DLB
MCID: LWY001
MIFTS: 63

Lewy Body Dementia (DLB) malady

Genetic diseases, Rare diseases, Mental diseases, Neuronal diseases categories
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Summaries for Lewy Body Dementia

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MedlinePlus:33 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 based summary: Lewy Body Dementia, also known as lewy body disease, is related to dementia and alzheimer's disease, and has symptoms including 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.

Disease Ontology:8 A dementia and a synucleinopathy that is characterized by the development of abnormal proteinaceous (alpha-synuclein) cytoplasmic inclusions, called lewy bodies, throughout the brain that results in progressive decline in mental abilities.

NIH Rare Diseases:42 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:43 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:65 Dementia with Lewy bodies (DLB), also known under a variety of other names including Lewy body dementia... more...

Description from OMIM:46 127750

Aliases & Classifications for Lewy Body Dementia

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Lewy Body Dementia, Aliases & Descriptions:

Name: Lewy Body Dementia 8 42 43 46 10
Lewy Body Disease 8 42 44 33 62
Dementia, Lewy Body 9 22 46 62
Diffuse Lewy Body Disease 8 42 62
 
Dementia with Lewy Bodies 8 43 62
Autosomal Dominant Diffuse Lewy Body Disease 42 62
Senile Dementia of the Lewy Body Type 8 62
Dlb 42


Classifications:

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


External Ids:

Disease Ontology8 DOID:12217
OMIM46 127750
NCIt39 C84826
ICD9CM27 331.82
MeSH34 D020961

Related Diseases for Lewy Body Dementia

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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 75)
idRelated DiseaseScoreTop Affiliating Genes
1dementia31.3GBA, APP, CHAT, PSEN2, SNCA, SNCB
2alzheimer's disease31.0APP, CHAT, PSEN2, SNCA, SNCB
3vascular dementia30.8APP, CHAT, SNCA
4frontotemporal dementia30.7APP, PSEN2, SNCA
5multiple system atrophy30.7TPPP, CHAT, SNCA, SNCB
6tauopathy30.7APP, SNCA
7memory impairment30.6SNCA, CHAT, APP
8progressive supranuclear palsy30.6APP, CHAT, SNCA
9pick's disease30.5SNCA, CHAT, APP
10parkinson's disease30.5SLC18A2, SNCB, SNCA, CHAT, APP, GBA
11creutzfeldt-jakob disease30.4SNCA, APP
12huntington's disease30.4APP, CHAT, PSEN2, SNCA
13schizophrenia29.8SLC18A2, SNCA, CHAT
14neuroleptic malignant syndrome10.5
15posterior cortical atrophy10.5
16parkinson disease 110.5SNCA
17parkinson disease 410.4SNCA
18cerebral atrophy10.4
19neuronitis10.4
20dysphagia10.4
21relapsing polychondritis10.3
22cerebral amyloid angiopathy10.3
23schizoaffective disorder10.3
24cortical blindness10.3
25striatonigral degeneration10.3
26aphasia10.3
27charles bonnet syndrome10.3
28logopenic progressive aphasia10.3
29ataxia10.3
30dysautonomia10.3
31myoclonus10.3
32tremor10.3
33blindness10.3
34niemann-pick disease type c110.3APP, SNCA
35synucleinopathy10.2
36bruxism10.2
37parametritis10.2
38capgras syndrome10.2
39hyperhomocysteinemia10.2
40rem sleep behavior disorder10.2
41cerebritis10.2
42cerebrovascular disease10.2
43tuberculosis10.2
44vascular disease10.2
45tardive dyskinesia10.2
46dementia - subcortical10.2
47amyotrophic lateral sclerosis10.2
48neuroaxonal dystrophy10.2
49lateral sclerosis10.2
50corticobasal degeneration10.2

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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Symptoms by clinical synopsis from OMIM:

127750

Clinical features from OMIM:

127750

HPO human phenotypes related to Lewy Body Dementia:

