Kluver Bucy Syndrome

What is Kluver Bucy Syndrome?

Kluver Bucy syndrome (KBS) is an atypical behavioural syndrome developed due to a neurological disorder. The name of this syndrome is based on two medical researchers- Heinrich Klüver and Paul Bucy, who first discover this abnormality in the rhesus monkey in 1939. It is found that the bilateral temporal injure is the most common reason behind the individuals who suffer from this neuro-behavioural syndrome. There is a controversy in medical science about the exact anatomical location responsible for the development of the KBS. It can occur at any age, but children are more susceptible.

Kluver Bucy Syndrome Symptoms

 

Following are the symptoms of KBS:

• Visual agnosia (unable to identify things without general defeat of visual discrimination)
• Too much oral propensity (oral investigation of objects)
• Hypermetamorphosis (Frequent and hasty change of ideas depends on visual consideration)
• Indifferent response without having any normal fear or anger
• Sexual behavioural alteration
• Apparently noticeable arbitrary hyper sexuality, and
• Alter in consumption behaviour.

KSB is a rare disorder and less reported identified human case history. Above mentioned symptoms are mainly discovered from animal studies. In human, these symptoms may partially or completely arises. With age variations the symptoms are more or less differ. Some symptoms are more prominent in children, such as

• Self excitation of genital organs and hyperorality
• Comprehensive cognitive and sensory insufficiency
• No affection with family members
• Convulsion or attack of seizures, particularly in case of temporal lobe epilepsy
• Tendency to put the things in mouth other than food items
• Boredom and easy attention diversions are rare

Adults are more prone to

• Develop greater than the usual sexual urge without considering social restrictions.
• Wight gain as they not eat due to their hunger, but due to the development of bulimia (eating disorder).
• Unrecognising of known place or person.

Kluver Bucy Syndrome Causes

Kluver bucy syndrome arises as an outcome of degenerative neurological ailments which mainly affects bilateral mesial temporal lobe arrangements and causes malfunctioning or damage. Anatomical studies and medical research revealed that there is a connection between the phylogenetically old medial temporal lobe regions and bilateral lesions of Ammon’s horn which involve in the progression of KBS. Rigidity or firmness of both hippocampi also causes KSB without producing any lesions in the temporal lobe. It is found that herpes simplex meningoencephalitis causes damage in the temporal lobe. The following central nervous system disorder or related ailments which cause cerebral atrophy are reasons behind KBS progression.

• Alzheimer’s disease
• Pick disease
• Herpes simplex encephalitis (HSE)
• Hypoglycemia
• Tuberculosis
• Acute sporadic porphyria
• Huntington’s disease
• Juvenile neuronal lipofuscinosis
• Toxoplasmosis
• Epilepsy
• Distressing brain damage
• Traumatic brain injury
• Meningitis
• Primary cerebral Whipple’s disease.
• Heat stroke and
• Shigellosis

Kluver Bucy Syndrome Management

The most of the cases of KBS are undiscovered or untreated due to the following limitations.

• Hesitant attitude of the parents or not consult with doctor about hypersexuality in children due to embarrassment.
• Non cooperative patient’s attitude creates barrier to diagnose the disease accurately.
• The gap between the onset of disease and the symptom development.
• Comprehensive diagnosis of KBS is difficult as thorough work-up is expensive.
• Patient and their representatives are reluctant about follow up visit for consultation with clinicians.

Patient Awareness

Some general awareness about the KBS helps to detect the syndrome in its early stage.

• If the patient is gaining weight and become obese due to the development of the eating abnormality (bulimia).
• Abnormal sexual activity or urge.
• Abrupt behavioural or emotional alterations following herpes simplex encephalitis (HSE).

Kluver Bucy Syndrome Diagnosis

The diagnosis varies from patient to patient as the causes of the syndrome differ. Some physical examinations like body temperature, consciousness, episode of seizure attacks are common. Other laboratory examinations include

• MRI
• CT scan
• Enzyme linked immunoabsorbant assay (ELISA)
• Polymerize chain reaction (PCR)
• EEG
• Cerebrospinal fluid (CSF) examination

Medicines

Diagnosis of the exact reason for the development of KBS is important to treat the patient.  KBS has some psychological disorders. Usual treatment to control the psychological activity the prescribed medicine is Selective serotonin reuptake inhibitors (SSRIs) group of drugs. Other medicines which are beneficial to control abnormal behavioural symptoms associated with KBS are haloperidol and cholinergics receptors blockers. Usually carbamazepine and leuprolides are prescribed for controlling the sexual over activity. These medications should take under the supervision of a doctor, as these medicines are having too much side effects. Medication list is modified according to the patient condition.

It is important to treat herpes simplex encephalitis (HSE) with antiviral drug such as acyclovir, as a post effect of HSE increases the chance of development of KBS.

Kluver Bucy Syndrome Life Expectancy

Kluver bucy syndrome is a life threatening disorder, but early diagnosis and proper treatment can help to survive the patient prolong. Significant features which influence mortality and morbidity are

• Aciclovir therapy completion after HSE
• Immunity of the patient
• Extent of disease and
• Degree of consciousness before introduction of treatment.

References

1. http://patient.info/doctor/kluver-bucy-syndrome
2. http://www.ncbi.nlm.nih.gov/pubmed/23622339
3. http://www.neurologyindia.com/article.asp?issn=0028-3886;year=2004;volume=52;issue=3;spage=369;epage=371;aulast=Jha
4. http://www.medscape.com/viewarticle/444160
5. http://www.bioline.org.br/pdf?ni04119