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Kluver-Bucy syndrome

MedGen UID:: 124361
•Concept ID:: C0270707
•: Mental or Behavioral Dysfunction

Synonyms:	Bilateral temporal lobe disorder; Memory loss, extreme sexual behavior, placidity, and visual distractibility; Post-encephalitic Kluver Bucy syndrome (type); Post-traumatic Kluver Bucy syndrome (type)
SNOMED CT:	Klüver-Bucy syndrome (10651001)

Monarch Initiative:	MONDO:0005817
Orphanet:	ORPHA157823

Definition

A rare neurologic disease characterized by visual agnosia, hyperorality (strong tendency to examine objects orally), hypermetamorphosis (described as the irresistible impulse to notice and react to everything within sight), hypersexuality, changes in dietary habits and hyperphagia, placidity, and amnesia, due to bilateral lesions of the temporal lobe including the hippocampus and amygdala. [from ORDO]

Term Hierarchy

GTR
MeSH
Orphanet

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVKluver-Bucy syndrome

Disease
- Disorder by Site
  - Disorder of nervous system
    - Disorder of the central nervous system
      - Brain disorder
        Dementia
        Kluver-Bucy syndrome

Follow this link to review classifications for Kluver-Bucy syndrome in Orphanet.

Professional guidelines

PubMed

Klüver-Bucy Syndrome Following Traumatic Brain Injury: A Systematic Synthesis and Review of Pharmacological Treatment From Cases in Adolescents and Adults.

Clay FJ, Kuriakose A, Lesche D, Hicks AJ, Zaman H, Azizi E, Ponsford JL, Jayaram M, Hopwood M
J Neuropsychiatry Clin Neurosci 2019 Winter;31(1):6-16. Epub 2018 Oct 31 doi: 10.1176/appi.neuropsych.18050112. PMID: 30376788

Neuropsychiatric features of frontotemporal dementia: evaluation of consensus criteria and review.

Mendez MF, Perryman KM
J Neuropsychiatry Clin Neurosci 2002 Fall;14(4):424-9. doi: 10.1176/jnp.14.4.424. PMID: 12426410

Klüver-Bucy syndrome. Successful treatment with carbamazepine.

Hooshmand H, Sepdham T, Vries JK
JAMA 1974 Sep 23;229(13):1782. PMID: 4479148

See all (3)

Recent clinical studies

Etiology

Visuospatial learning and memory in children pre- and posttemporal lobe resection: Patterns of localization and lateralization.

Stewart E, Smith ML
Epilepsy Behav 2019 May;94:189-194. Epub 2019 Apr 7 doi: 10.1016/j.yebeh.2019.03.031. PMID: 30970297

Quantifying behaviors of children with Sanfilippo syndrome: the Sanfilippo Behavior Rating Scale.

Shapiro EG, Nestrasil I, Ahmed A, Wey A, Rudser KR, Delaney KA, Rumsey RK, Haslett PA, Whitley CB, Potegal M
Mol Genet Metab 2015 Apr;114(4):594-8. Epub 2015 Mar 5 doi: 10.1016/j.ymgme.2015.02.008. PMID: 25770355 Free PMC Article

Mucopolysaccharidosis Type IIIA presents as a variant of Klüver-Bucy syndrome.

Potegal M, Yund B, Rudser K, Ahmed A, Delaney K, Nestrasil I, Whitley CB, Shapiro EG
J Clin Exp Neuropsychol 2013;35(6):608-16. Epub 2013 Jun 8 doi: 10.1080/13803395.2013.804035. PMID: 23745734 Free PMC Article

Kluver Bucy syndrome in young children.

Pradhan S, Singh MN, Pandey N
Clin Neurol Neurosurg 1998 Dec;100(4):254-8. doi: 10.1016/s0303-8467(98)00055-9. PMID: 9879850

Post-ictal Klüver-Bucy syndrome after temporal lobectomy.

Anson JA, Kuhlman DT
J Neurol Neurosurg Psychiatry 1993 Mar;56(3):311-3. doi: 10.1136/jnnp.56.3.311. PMID: 8459250 Free PMC Article

See all (23)

Diagnosis

Partial Klüver-Bucy syndrome in a Paediatric patient: A post-neurosurgical and neuropsychological cases.