(show all 6)
id Description Frequency HPO Source Accession
1 autosomal dominant inheritance HP:0000006
2 dementia HP:0000726
3 delusions HP:0000746
4 parkinsonism HP:0001300
5 visual hallucinations HP:0002367
6 fluctuations in consciousness HP:0007159

Drugs & Therapeutics for Lewy Body Dementia

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Drug clinical trials:

Search ClinicalTrials for Lewy Body Dementia

Search NIH Clinical Center for Lewy Body Dementia

Genetic Tests for Lewy Body Dementia

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Genetic tests related to Lewy Body Dementia:

id Genetic test Affiliating Genes
1 Lewy Body Dementia22

Anatomical Context for Lewy Body Dementia

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MalaCards organs/tissues related to Lewy Body Dementia:

32
Brain, Testes, Eye, Heart, Skin, T cells, Temporal lobe

Animal Models for Lewy Body Dementia or affiliated genes

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MGI Mouse Phenotypes related to Lewy Body Dementia:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053858.0SLC18A2, SNCA, PSEN2, CHAT, GBA
2MP:00028737.7EIF4G1, SNCB, PSEN2, CHAT, APP, GBA
3MP:00036317.4GBA, APP, CHAT, PSEN2, SNCA, SNCB
4MP:00053787.4GBA, APP, CHAT, PSEN2, SNCA, SNCB
5MP:00053867.3GBA, APP, CHAT, PSEN2, SNCA, SLC18A2
6MP:00053767.3GBA, APP, CHAT, PSEN2, SNCA, SNCB
7MP:00107687.2GBA, APP, CHAT, PSEN2, SNCA, SNCB

Publications for Lewy Body Dementia

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Articles related to Lewy Body Dementia:

