Klüver-Bucy Syndrome
Introduction[edit | edit source]
Klüver-Bucy Syndrome (KBS) is a dysfunction arising from lesions of bilateral medial temporal lobes, including nucleus of the amygdala.[1] Though, it is a neurologic dysfunction, it may also be classified under “psychiatry”. It was first recorded among humans who had undergone temporal lobectomy in 1955[2].
The investigation leading to the discovery was carried out by Heinrich Klüver and Paul Bucy, a neurosurgeon on a number of rhesus monkeys in the 1930s.[3]
Mainly KBS presented with:
- psychic blindness or visual agnosia,
- strong oral tendencies,
- hypermetamorphosis or excessive tendency to react to every visual stimulation,
- decrease in aggressive behavior and fear reaction, and
- hypersexuality[4].
Clinical Presentation[edit | edit source]
Clinical presentations are not agreed upon and vary in literature according to source. Generally, it includes the following;[5][6][7]
- Amnesia: this is essentially inability to recall past experiences (memories) which may be anterograde – inability to recall events from the period of the amnesic episode, or retrograde – loss of memory from the period before the amnesic episode.
- Tameness: also termed “placidity” or “docility”, it is characterized by showing reduced ‘flight or fight’ response.
- Hyperphagia and dietary changes: this can present as pica (eating inappropriate objects) and/or overeating
- Hyperorality: “oral tendency or compulsion to examine objects by mouth”
- Hypersexuality: manifested as a heightened sex drive and propensity to seek sexual stimulation from unusual and inappropriate objects.
- Visual agnosia: inability to identify familial items and people.
Some presentations which are found to be inconsistent include;
- Hypermetamorphorsis; “an irresistible impulse to notice and react to everything in sight”
- Diminished or lack of emotional response
Diagnostic Procedures[edit | edit source]
It is uncommon for patients to manifest all symptoms, three or more of which is essential for diagnosis. The commonest symptoms in humans include tameness, hyperorality and dietary changes.[5]
Pathophysiology[edit | edit source]
The clinical symptoms of KBS are mainly due to[8]:
- destruction of either the temporal neocortex
- the amygdala bilaterally.
- The disturbances in the temporal portions of limbic networks that connect with multiple cortical and subcortical circuits to modulate emotional behavior and affect.
- injury to the amygdala, uncus, hippocampus, orbitofrontal and cingulate gyri, and insular cortex.[8]
This syndrome is uncommon in humans because the anterior temporal lobe dysfunction is usually less severe as compared to that following total temporal lobe resection in monkeys.
Predisposing Conditions[edit | edit source]
Conditions which predisposes an individual to the diagnosis of KBS include;[5][9]
- Temporal lobectomy
- Meningoencephalitis
- Acute herpes simplex encephalitis
- Stroke
- Pick disease
- Alzheimer’s disease
- Ischemia
- Anoxia
- Progressive subcortical gliosis
- Rett syndrome
- Porphyria
- Carbon monoxide poisoning, among others
Management / Interventions[edit | edit source]
Studies have shown pharmacotherapy as an effective way of combating KBS with literature on physiotherapy intervention and management very sparse. Pharmacological interventions have been known to include treatment with;[10]
- Carbamezine
- Valproate
- Topiramate
- Quetiapine,
- Propranolol
- Benztropine
- Haloperidol
- Trazodone
- Sertraline
- Olanzapine
- Lorazepam
- Valproic acid
- Thiothixene
- Bromocriptine
References[edit | edit source]
- ↑ Afifi A, Bergman R. Functional Neuroanatomy. McGraw-Hill. The Kluver-Bucy syndrome is a clinical syndrome observed in humans and other animals after bilateral lesions in the temporal lobe that involve the amygdala, hippocampal formation, and adjacent neural structures,1998.
- ↑ Terzian H, Ore GD. Syndrome of Klüver and Bucy; reproduced in man by bilateral removal of the temporal lobes. Neurology. 1955 Jun;5(6):373-80.
- ↑ Klüver H, Bucy P. Preliminary analysis of functions of the temporal lobes in monkeys. Arch Neurol Psychiatry. 1939; 42:979-1000
- ↑ Hooshmand H, Sepdham T, Vries JK. Klüver-Bucy Syndrome: Successful Treatment With Carbamazepine. JAMA. 1974;229(13):1782. doi:10.1001/jama.1974.03230510056026
- ↑ 5.0 5.1 5.2 Salloway S, Malloy P; Cummings. The Neuropsychiatry of Limbic and Subcortical Disorders. American Psychiatric Pub.1997. p. 125. ISBN 0-88048-942-1.
- ↑ Ozawa H, Sasaki M; Sugai K; Hashimoto T; Matsuda H, Takashima S, Uno A, Okawa T. "Single-Photon Emission CT and MR Findings in Klüver-Bucy" (PDF). American journal of neuroradiology (Oak Brook, IL,: American Society of Neuroradiology), 1997, 18 (3): 540–542. ISSN 0195-6108. PMID 9090419
- ↑ Afifi, Adel K., Bergman R. Functional Neuroanatomy: Text and Atlas. McGraw-Hill Professional. 2005, p. 299. ISBN 0-07-140812-6.
- ↑ 8.0 8.1 Lanska DJ. The Klüver-Bucy Syndrome. Front Neurol Neurosci. 2018;41:77-89.
- ↑ Tancredi, Laurence R. Hardwired Behavior: What Neuroscience Reveals about Morality. Cambridge University Press. 2005. pp. 98–99. ISBN 0-521-86001-6.
- ↑ Clay FJ, Kiriakose A, Lesche D, Hicks AJ, zaman H, Azizi E, Ponsford JL, Jayaram M. Hopwood M. Klüver-Bucy Syndrome following Traumatic Brain Injury: a systematic synthesis and review of pharmacological treatment from case in adolescent and adult. Journal of neuropsychiatry and clinical neurosciences. 2019:31:1, 6-16