MCID: KLV001
MIFTS: 34

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 58 44 15 17 70
Bilateral Temporal Lobe Disorder 20 70
Klüver-Bucy Syndrome 73 53
Memory Loss, Extreme Sexual Behavior, Placidity, and Visual Distractibility 20
Post-Encephalitic Kluver Bucy Syndrome 20
Post-Traumatic Kluver Bucy Syndrome 20
Kluver Bucy Syndrome 20
Klver-Bucy Syndrome 12

Classifications:

Orphanet: 58  
Rare neurological diseases
Rare infectious diseases


External Ids:

Disease Ontology 12 DOID:2510
MeSH 44 D020232
NCIt 50 C84802
SNOMED-CT 67 10651001
MESH via Orphanet 45 D020232
UMLS via Orphanet 71 C0270707
Orphanet 58 ORPHA157823
UMLS 70 C0270707 C2931871

Summaries for Kluver-Bucy Syndrome

GARD : 20 Kluver Bucy syndrome is 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 mucopolysaccharidoses and mucopolysaccharidosis iii. An important gene associated with Kluver-Bucy Syndrome is SGSH (N-Sulfoglucosamine Sulfohydrolase), and among its related pathways/superpathways are Lysosome and Glycosaminoglycan degradation. Affiliated tissues include temporal lobe, brain and cortex, and related phenotypes are nervous system and renal/urinary system

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.

NINDS : 53 Klüver-Bucy syndrome is a rare behavioral impairment that is associated with damage to both of the anterior temporal lobes of the brain. It causes individuals to put objects in their mouths and engage in inappropriate sexual behavior. Other symptoms may include visual agnosia (inability to visually recognize objects), loss of normal fear and anger responses, memory loss, distractibility, seizures, and dementia. The disorder may be associated with herpes encephalitis and trauma, which can result in brain damage.

Wikipedia : 73 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 113)
# Related Disease Score Top Affiliating Genes
1 mucopolysaccharidoses 30.4 SGSH NAGLU HGSNAT
2 mucopolysaccharidosis iii 30.4 SGSH NAGLU HGSNAT
3 mucopolysaccharidosis-plus syndrome 30.3 SGSH NAGLU HGSNAT
4 prosopagnosia 30.2 OXT GRN
5 mucopolysaccharidosis, type iiic 30.2 SGSH NAGLU HGSNAT
6 mucopolysaccharidosis, type iiia 30.1 SGSH NAGLU HGSNAT
7 lysosomal storage disease 30.0 SGSH NAGLU HGSNAT
8 encephalitis 10.7
9 agnosia 10.6
10 visual agnosia 10.6
11 herpes simplex encephalitis 10.6
12 herpes simplex 10.5
13 meningoencephalitis 10.5
14 head injury 10.5
15 aphasia 10.4
16 encephalopathy 10.4
17 systemic lupus erythematosus 10.3
18 ocular motor apraxia 10.3
19 yemenite deaf-blind hypopigmentation syndrome 10.3
20 auditory agnosia 10.3
21 amnestic disorder 10.3
22 status epilepticus 10.3
23 viral encephalitis 10.3
24 tuberculous meningitis 10.3
25 traumatic brain injury 10.3
26 alzheimer disease 10.3
27 frontotemporal dementia 10.3
28 apraxia 10.3
29 hydrocephalus 10.3
30 porphyria 10.3
31 acute disseminated encephalomyelitis 10.3
32 hypoglycemia 10.3
33 seizure disorder 10.3
34 mucopolysaccharidosis, type vi 10.2 NAGLU HGSNAT
35 huntington disease 10.1
36 attention deficit-hyperactivity disorder 10.1
37 pick disease of brain 10.1
38 lymphoma, hodgkin, classic 10.1
39 adrenoleukodystrophy 10.1
40 ataxia and polyneuropathy, adult-onset 10.1
41 lymphoma, non-hodgkin, familial 10.1
42 nasopharyngeal carcinoma 10.1
43 encephalopathy, acute, infection-induced 1 10.1
44 chorea, childhood-onset, with psychomotor retardation 10.1
45 limbic encephalitis 10.1
46 cysticercosis 10.1
47 hemiplegia 10.1
48 choreatic disease 10.1
49 reye syndrome 10.1
50 temporal lobe epilepsy 10.1

