MCID: KLV001
MIFTS: 42

Kluver-Bucy Syndrome

Categories: Eye diseases, Infectious diseases, Mental diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Kluver-Bucy Syndrome

MalaCards integrated aliases for Kluver-Bucy Syndrome:

Name: Kluver-Bucy Syndrome 12 44 15 73
Bilateral Temporal Lobe Disorder 53 73
Klüver-Bucy Syndrome 54 59
Memory Loss, Extreme Sexual Behavior, Placidity, and Visual Distractibility 53
Post-Encephalitic Kluver Bucy Syndrome 53
Post-Traumatic Kluver Bucy Syndrome 53
Kluver Bucy Syndrome 53
Klver-Bucy Syndrome 12
Kl�verbucy Syndrome 76

Classifications:



External Ids:

Disease Ontology 12 DOID:2510
MeSH 44 D020232
NCIt 50 C84802
SNOMED-CT 68 10651001
Orphanet 59 ORPHA157823
MESH via Orphanet 45 D020232
UMLS via Orphanet 74 C0270707

Summaries for Kluver-Bucy Syndrome

NIH Rare Diseases : 53 Kluver Bucy syndromeis a rare behavioral impairment characterized by inappropriate sexual behaviors and mouthing of objects. Other signs and symptoms, include a diminished ability to visually recognize objects, loss of normal fear and anger responses, memory loss, distractibility, seizures, and dementia. It is associated with damage to the anterior temporal lobes of the brain. Cases have been reported in association with herpes encephalitis and head trauma. Treatment is symptomatic and may include the use of psychotropic medications.

MalaCards based summary : Kluver-Bucy Syndrome, also known as bilateral temporal lobe disorder, is related to aphasia and encephalitis. An important gene associated with Kluver-Bucy Syndrome is HGSNAT (Heparan-Alpha-Glucosaminide N-Acetyltransferase), and among its related pathways/superpathways are Response to elevated platelet cytosolic Ca2+ and Factors involved in megakaryocyte development and platelet production. Affiliated tissues include temporal lobe, brain and eye, and related phenotypes are behavior/neurological and cellular

Disease Ontology : 12 An impulse control disorder that involves abnormalities in memory, social and sexual functioning and idiosyncratic behaviors resulting from damage from trauma or infection to both temporal lobes causing individuals to put objects in their mouths and engage in inappropriate sexual behavior.

Wikipedia : 76 Klüver�??Bucy syndrome is a syndrome resulting from bilateral lesions of the medial temporal lobe... more...

Related Diseases for Kluver-Bucy Syndrome

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

(show top 50) (show all 63)
# Related Disease Score Top Affiliating Genes
1 aphasia 30.0 APP MAPT PSEN1
2 encephalitis 10.7
3 herpes simplex 10.6
4 herpes simplex encephalitis 10.6
5 systemic lupus erythematosus 10.4
6 lupus erythematosus 10.4
7 encephalopathy 10.3
8 lymphoma, hodgkin, classic 10.3
9 lymphoma, non-hodgkin, familial 10.3
10 brain injury 10.3
11 traumatic brain injury 10.3
12 lymphoma 10.3
13 status epilepticus 10.3
14 epilepsy 10.3
15 bronchitis 10.3
16 meningitis 10.3
17 glioma 10.3
18 head injury 10.3
19 mucopolysaccharidosis, type iiia 10.2 NAGLU SGSH
20 mucopolysaccharidosis iii 10.1 HGSNAT NAGLU SGSH
21 mucopolysaccharidosis-plus syndrome 10.1 NAGLU SGSH
22 hemoglobin lepore-beta-thalassemia syndrome 10.1 HBB HBD
23 hemoglobin d disease 10.1 HBB HBD
24 lysosomal storage disease 10.1 HGSNAT NAGLU SGSH
25 malignant secondary hypertension 10.1 HBB HBD
26 hemoglobin c disease 10.1 HBB HBD
27 hemoglobin e disease 10.1 HBB HBD
28 osgood-schlatter's disease 10.1 HBB HBD
29 fetal hemoglobin quantitative trait locus 1 10.1 HBB HBD
30 malignant essential hypertension 10.1 HBB HBD
31 blind loop syndrome 10.0 HBB HBD
32 nominal aphasia 10.0 MAPT PSEN1
33 pleuropneumonia 10.0 HBB HBD
34 progressive non-fluent aphasia 10.0 MAPT PSEN1
35 behavioral variant of frontotemporal dementia 10.0 MAPT PSEN1
36 blood protein disease 10.0 HBB HBD
37 semantic dementia 10.0 MAPT PSEN1
38 alpha thalassemia-intellectual disability syndrome type 1 10.0 HBB HBD
39 echolalia 10.0 MAPT PSEN1
40 agraphia 10.0 MAPT PSEN1
41 hemoglobin h disease 10.0 HBB HBD
42 early-onset, autosomal dominant alzheimer disease 10.0 APP PSEN1
43 gait apraxia 9.9 APP PSEN1
44 sickle cell disease 9.9 HBB HBD
45 frontotemporal dementia and/or amyotrophic lateral sclerosis 1 9.9 MAPT PSEN1
46 dopamine beta-hydroxylase deficiency, congenital 9.9 APP PSEN1
47 alzheimer disease mitochondrial 9.9 APP MAPT
48 binswanger's disease 9.9 APP MAPT
49 hemoglobinopathy 9.8 HBB HBD
50 familial idiopathic basal ganglia calcification 9.7 APP MAPT PSEN1

Graphical network of the top 20 diseases related to Kluver-Bucy Syndrome:



Diseases related to Kluver-Bucy Syndrome

Symptoms & Phenotypes for Kluver-Bucy Syndrome

MGI Mouse Phenotypes related to Kluver-Bucy Syndrome:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 behavior/neurological MP:0005386 9.91 APP HGSNAT KNTC1 MAPT NAGLU PSEN1
2 cellular MP:0005384 9.87 APP HGSNAT MAPT NAGLU PSEN1 SGSH
3 hematopoietic system MP:0005397 9.8 APP HGSNAT MAPT NAGLU PSEN1 SGSH
4 immune system MP:0005387 9.7 APP HGSNAT MAPT NAGLU PSEN1 SGSH
5 integument MP:0010771 9.43 APP KNTC1 MAPT NAGLU PSEN1 SGSH
6 renal/urinary system MP:0005367 9.02 HGSNAT MAPT NAGLU PSEN1 SGSH

Drugs & Therapeutics for Kluver-Bucy Syndrome

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Neurobehavioral Phenotypes in MPS III Completed NCT01873911

Search NIH Clinical Center for Kluver-Bucy Syndrome

Cochrane evidence based reviews: kluver-bucy syndrome

Genetic Tests for Kluver-Bucy Syndrome

Anatomical Context for Kluver-Bucy Syndrome

MalaCards organs/tissues related to Kluver-Bucy Syndrome:

41
Temporal Lobe, Brain, Eye

Publications for Kluver-Bucy Syndrome

Articles related to Kluver-Bucy Syndrome:

(show all 50)
# Title Authors Year
1
Klüver-Bucy Syndrome Following Traumatic Brain Injury: A Systematic Synthesis and Review of Pharmacological Treatment From Cases in Adolescents and Adults. ( 30376788 )
2018
2
Pediatric Klüver-Bucy Syndrome: Report of Two Cases and Review of the Literature. ( 30086555 )
2018
3
Klüver & Bucy syndrome: an investigation of social and affective cognition. ( 30247092 )
2018
4
Partial Kluver-Bucy syndrome secondary to tubercular meningitis. ( 27530874 )
2016
5
Bilateral Anterior Opercular Syndrome With Partial Kluver-Bucy Syndrome in a Stroke Patient: A Case Report. ( 27446793 )
2016
6
Transient Kluver-Bucy syndrome from methamphetamine withdrawal. ( 25948002 )
2015
7
A case of acute-onset partial Kluver-Bucy syndrome in a patient with a history of traumatic brain injury. ( 25093767 )
2014
8
Retraction: relapsing herpes simplex encephalitis resulting in Kluver-Bucy syndrome. ( 23207135 )
2012
9
Transient Kluver-Bucy syndrome caused by cerebral edema following aneurysmal subarachnoid hemorrhage. ( 22152785 )
2012
10
Kluver-Bucy syndrome in one case with systemic lupus erythematosus. ( 21463840 )
2011
11
Relapsing herpes simplex encephalitis resulting in Kluver-Bucy syndrome. ( 21467713 )
2011
12
Temporal and pontine involvement in a case of herpes simplex encephalitis, presenting as kluver bucy syndrome - a case report. ( 22315710 )
2011
13
Kluver-Bucy syndrome, hypersexuality, and the law. ( 19927260 )
2010
14
Partial Kluver-Bucy syndrome as a delayed manifestation of head injury. ( 21180489 )
2009
15
A child with incomplete Kluver-Bucy syndrome developed during acute encephalitis. ( 18343076 )
2008
16
Kluver-Bucy syndrome in a boy with non-Hodgkin lymphoma. ( 17454782 )
2007
17
Fluctuating Kluver-Bucy syndrome in a child with epilepsy due to bilateral anterior temporal congenital malformations. ( 17218157 )
2007
18
Kluver-Bucy syndrome after mycoplasmal bronchitis. ( 16356778 )
2006
19
Kluver-Bucy syndrome--a rare complication of herpes simplex encephalitis. ( 17444064 )
2006
20
Kluver Bucy syndrome. ( 15857007 )
2005
21
Kluver - Bucy Syndrome Following Herpes Simplex Encephalitis. ( 27407819 )
2005
22
Human Kluver-Bucy syndrome following acute subdural haematoma. ( 15365793 )
2004
23
Kluver-Bucy syndrome related to glioma. ( 15249638 )
2004
24
Kluver-Bucy syndrome -- an experience with six cases. ( 15472430 )
2004
25
Postcontusional Kluver-Bucy syndrome. ( 12811725 )
2003
26
Transient Kluver-Bucy syndrome following complex partial status epilepticus. ( 12791340 )
2003
27
Partial Kluver-Bucy syndrome: two cases. ( 16113630 )
2001
28
Post-encephalitic Kluver-Bucy syndrome. ( 10832228 )
2000
29
Kluver Bucy syndrome in young children. ( 9879850 )
1998
30
Clinico-radiological profile of Kluver-Bucy syndrome. ( 11273358 )
1998
31
Klüver-Bucy syndrome in late delayed postirradiation encephalopathy. ( 9669483 )
1998
32
The Kluver-Bucy syndrome. ( 9444580 )
1997
33
The Kluver-Bucy syndrome. ( 9202769 )
1997
34
Kluver-Bucy syndrome following heat stroke in a 12-year-old girl. ( 7575855 )
1995
35
Unilateral temporal lobe damage and the partial Kluver-Bucy syndrome. ( 24487427 )
1995
36
Kluver-Bucy syndrome: report of a case with nasopharyngeal cancer after irradiation and chemotherapy. ( 7531610 )
1994
37
Kluver-Bucy syndrome. A case report. ( 2360478 )
1990
38
Kluver-Bucy syndrome following recovery from transtentorial herniation. ( 2803598 )
1989
39
Kluver-Bucy syndrome in systemic lupus erythematosus. ( 2915229 )
1989
40
Kluver Bucy syndrome with severe amnesia secondary to herpes encephalitis. ( 3203279 )
1988
41
Kluver-Bucy syndrome associated with delayed post-anoxic leucoencephalopathy following carbon monoxide poisoning. ( 3351520 )
1988
42
Kluver-Bucy syndrome in children. ( 3508062 )
1987
43
Kluver-Bucy syndrome in Huntington's chorea. ( 3161995 )
1985
44
Incomplete Kluver-Bucy syndrome and fluent aphasia. ( 6518356 )
1984
45
Epileptic Kluver-Bucy syndrome: case report. ( 6693368 )
1984
46
Kluver-Bucy syndrome in Pick disease: clinical and pathologic correlations. ( 7198189 )
1981
47
The Klüver-Bucy syndrome produced by partial isolation of the temporal lobe. ( 4825731 )
1974
48
Pneumoencephalographic changes in Kluver-Bucy syndrome: a case report. ( 4981679 )
1969
49
Kluver-Bucy syndrome in monkeys with neocortical ablations of temporal lobe. ( 13859910 )
1961
50
Rhinencephalic lesions and behavior in cats: an analysis of the Kluver-Bucy syndrome with particular reference to normal and abnormal sexual behavior. ( 13525460 )
1957

