Search GEO for disease gene expression data for Lewy Body Dementia.

Pathways for genes affiliated with Lewy Body Dementia

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Sources:
51PathCards, 56Reactome, 39NCBI BioSystems Database, 54QIAGEN, 55R&D Systems, 31KEGG, 5Cell Signaling Technology
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Compounds for genes affiliated with Lewy Body Dementia

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Sources:
46Novoseek, 25HMDB, 12DrugBank, 52PharmGKB, 30IUPHAR, 62Tocris Bioscience
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Compounds related to Lewy Body Dementia according to GeneCards/GeneDecks:

(show top 50)    (show all 54)
idCompoundScoreTop Affiliating Genes
1thioflavin4610.2APP, SNCA
2thioflavine s4610.1SNCA, APP
3thioflavin t4610.1APP, SNCA
4methionine sulfoxide46 25 1212.1APP, SNCA
5huperzine a4610.0APP, CHAT
6propentofylline4610.0APP, CHAT
7physostigmine46 1211.0CHAT, APP
8donepezil46 52 25 1213.0CHAT, APP
91 methyl 4 phenylpyridinium469.9SLC18A2, SNCA
10apomorphine30 46 1211.9SNCA, CHAT
11formate469.9SNCA, APP
12benzodiazepine469.8CHAT, SLC18A2
13l-amino acid469.8CHAT, SLC18A2
146-hydroxydopamine469.7SNCA, CHAT, APP
15ab-42469.7APP, PSEN2
164-hydroxynonenal46 2510.7CHAT, APP, SNCA
17risperidone46 52 30 62 25 1214.6APP, CHAT
18mptp469.6SNCA, SLC18A2
19methamphetamine46 52 1211.6SLC18A2, SNCA, CHAT
20catecholamine469.6SLC18A2, SNCA, CHAT
21cocaine46 1210.5CHAT, SLC18A2, SNCA
22phospholipid469.5CHAT, SNCA, GBA
235-hydroxytryptamine469.5APP, SLC18A2, CHAT
24nicotine46 30 52 1212.4SLC18A2, CHAT, APP
25gaba469.4SLC18A2, CHAT, APP
26ethanol46 52 25 1212.4CHAT, SNCA, SLC18A2
27nmda46 3010.3CHAT, APP, SLC18A2
28amphetamine46 52 1211.3SNCA, SLC18A2
29lactacystin469.3PSEN2, APP, SNCA
30iron46 2510.3APP, SLC18A2, SNCA, SNCB
31valine469.2SNCA, PSEN2, APP
32norepinephrine46 25 1211.2CHAT, APP, SLC18A2, SNCA
33adenylate469.2GBA, EIF4G1, SNCA, APP
34acetylcholine46 52 30 25 1213.2SNCA, CHAT, APP, SLC18A2
35dopamine46 30 25 1212.1APP, SLC18A2, CHAT, SNCA
36lipid469.0SNCB, CHAT, SNCA, GBA, APP
37h2o2468.9PSEN2, SNCB, APP, SNCA
38cholesterol46 30 25 1211.9SNCA, GBA, APP, PSEN2
39alanine468.9APP, EIF4G1, CHAT, SNCA
40choline46 25 1210.9PSEN2, CHAT, APP, SNCA
41paraffin468.8SNCA, CHAT, PSEN2
42cysteine468.7SNCA, EIF4G1, SLC18A2, APP, GBA
43aspartate468.6CHAT, APP, SLC18A2, PSEN2
44oxygen46 259.6PSEN2, SNCA, SLC18A2, GBA
45nitric oxide46 25 1210.5GBA, SLC18A2, SNCA, PSEN2, CHAT
46serine468.4GBA, APP, PSEN2, SLC18A2, SNCA
47retinoic acid46 259.3CHAT, APP, PSEN2, SLC18A2
48glucose467.9SLC18A2, EIF4G1, SNCA, PSEN2, GBA, CHAT
49glutamate467.7EIF4G1, SLC18A2, SNCA, PSEN2, CHAT, APP
50calcium46 52 25 1210.5PSEN2, SLC18A2, SNCA, SNCB, EIF4G1, CHAT

GO Terms for genes affiliated with Lewy Body Dementia

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17Gene Ontology
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Cellular components related to Lewy Body Dementia according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1terminal boutonGO:0431959.5SNCB, SLC18A2, SNCA
2ciliary rootletGO:0352539.3PSEN2, APP
3neuromuscular junctionGO:0315949.2APP, PSEN2
4growth coneGO:0304269.1PSEN2, SNCA, SNCB
5dendritic shaftGO:0431988.9PSEN2, APP
6axonGO:0304248.6PSEN2, APP, SNCA, CHAT
7neuronal cell bodyGO:0430258.5SLC18A2, SNCB, PSEN2, CHAT
8Golgi apparatusGO:0057948.5APP, PSEN2, SNCA, SNCB
9perinuclear region of cytoplasmGO:0484718.4SNCA, PSEN2, TPPP, APP

Biological processes related to Lewy Body Dementia according to GeneCards/GeneDecks:

(show all 8)
idNameGO IDScoreTop Affiliating Genes
1synapse organizationGO:05080810.0SNCA, SNCB
2dendrite developmentGO:0163589.7APP, CHAT
3adult locomotory behaviorGO:0083449.7SNCA, APP
4regulation of epidermal growth factor-activated receptor activityGO:0071769.4PSEN2, APP
5neurotransmitter secretionGO:0072699.4SLC18A2, CHAT
6negative regulation of protein phosphorylationGO:0019339.4SNCA, PSEN2
7synaptic transmissionGO:0072689.2SNCB, CHAT, SLC18A2
8forebrain developmentGO:0309008.9PSEN2, APP

Molecular functions related to Lewy Body Dementia according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1beta-tubulin bindingGO:0484879.8SNCB, SNCA
2alpha-tubulin bindingGO:0430149.5SNCB, SNCA

Products for genes affiliated with Lewy Body Dementia

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  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Lewy Body Dementia

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4CDC
14ExPASy
15FMA
23GTR
24HGMD
25HMDB
26ICD10
27ICD10 via Orphanet
28ICD9CM
30IUPHAR
31KEGG
36MeSH
37MESH via Orphanet
38MGI
41NCIt
42NDF-RT
45NINDS
46Novoseek
48OMIM
49OMIM via Orphanet
53PubMed
54QIAGEN
60SNOMED-CT via Orphanet
63UMLS
64UMLS via Orphanet