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 nervous system MP:0003631 9.5 AMT GRN HCRT HGSNAT NAGLU OXT
2 renal/urinary system MP:0005367 9.02 GRN HGSNAT NAGLU OXT SGSH

Drugs & Therapeutics for Kluver-Bucy Syndrome

Search Clinical Trials , 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:

40
Temporal Lobe, Brain, Cortex, Amygdala, Prefrontal Cortex

Publications for Kluver-Bucy Syndrome

Articles related to Kluver-Bucy Syndrome:

(show top 50) (show all 226)
# Title Authors PMID Year
1
Hrayr Terzian (1925-1988): a life between experimental neurophysiology and clinical neurology. 61
33495930 2021
2
Sexual Behavioral Disinhibition Associated with Nucleus Lentiformis Lesion: A Forensic Neuroscience Perspective Through a Case. 61
32525581 2020
3
Neuropsychiatry of Susac Syndrome: a Case Report. 61
33735032 2020
4
Klüver-Bucy Syndrome after a Head Trauma in Conditions of Child Abuse and Neglect. 61
33370744 2020
5
Kluver-Bucy syndrome in a girl with anti-NMDAR encephalitis. 61
31347577 2019
6
Klüver-Bucy Syndrome Following Traumatic Brain Injury: A Systematic Synthesis and Review of Pharmacological Treatment From Cases in Adolescents and Adults. 61
30376788 2019
7
Pediatric Klüver-Bucy Syndrome: Report of Two Cases and Review of the Literature. 61
30086555 2018
8
Kluver-Bucy Syndrome in an Adolescent Girl: A Sequel of Encephalitis. 61
30937109 2018
9
Klüver & Bucy syndrome: an investigation of social and affective cognition. 61
30247092 2018
10
Pediatric Klüver-Bucy Syndrome: Report of Two Cases and Review of the Literature. 61
29237192 2018
11
The Klüver-Bucy Syndrome. 61
29145186 2018
12
Editorial. The Klüver-Bucy syndrome and the golden age of localization. 61
28859563 2017
13
Sanger Brown and Edward Schäfer before Heinrich Klüver and Paul Bucy: their observations on bilateral temporal lobe ablations. 61
28859570 2017
14
Klüver-Bucy syndrome associated with a recessive variant in HGSNAT in two siblings with Mucopolysaccharidosis type IIIC (Sanfilippo C). 61
27827379 2017
15
Clinical manifestations and morphological changes in one case with post-stroke Klüver-Bucy syndrome. 61
28730259 2017
16
Partial Kluver-Bucy syndrome secondary to tubercular meningitis. 61
27530874 2016
17
Mesiotemporal Disconnection and Hypoactivity in Klüver-Bucy Syndrome: Case Series and Literature Review. 61
27380585 2016
18
Bilateral Anterior Opercular Syndrome With Partial Kluver-Bucy Syndrome in a Stroke Patient: A Case Report. 61
27446793 2016
19
Coprophagia in neurologic disorders. 61
27017341 2016
20
Frontotemporal dementia mimicking bipolar affective disorder, normal pressure hydrocephalus and Klüver-Bucy syndrome. 61
26482719 2015
21
[Update on Herpes Simplex Encephalitis]. 61
26160820 2015
22
[Nonconvulsive status epilepticus; progress in clinical practice and research]. 61
25957201 2015
23
Quantifying behaviors of children with Sanfilippo syndrome: the Sanfilippo Behavior Rating Scale. 61
25770355 2015
24
Transient Kluver-Bucy syndrome from methamphetamine withdrawal. 61
25948002 2015
25
Catatonia and klüver-bucy syndrome in a patient with acute disseminated encephalomyelitis. 61
25923869 2015
26
Hypersexuality in neurocognitive disorders in elderly people - a comprehensive review of the literature and case study. 61
25413509 2014
27
[Klüver-Bucy syndrome complicating herpes meningoencephalitis in a pregnant woman]. 61
24703732 2014
28