(show top 50)    (show all 132)
idTitleAuthorsYear
1
Lewy body dementia: the impact on patients and caregivers. (25031635)
2014
2
Intrusion of rapid eye movement (REM) behavior disorder episodes into NREM sleep in a case of Lewy body dementia. (23063303)
2013
3
Visual hallucinations treated with the reinitiation of memantine in a patient with Lewy body dementia. (23386073)
2013
4
Prevalence of cerebrovascular lesions in patients with Lewy body dementia: a neuropathological study. (23237637)
2013
5
Dysphagia due to isoniazid therapy for tuberculosis in a patient with Lewy body dementia. (22608343)
2013
6
Neuropsychological Correlates of Behavioral Symptoms in Alzheimer's Disease, Frontal Variant of Frontotemporal, Subcortical Vascular, and Lewy Body Dementias: A Comparative Study. (24254701)
2013
7
Amyloid-specific T-cells differentiate Alzheimer's disease from Lewy body dementia. (22330173)
2012
8
Finding hope in the midst of despair: my decade with Parkinson's disease and Lewy body dementia. (22890610)
2012
9
Cognitive and structural magnetic resonance imaging features of Lewy body dementia and Alzheimer's disease. (22546353)
2012
10
Neuropathological correlates of volumetric MRI in autopsy-confirmed Lewy body dementia. (21353336)
2012
11
The high frequency of periodic limb movements in patients with Lewy body dementia. (22835911)
2012
12
Depressive symptoms in Alzheimer's disease and lewy body dementia: a one-year follow-up study. (21986003)
2011
13
Presenilin-2 gene mutation presenting as Lewy body dementia? (21409510)
2011
14
Accuracy of fluoropropyl-2beta-carbomethoxy-3beta-(4- iodophenyl) nortropane datscan for the diagnosis of lewy body dementia. (20122075)
2010
15
Lewy body dementia: the caregiver experience of clinical care. (20434939)
2010
16
Motor worsening and tardive dyskinesia with aripiprazole in Lewy body dementia. (21686897)
2009
17
BuChE-K and APOE epsilon4 allele frequencies in Lewy body dementias, and influence of genotype and hyperhomocysteinemia on cognitive decline. (19006190)
2009
18
Twin pairs discordant for neuropathologically confirmed Lewy body dementia. (19372291)
2009
19
Significance of brain lesions in Parkinson disease dementia and Lewy body dementia. (19182469)
2009
20
Defining "prodromal" Alzheimer's disease, frontotemporal dementia, and Lewy body dementia: are we there yet? (18327642)
2008
21
Amyloid load in Parkinson's disease dementia and Lewy body dementia measured with [11C]PIB positron emission tomography. (18653550)
2008
22
Memantine-induced decompensation in a patient with lewy body dementia. (17767347)
2007
23
Patients with Lewy body dementia use more resources than those with Alzheimer's disease. (17195278)
2007
24
A predominance of category deficits for living things in Alzheimer's disease and Lewy body dementia. (17445288)
2007
25
Detailed electroencephalographic long-term follow-up study in Lewy body dementia with periodic sharp wave complexes. (17345046)
2007
26
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
27
Rapid loss of striatal VMAT2 binding associated with onset of Lewy body dementia. (16258980)
2006
28
Lewy body dementia and creativity: case report. (16930639)
2006
29
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
30
Correlation of entorhinal amyloid with memory in Alzheimer's and vascular but not Lewy body dementia. (15572872)
2005
31
Severe worsening of parkinsonism in Lewy body dementia due to donepezil. (14638981)
2003
32
Differentiating between lewy body dementia and Alzheimer's disease: a retrospective brain bank study. (12812570)
2001
33
The pathology of REM sleep behavior disorder with comorbid Lewy body dementia. (11113232)
2000
34
Clinical Lewy body dementia and the impact of vascular components. (10637403)
2000
35
Fatal attractions: abnormal protein aggregation and neuron death in Parkinson's disease and Lewy body dementia. (10203692)
1998
36
Combination of risperidone and donepezil in Lewy body dementia. (9598283)
1998
37
Distinguishing Lewy body dementia. (9484298)
1998
38
Risperidol and withdrawal bruxism in Lewy body dementia. (9489584)
1998
39
Lewy body dementia--clinical, pathological and neurochemical interconnections. (9470131)
1997
40
Neuropsychological performance in Lewy body dementia and Alzheimer's disease. (9093505)
1997
41
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
42
Alteration in nicotine binding sites in Parkinson's disease, Lewy body dementia and Alzheimer's disease: possible index of early neuropathology. (7700528)
1995
43
Neuroleptic sensitivity to risperidone in Lewy body dementia. (7544860)
1995
44
Amyloid precursor protein accumulation in Lewy body dementia and Alzheimer's disease. (7606281)
1995
45
Alzheimer's disease and Lewy body dementia. (8155133)
1993
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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UniProtKB/Swiss-Prot genetic disease variations for Lewy Body Dementia:

64
id Symbol AA change Variation ID SNP ID
1SNCAp.Glu46LysVAR_022703
2SNCAp.Glu46LysVAR_022703

Clinvar genetic disease variations for Lewy Body Dementia:

6
id Gene Name Type Significance SNP ID Assembly Location
1SNCANM_000345.3(SNCA): c.136G> A (p.Glu46Lys)single nucleotide variantPathogenicrs104893875GRCh37Chr 4, 90749321: 90749321
2SNCASNCA, DUPLICATIONduplicationPathogenic
3GBANM_001005741.2(GBA): c.1448T> C (p.Leu483Pro)single nucleotide variantPathogenicrs421016GRCh37Chr 1, 155205043: 155205043
4GBANM_001005741.2(GBA): c.1448T> C (p.Leu483Pro)single nucleotide variantPathogenicrs421016GRCh37Chr 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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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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Compounds for genes affiliated with Lewy Body Dementia

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Compounds related to Lewy Body Dementia according to GeneCards/GeneDecks:

(show top 50)    (show all 54)
idCompoundScoreTop Affiliating Genes
1thioflavin4410.2SNCA, APP
2thioflavine s4410.1SNCA, APP
3thioflavin t4410.1SNCA, APP
4methionine sulfoxide44 24 1112.1APP, SNCA
5huperzine a4410.0CHAT, APP
6physostigmine44 1111.0APP, CHAT
7propentofylline4410.0APP, CHAT
8donepezil44 50 24 1113.0APP, CHAT
91 methyl 4 phenylpyridinium449.9SNCA, SLC18A2
10apomorphine28 44 1111.9SNCA, CHAT
11formate449.9APP, SNCA
12benzodiazepine449.8SLC18A2, CHAT
13l-amino acid449.8SLC18A2, CHAT
146-hydroxydopamine449.7APP, CHAT, SNCA
15ab-42449.7APP, PSEN2
164-hydroxynonenal44 2410.7APP, CHAT, SNCA
17risperidone44 50 28 61 24 1114.6CHAT, APP
18mptp449.6SLC18A2, SNCA
19methamphetamine44 50 1111.6SLC18A2, SNCA, CHAT
20catecholamine449.6CHAT, SNCA, SLC18A2
21cocaine44 1110.5SLC18A2, SNCA, CHAT
22phospholipid449.5SNCA, CHAT, GBA
235-hydroxytryptamine449.5APP, CHAT, SLC18A2
24nicotine44 28 50 1112.4SLC18A2, CHAT, APP
25gaba449.4APP, CHAT, SLC18A2
26ethanol44 50 24 1112.4SLC18A2, SNCA, CHAT
27nmda44 2810.3APP, CHAT, SLC18A2
28amphetamine44 50 1111.3SLC18A2, SNCA
29lactacystin449.3SNCA, PSEN2, APP
30iron44 2410.3APP, SNCA, SNCB, SLC18A2
31valine449.2APP, PSEN2, SNCA
32norepinephrine44 24 1111.2APP, CHAT, SNCA, SLC18A2
33adenylate449.2GBA, APP, SNCA, EIF4G1
34acetylcholine44 50 28 24 1113.2APP, CHAT, SNCA, SLC18A2
35dopamine44 28 24 1112.1APP, CHAT, SNCA, SLC18A2
36lipid449.0SNCB, SNCA, CHAT, APP, GBA
37h2o2448.9APP, PSEN2, SNCA, SNCB
38cholesterol44 28 24 1111.9SNCA, PSEN2, APP, GBA
39alanine448.9APP, CHAT, SNCA, EIF4G1
40choline44 24 1110.9APP, CHAT, PSEN2, SNCA
41paraffin448.8CHAT, PSEN2, SNCA
42cysteine448.7SLC18A2, EIF4G1, SNCA, APP, GBA
43aspartate448.6APP, CHAT, PSEN2, SLC18A2
44oxygen44 249.6GBA, PSEN2, SNCA, SLC18A2
45nitric oxide44 24 1110.5SLC18A2, SNCA, PSEN2, CHAT, GBA
46serine448.4GBA, APP, PSEN2, SNCA, SLC18A2
47retinoic acid44 249.3APP, CHAT, PSEN2, SLC18A2
48glucose447.9GBA, CHAT, PSEN2, SNCA, EIF4G1, SLC18A2
49glutamate447.7SLC18A2, APP, CHAT, PSEN2, SNCA, EIF4G1
50calcium44 50 24 1110.5GBA, CHAT, PSEN2, SNCA, SNCB, EIF4G1

GO Terms for genes affiliated with Lewy Body Dementia

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

idNameGO IDScoreTop Affiliating Genes
1terminal boutonGO:0431959.5SLC18A2, SNCB, SNCA
2ciliary rootletGO:0352539.3APP, PSEN2
3neuromuscular junctionGO:0315949.2PSEN2, APP
4growth coneGO:0304269.1SNCB, SNCA, PSEN2
5dendritic shaftGO:0431988.9PSEN2, APP
6axonGO:0304248.6SNCA, PSEN2, CHAT, APP
7neuronal cell bodyGO:0430258.5CHAT, PSEN2, SNCB, SLC18A2
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:05080810.0SNCB, SNCA
2dendrite developmentGO:0163589.7CHAT, APP
3adult locomotory behaviorGO:0083449.7APP, SNCA
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:0072689.2SLC18A2, SNCB, CHAT
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

Sources for Lewy Body Dementia

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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
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet