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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

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVKluver-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

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: 25770355Free 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: 23745734Free 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: 8459250Free PMC Article

Diagnosis

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: 35817482Free PMC Article
Clinical manifestations and morphological changes in one case with post-stroke Klüver-Bucy syndrome.
Hreniuc NC, NeamŢu C, Sferdian MF, Ghib Para CG, Precup CV, Ioiart I
Rom J Morphol Embryol 2017;58(2):665-669. PMID: 28730259
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 Kluver-Bucy syndrome.
Góscínski I, Kwiatkowski S, Polak J, Orlowiejska M, Partyk A
J Neurosurg Sci 1997 Sep;41(3):269-72. PMID: 9444580
Kluver-Bucy syndrome in children.
Tonsgard JH, Harwicke N, Levine SC
Pediatr Neurol 1987 May-Jun;3(3):162-5. doi: 10.1016/0887-8994(87)90084-1. PMID: 3508062

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: 27530874Free 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
Neuroleptic-induced Klüver-Bucy syndrome.
Varga E, Haher EJ, Simpson GM
Biol Psychiatry 1975 Feb;10(1):65-8. PMID: 235332
Klüver-Bucy syndrome. Successful treatment with carbamazepine.
Hooshmand H, Sepdham T, Vries JK
JAMA 1974 Sep 23;229(13):1782. PMID: 4479148

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: 34261516Free 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

Clinical prediction guides

Hrayr Terzian (1925-1988): a life between experimental neurophysiology and clinical neurology.
Brigo F
Neurol Sci 2021 Sep;42(9):3939-3942. Epub 2021 Jan 26 doi: 10.1007/s10072-021-05070-z. PMID: 33495930
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 neuropsychology of the Klüver-Bucy syndrome in children.
Lippe S, Gonin-Flambois C, Jambaqué I
Handb Clin Neurol 2013;112:1285-8. doi: 10.1016/B978-0-444-52910-7.00051-9. PMID: 23622339
The Kluver-Bucy syndrome.
Góscínski I, Kwiatkowski S, Polak J, Orlowiejska M, Partyk A
J Neurosurg Sci 1997 Sep;41(3):269-72. PMID: 9444580
The Kluver-Bucy syndrome.
Gościński I, Kwiatkowski S, Polak J, Orłowiejska M
Acta Neurochir (Wien) 1997;139(4):303-6. doi: 10.1007/BF01808825. PMID: 9202769

Recent systematic reviews

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

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