A case of acute-onset partial Kluver-Bucy syndrome in a patient with a history of traumatic brain injury. 61
25093767 2014
29
[Klüver-Bucy syndrome after herpes simplex encephalitis]. 61
23846508 2013
30
Klüver-Bucy syndrome after unilateral frontotemporal resection in a child with tuberous sclerosis. 61
23636147 2013
31
[Epilepsy and cognitive dysfunction]. 61
23667119 2013
32
The neuropsychology of the Klüver-Bucy syndrome in children. 61
23622339 2013
33
Mucopolysaccharidosis Type IIIA presents as a variant of Klüver-Bucy syndrome. 61
23745734 2013
34
Successful treatment of severe disruptive disorder featuring symptoms of the Klüver-Bucy Syndrome following a massive right temporal-parietal hemorrhage. 61
22215142 2013
35
Violence, mental illness, and the brain - A brief history of psychosurgery: Part 2 - From the limbic system and cingulotomy to deep brain stimulation. 61
23776761 2013
36
Transient Kluver-Bucy syndrome caused by cerebral edema following aneurysmal subarachnoid hemorrhage. 61
22152785 2012
37
Klüver-bucy syndrome with isolated bilateral hippocampal atrophy following status epilepticus. 61
24649454 2012
38
Retraction: Relapsing herpes simplex encephalitis resulting in Kluver-Bucy syndrome. 61
23207135 2012
39
Superior colliculus lesions impair threat responsiveness in infant capuchin monkeys. 61
21970966 2011
40
Kluver-Bucy syndrome in one case with systemic lupus erythematosus. 61
21463840 2011
41
Relapsing herpes simplex encephalitis resulting in Kluver-Bucy syndrome. 61
21467713 2011
42
Temporal and pontine involvement in a case of herpes simplex encephalitis, presenting as kluver bucy syndrome - a case report. 61
22315710 2011
43
Effects of neonatal medial versus lateral temporal cortex injury: theoretical comment on Malkova et al. (2010). 61
21133537 2010
44
Ictal kissing: a release phenomenon in non-dominant temporal lobe epilepsy. 61
21059491 2010
45
Long-term neuropsychological follow-up of a child with Klüver-Bucy syndrome. 61
20926352 2010
46
Klüver Bucy syndrome following hypoglycaemic coma in a patient with glycogen storage disease type Ib. 61
21103936 2010
47
Partial Klüver-Bucy syndrome in a patient with acute disseminated encephalomyelitis. 61
20638284 2010
48
The effect of surgery on the postictal state. 61
20801722 2010
49
[Postraumatic klüver-Bucy syndrome--description and comparison of two clinical cases]. 61
20672516 2010
50
Kluver-Bucy syndrome, hypersexuality, and the law. 61
19927260 2010

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

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

# Super pathways Score Top Affiliating Genes
1 11.24 SGSH NAGLU HGSNAT
2
Show member pathways
10.1 SGSH NAGLU HGSNAT

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 lysosome GO:0005764 8.92 SGSH NAGLU HGSNAT GRN

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

# Name GO ID Score Top Affiliating Genes
1 lysosome organization GO:0007040 9.32 NAGLU GRN
2 eating behavior GO:0042755 9.26 OXT HCRT
3 lysosomal transport GO:0007041 9.16 HGSNAT GRN
4 sleep GO:0030431 8.96 OXT HCRT
5 glycosaminoglycan catabolic process GO:0006027 8.8 SGSH NAGLU HGSNAT

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

# Name GO ID Score Top Affiliating Genes
1 neuropeptide hormone activity GO:0005184 8.62 OXT HCRT

Sources for Kluver-Bucy Syndrome

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Apr-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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