Hernández-Martínez AE, Serrano-Juárez CA, Barrera-Medellín KG, Ramírez-Quiroga CI, Ramírez-Reyes AG, Casarrubias Islas R, Prieto-Corona B
J Neuropsychol 2024 Mar;18 Suppl 1:61-72. Epub 2023 May 4 doi: 10.1111/jnp.12320. PMID: 37139904

Unusual complication of Herpes simplex encephalitis: complete Klüver-Bucy syndrome.

Hernandez-Vega M, Badial-Ochoa S, Rivas-Ruvalcaba FJ, Rodriguez-Leyva I
BMJ Case Rep 2022 Jul 11;15(7) doi: 10.1136/bcr-2022-250745. PMID: 35817482 Free PMC Article

Mesiotemporal Disconnection and Hypoactivity in Klüver-Bucy Syndrome: Case Series and Literature Review.

Caro MA, Jimenez XF
J Clin Psychiatry 2016 Aug;77(8):e982-8. doi: 10.4088/JCP.14r09497. PMID: 27380585

Kluver Bucy syndrome.

Khandelwal DC, Sethi PK, Anand I
J Assoc Physicians India 2005 Jan;53:23. PMID: 15857007

Klüver-Bucy syndrome and psychiatric illness.

Clarke DJ, Brown NS
Br J Psychiatry 1990 Sep;157:439-41. doi: 10.1192/bjp.157.3.439. PMID: 2245280

See all (58)

Therapy

Partial Kluver-Bucy syndrome secondary to tubercular meningitis.

Jha KK, Singh SK, Kumar P, Arora CD
BMJ Case Rep 2016 Aug 16;2016 doi: 10.1136/bcr-2016-215926. PMID: 27530874 Free PMC Article

Kluver-Bucy syndrome, hypersexuality, and the law.

Devinsky J, Sacks O, Devinsky O
Neurocase 2010 Apr;16(2):140-5. Epub 2009 Nov 18 doi: 10.1080/13554790903329182. PMID: 19927260

Mycoplasma pneumoniae central nervous system infections.

Daxboeck F
Curr Opin Neurol 2006 Aug;19(4):374-8. doi: 10.1097/01.wco.0000236617.04958.60. PMID: 16914976

Kluver-Bucy syndrome after mycoplasmal bronchitis.

Auvichayapat N, Auvichayapat P, Watanatorn J, Thamaroj J, Jitpimolmard S
Epilepsy Behav 2006 Feb;8(1):320-2. Epub 2005 Dec 13 doi: 10.1016/j.yebeh.2005.10.005. PMID: 16356778

Kluver-Bucy syndrome in Huntington's chorea.

Janati A
J Nerv Ment Dis 1985 Oct;173(10):632-5. doi: 10.1097/00005053-198510000-00010. PMID: 3161995

See all (39)

Prognosis

Klüver-Bucy syndrome secondary to a nondominant middle cerebral artery ischemic stroke: a case report and review of the literature.

Al-Attas AA, Aldayel AY, Aloufi TH, Biary N
J Med Case Rep 2021 Jul 15;15(1):346. doi: 10.1186/s13256-021-02932-0. PMID: 34261516 Free PMC Article

Mesiotemporal Disconnection and Hypoactivity in Klüver-Bucy Syndrome: Case Series and Literature Review.

Caro MA, Jimenez XF
J Clin Psychiatry 2016 Aug;77(8):e982-8. doi: 10.4088/JCP.14r09497. PMID: 27380585

The neuropsychiatry of Parkinson's disease and related disorders.

Lauterbach EC
Psychiatr Clin North Am 2004 Dec;27(4):801-25. doi: 10.1016/j.psc.2004.07.001. PMID: 15550293

Presence of Klùver-Bucy syndrome as a positive prognostic feature for the remission of traumatic prolonged disturbances of consciousness.

Formisano R, Saltuari L, Gerstenbrand F
Acta Neurol Scand 1995 Jan;91(1):54-7. doi: 10.1111/j.1600-0404.1995.tb05843.x. PMID: 7732775

Familial progressive subcortical gliosis.

Lanska DJ, Currier RD, Cohen M, Gambetti P, Smith EE, Bebin J, Jackson JF, Whitehouse PJ, Markesbery WR
Neurology 1994 Sep;44(9):1633-43. doi: 10.1212/wnl.44.9.1633. PMID: 7936288

See all (19)