Variations for Kluver-Bucy Syndrome

Expression for Kluver-Bucy Syndrome

Search GEO for disease gene expression data for Kluver-Bucy Syndrome.

Pathways for Kluver-Bucy Syndrome

GO Terms for Kluver-Bucy Syndrome

Cellular components related to Kluver-Bucy Syndrome according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 membrane raft GO:0045121 9.61 APP MAPT PSEN1
2 growth cone GO:0030426 9.5 APP MAPT PSEN1
3 dendritic shaft GO:0043198 9.4 APP PSEN1
4 smooth endoplasmic reticulum GO:0005790 9.37 APP PSEN1
5 hemoglobin complex GO:0005833 9.26 HBB HBD
6 haptoglobin-hemoglobin complex GO:0031838 9.16 HBB HBD
7 ciliary rootlet GO:0035253 8.96 APP PSEN1
8 main axon GO:0044304 8.62 APP MAPT

Biological processes related to Kluver-Bucy Syndrome according to GeneCards Suite gene sharing:

(show all 21)
# Name GO ID Score Top Affiliating Genes
1 blood coagulation GO:0007596 9.73 HBB HBD SH2B3
2 regulation of synaptic plasticity GO:0048167 9.6 MAPT PSEN1
3 cellular response to nerve growth factor stimulus GO:1990090 9.59 APP MAPT
4 neuron apoptotic process GO:0051402 9.58 APP PSEN1
5 cellular response to amyloid-beta GO:1904646 9.58 APP PSEN1
6 positive regulation of phosphorylation GO:0042327 9.57 APP PSEN1
7 hydrogen peroxide catabolic process GO:0042744 9.55 HBB HBD
8 regulation of protein binding GO:0043393 9.54 APP PSEN1
9 response to lead ion GO:0010288 9.52 APP MAPT
10 oxygen transport GO:0015671 9.51 HBB HBD
11 synapse organization GO:0050808 9.5 APP MAPT PSEN1
12 neuron projection maintenance GO:1990535 9.49 APP PSEN1
13 amyloid fibril formation GO:1990000 9.48 APP MAPT
14 modulation of age-related behavioral decline GO:0090647 9.46 APP PSEN1
15 negative regulation of low-density lipoprotein receptor activity GO:1905598 9.43 APP PSEN1
16 positive regulation of amyloid fibril formation GO:1905908 9.4 APP PSEN1
17 regulation of epidermal growth factor-activated receptor activity GO:0007176 9.37 APP PSEN1
18 astrocyte activation involved in immune response GO:0002265 9.26 APP PSEN1
19 smooth endoplasmic reticulum calcium ion homeostasis GO:0051563 9.16 APP PSEN1
20 glycosaminoglycan catabolic process GO:0006027 9.13 HGSNAT NAGLU SGSH
21 astrocyte activation GO:0048143 8.8 APP MAPT PSEN1

Molecular functions related to Kluver-Bucy Syndrome according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 oxygen binding GO:0019825 9.37 HBB HBD
2 peroxidase activity GO:0004601 9.32 HBB HBD
3 oxygen carrier activity GO:0005344 9.26 HBB HBD
4 organic acid binding GO:0043177 9.16 HBB HBD
5 haptoglobin binding GO:0031720 8.96 HBB HBD
6 hemoglobin alpha binding GO:0031721 8.62 HBB HBD

Sources for Kluver-Bucy Syndrome

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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