MCID: KLV001
MIFTS: 41

Kluver-Bucy Syndrome

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

Aliases & Classifications for Kluver-Bucy Syndrome

MalaCards integrated aliases for Kluver-Bucy Syndrome:

Name: Kluver-Bucy Syndrome 12 44 15 73 54 59
Bilateral Temporal Lobe Disorder 53 73
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

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 mucopolysaccharidosis, type iiia. 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 and brain, 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 29.0 APP MAPT PSEN1
2 mucopolysaccharidosis, type iiia 10.6 NAGLU SGSH
3 encephalitis 10.5
4 herpes simplex 10.4
5 herpes simplex encephalitis 10.4
6 mucopolysaccharidosis-plus syndrome 10.4 NAGLU SGSH
7 mucopolysaccharidosis iii 10.4 HGSNAT NAGLU SGSH
8 hemoglobin lepore-beta-thalassemia syndrome 10.4 HBB HBD
9 hemoglobin d disease 10.3 HBB HBD
10 lysosomal storage disease 10.3 HGSNAT NAGLU SGSH
11 malignant secondary hypertension 10.3 HBB HBD
12 hemoglobin e disease 10.3 HBB HBD
13 systemic lupus erythematosus 10.3
14 lupus erythematosus 10.3
15 hemoglobin c disease 10.3 HBB HBD
16 osgood-schlatter's disease 10.2 HBB HBD
17 fetal hemoglobin quantitative trait locus 1 10.2 HBB HBD
18 malignant essential hypertension 10.2 HBB HBD
19 progressive non-fluent aphasia 10.1 MAPT PSEN1
20 behavioral variant of frontotemporal dementia 10.1 MAPT PSEN1
21 lymphoma, hodgkin, classic 10.1
22 lymphoma, non-hodgkin, familial 10.1
23 brain injury 10.1
24 traumatic brain injury 10.1
25 lymphoma 10.1
26 nasopharyngitis 10.1
27 status epilepticus 10.1
28 epilepsy 10.1
29 cerebritis 10.1
30 bronchitis 10.1
31 meningitis 10.1
32 glioma 10.1
33 aneurysm 10.1
34 head injury 10.1
35 pleuropneumonia 10.1 HBB HBD
36 semantic dementia 10.1 MAPT PSEN1
37 echolalia 10.0 MAPT PSEN1
38 agraphia 10.0 MAPT PSEN1
39 nominal aphasia 9.9 MAPT PSEN1
40 hemoglobin h disease 9.9 HBB HBD
41 early-onset, autosomal dominant alzheimer disease 9.9 APP PSEN1
42 gait apraxia 9.8 APP PSEN1
43 hemoglobinopathy 9.8 HBB HBD
44 basal ganglia disease 9.8 MAPT PSEN1
45 early-onset familial alzheimer disease 9.7 APP PSEN1
46 sickle cell disease 9.7 HBB HBD
47 leukoencephalopathy, hereditary diffuse, with spheroids 9.6 APP MAPT
48 binswanger's disease 9.6 APP MAPT
49 niemann-pick disease, type c1 9.4 APP MAPT
50 ideomotor apraxia 9.4 ATP2B3 MAPT NAGLU 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 SGSH APP HGSNAT KNTC1 MAPT NAGLU
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

Publications for Kluver-Bucy Syndrome

Articles related to Kluver-Bucy Syndrome:

(show all 43)
# Title Authors Year
1
Partial Kluver-Bucy syndrome secondary to tubercular meningitis. ( 27530874 )
2016
2
Bilateral Anterior Opercular Syndrome With Partial Kluver-Bucy Syndrome in a Stroke Patient: A Case Report. ( 27446793 )
2016
3
Transient Kluver-Bucy syndrome from methamphetamine withdrawal. ( 25948002 )
2015
4
A case of acute-onset partial Kluver-Bucy syndrome in a patient with a history of traumatic brain injury. ( 25093767 )
2014
5
Retraction: relapsing herpes simplex encephalitis resulting in Kluver-Bucy syndrome. ( 23207135 )
2012
6
Transient Kluver-Bucy syndrome caused by cerebral edema following aneurysmal subarachnoid hemorrhage. ( 22152785 )
2012
7
Kluver-Bucy syndrome in one case with systemic lupus erythematosus. ( 21463840 )
2011
8
Relapsing herpes simplex encephalitis resulting in Kluver-Bucy syndrome. ( 21467713 )
2011
9
Temporal and pontine involvement in a case of herpes simplex encephalitis, presenting as kluver bucy syndrome - a case report. ( 22315710 )
2011
10
Kluver-Bucy syndrome, hypersexuality, and the law. ( 19927260 )
2010
11
Partial Kluver-Bucy syndrome as a delayed manifestation of head injury. ( 21180489 )
2009
12
A child with incomplete Kluver-Bucy syndrome developed during acute encephalitis. ( 18343076 )
2008
13
Kluver-Bucy syndrome in a boy with non-Hodgkin lymphoma. ( 17454782 )
2007
14
Fluctuating Kluver-Bucy syndrome in a child with epilepsy due to bilateral anterior temporal congenital malformations. ( 17218157 )
2007
15
Kluver-Bucy syndrome after mycoplasmal bronchitis. ( 16356778 )
2006
16
Kluver-Bucy syndrome--a rare complication of herpes simplex encephalitis. ( 17444064 )
2006
17
Kluver Bucy syndrome. ( 15857007 )
2005
18
Human Kluver-Bucy syndrome following acute subdural haematoma. ( 15365793 )
2004
19
Kluver-Bucy syndrome related to glioma. ( 15249638 )
2004
20
Kluver-Bucy syndrome -- an experience with six cases. ( 15472430 )
2004
21
Postcontusional Kluver-Bucy syndrome. ( 12811725 )
2003
22
Transient Kluver-Bucy syndrome following complex partial status epilepticus. ( 12791340 )
2003
23
Partial Kluver-Bucy syndrome: two cases. ( 16113630 )
2001
24
Post-encephalitic Kluver-Bucy syndrome. ( 10832228 )
2000
25
Kluver Bucy syndrome in young children. ( 9879850 )
1998
26
Clinico-radiological profile of Kluver-Bucy syndrome. ( 11273358 )
1998
27
The Kluver-Bucy syndrome. ( 9444580 )
1997
28
The Kluver-Bucy syndrome. ( 9202769 )
1997
29
Kluver-Bucy syndrome following heat stroke in a 12-year-old girl. ( 7575855 )
1995
30
Kluver-Bucy syndrome: report of a case with nasopharyngeal cancer after irradiation and chemotherapy. ( 7531610 )
1994
31
Kluver-Bucy syndrome. A case report. ( 2360478 )
1990
32
Kluver-Bucy syndrome following recovery from transtentorial herniation. ( 2803598 )
1989
33
Kluver-Bucy syndrome in systemic lupus erythematosus. ( 2915229 )
1989
34
Kluver Bucy syndrome with severe amnesia secondary to herpes encephalitis. ( 3203279 )
1988
35
Kluver-Bucy syndrome associated with delayed post-anoxic leucoencephalopathy following carbon monoxide poisoning. ( 3351520 )
1988
36
Kluver-Bucy syndrome in children. ( 3508062 )
1987
37
Kluver-Bucy syndrome in Huntington's chorea. ( 3161995 )
1985
38
Incomplete Kluver-Bucy syndrome and fluent aphasia. ( 6518356 )
1984
39
Epileptic Kluver-Bucy syndrome: case report. ( 6693368 )
1984
40
Kluver-Bucy syndrome in Pick disease: clinical and pathologic correlations. ( 7198189 )
1981
41
Pneumoencephalographic changes in Kluver-Bucy syndrome: a case report. ( 4981679 )
1969
42
Kluver-Bucy syndrome in monkeys with neocortical ablations of temporal lobe. ( 13859910 )
1961
43
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 growth cone GO:0030426 9.43 APP MAPT PSEN1
2 dendritic shaft GO:0043198 9.37 APP PSEN1
3 smooth endoplasmic reticulum GO:0005790 9.32 APP PSEN1
4 hemoglobin complex GO:0005833 9.16 HBB HBD
5 ciliary rootlet GO:0035253 8.96 APP PSEN1
6 main axon GO:0044304 8.62 APP MAPT

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

(show all 20)
# Name GO ID Score Top Affiliating Genes
1 blood coagulation GO:0007596 9.73 HBB HBD SH2B3
2 regulation of synaptic plasticity GO:0048167 9.59 MAPT PSEN1
3 cellular response to nerve growth factor stimulus GO:1990090 9.58 APP MAPT
4 neuron apoptotic process GO:0051402 9.58 APP PSEN1
5 positive regulation of phosphorylation GO:0042327 9.57 APP PSEN1
6 cellular response to amyloid-beta GO:1904646 9.55 APP PSEN1
7 regulation of protein binding GO:0043393 9.54 APP PSEN1
8 synapse organization GO:0050808 9.54 APP MAPT PSEN1
9 response to lead ion GO:0010288 9.52 APP MAPT
10 oxygen transport GO:0015671 9.51 HBB HBD
11 amyloid fibril formation GO:1990000 9.49 APP MAPT
12 modulation of age-related behavioral decline GO:0090647 9.48 APP PSEN1
13 neuron projection maintenance GO:1990535 9.46 APP PSEN1
14 positive regulation of amyloid fibril formation GO:1905908 9.43 APP PSEN1
15 regulation of epidermal growth factor-activated receptor activity GO:0007176 9.4 APP PSEN1
16 astrocyte activation involved in immune response GO:0002265 9.32 APP PSEN1
17 negative regulation of low-density lipoprotein receptor activity GO:1905598 9.26 APP PSEN1
18 smooth endoplasmic reticulum calcium ion homeostasis GO:0051563 9.16 APP PSEN1
19 glycosaminoglycan catabolic process GO:0006027 9.13 HGSNAT NAGLU SGSH
20 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 8.96 HBB HBD
2 oxygen carrier activity GO:0005344 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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