Kluver-Bucy syndrome (KBS) is a neuro-psychiatric disorder due to lesions affecting bilateral temporal lobes, especially hippocampus and amygdala.
The following clinical features characterize the syndrome:
The clinical features of KBS were initially reported by Sanger Brown and Edward Albert Sharpey-Schäfer in 1888. But the complete syndrome was described later by Heinrich Kluver (neuropsychologist) and Paul Bucy (neurosurgeon) in 1939, unaware of the previous reporting. They described the behavioral syndrome which occurred in a Rhesus monkey (named Aurora) three weeks after bilateral temporal lobectomy. The first description of KBS in humans came from Dr. Terzian and Dr. Ore in 1955 in a 19-year-old man who underwent bilateral temporal lobectomy for seizures. The first identified and reported case of KBS was in a 22-year-old male patient with bilateral temporal damage due to herpes simplex meningoencephalitis by Marlowe et al.
KBS is known to be associated with several pathologies. There are many case reports of several conditions (listed below) ranging from infections like shigellosis to methamphetamine withdrawal. But how KBS occurred in these conditions remains unclear.
The most common pathologies leading to the development of KBS are head injury and stroke in adults and herpes simplex encephalitis in children.
Human KBS is more or less restricted to case series and reports. Hence the exact prevalence is difficult to estimate.
The significant clinical symptoms of KBS are produced by the destruction of either the temporal neocortex or the amygdala bilaterally. The full syndrome is rarely seen in humans because the anterior temporal lobe dysfunction is usually less severe in humans when compared to that following total temporal lobe resection in monkeys.
The exact anatomical basis of KBS is still controversial. KBS is thought to occur due to the disturbances in the temporal portions of limbic networks that connect with multiple cortical and subcortical circuits to modulate emotional behavior and affect. A sine qua non for KBS is the involvement of medial temporal lobe regions along with bilateral lesions of the Ammon horn. Even though KBS is always thought to follow bilateral malfunctions of the temporal lobes, it is important to note that the amygdala, uncus, hippocampus, orbitofrontal and cingulate gyri, and insular cortex have an important role in its pathogenesis.
Theories regarding the etiology of KBS:
The origin of various symptoms of KBS are explainable as follows:
Placidity, hyperorality, and dietary changes are the most commonly occurring symptoms in KBS.
KBS in children usually occurs secondary to HSE with classic features occurring only in a few.
The diagnosis of KBS is mainly clinical. Once diagnosed, proper evaluation to find out the underlying pathology will be helpful in the overall management.
Magnetic resonance imaging of the brain is useful in identifying the extent of temporal lobe damage.
Electroencephalogram is also useful to identify seizures originating especially from the temporal lobe.
In head injury and other conditions producing a long duration of loss of consciousness, the appropriate staging of the consciousness is possible with the Modified Innsbruck Remission Scale, which includes the Kluver Bucy phase as well.
The treatment of KBS can be challenging due to the facts that there is no specific treatment for the condition, and the clinical course will vary from patient to patient. Most of the treatment focuses on managing the symptoms. The main drugs used in the management are:
Carbamazepine and leuprolide are used to reduce the sexual behavioral abnormality, whereas haloperidol and anticholinergics are useful in treating behavioral abnormalities associated with KBS.
KBS requires differentiation from the following conditions:
Some KBS features (i.e., hyperorality, placidity, hypermetamorphosis) persist indefinitely, whereas others gradually resolve over several years.
The clinical course of the disease varies among the case reports.
KBS occurring secondary to epileptic seizures, infections or post-infectious, and traumatic brain injuries may have a better prognosis as many of the damages would be reversible if recognized early and managed appropriately.
Due to hyperorality and hypermetamorphosis, the patient may try to put whatever objects he comes across into his mouth, which can be dangerous.
Due to hypersexuality, he may try to engage in sex with others whom he does not even know, leading to criminal procedures against the patient there is no awareness of the diagnosis.
Bulimia can cause weight gain, electrolyte disturbance, and poor oral hygiene.
Patients' relatives should be educated about the condition and counseled that treatment may not always be successful. They should receive information that situations may arise, which require physical patient restraint.
A close interaction between the treating neurologist, psychiatrist, neurosurgeon, and the radiologist is necessary for coming to the final diagnosis of KBS. Careful monitoring of diet is required if they have symptoms consistent with eating disorders. Staff members, including nurses, should be cognizant about hypersexual behaviors in these patients.
The outcomes for these patients are poor; they often require medications to suppress abnormal behavior, and often, physical restraints are needed. Many end up in psychiatric institutions where they remain for life.
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