|Year : 2015 | Volume
| Issue : 4 | Page : 373-375
Kluver-Bucy syndrome: A morbid consequences of post herpes simplex encephalitis
Ajay Halder1, Uday Sankar Mandal2, Subrata Halder1, Abhinanda Biswas3
1 Department of Psychiatry, Institute of Post Graduate Medical Education and Research Hospital, Belur, West Bengal, India
2 Department of Psychiatry, R. G. Kar Medical College and Hospital, Kolkata, West Bengal, India
3 Department of General Medicine, Medical Officer of ESI Hospital, Belur, West Bengal, India
|Date of Web Publication||18-Sep-2015|
Puspak Apartment, GR-FR, FL-A, 14/17A, East Mall Road, Kolkata - 700 080, West Bengal
Source of Support: Nil., Conflict of Interest: No.
Kluver-Bucy syndrome (KBS) is a neuropsychiatric behavioral disorder that is most commonly associated with damage to both of the anterior temporal lobes of the brain. Most commonly it is due to infection of herpes simplex encephalitis (HSE). Although computed tomography (CT), magnetic resonance imaging (MRI) and electroencephalography (EEG) suggest the injury to the temporal region of brain, the definitive diagnosis is only established by the finding of the viral antigen in brain. Previously viral antigen is obtained by biopsy, currently it is demonstrated by the polymerase chain reaction technique. As we all know, it is a very rare disorder and most of the literature relates to animal models rather than human cases. We here describe an important human case of KBS following HSE.
Keywords: Herpes simplex encephalitis, herpes simplex virus, Kluver-Bucy syndrome
|How to cite this article:|
Halder A, Mandal US, Halder S, Biswas A. Kluver-Bucy syndrome: A morbid consequences of post herpes simplex encephalitis. CHRISMED J Health Res 2015;2:373-5
|How to cite this URL:|
Halder A, Mandal US, Halder S, Biswas A. Kluver-Bucy syndrome: A morbid consequences of post herpes simplex encephalitis. CHRISMED J Health Res [serial online] 2015 [cited 2020 Aug 14];2:373-5. Available from: http://www.cjhr.org/text.asp?2015/2/4/373/165742
| Introduction|| |
Kluver-Bucy syndrome (KBS) is a constellation of group of cognitive dysfunctions which includes psychic blindness or visual agnosia (i. e., inability to recognize familiar objects), hypersexuality, emotional behavioral alteration (particularly placidity), hyperorality, hypermetamorphosis (i. e., strong impulse to react and explore to every visual stimulus) and memory deficits., Kluver-Bucy syndrome is associated on the etiological ground with a variety of neuropathological disorders such as herpes simplex encephalitis (HSE), Pick's disease, Alzheimer's disease, cerebrovascular accidents, cerebral trauma, and temporal lobe epilepsy. The important common feature of all these etiologies is bilateral mesial temporal lobe destruction or dysfunction. Although the clinical findings of KBS are almost similar in both rhesus monkey and human, but some features such as amnesia, aphasia, dementia, and seizures are exclusively seen in human.,,
| Case Report|| |
Mr. D. K., 18-year-old, Muslim, unmarried male from a rural background of West Bengal (India) presented with acute onset of fever, vomiting, severe headache for last 10 days. There was also a history of altered sensorium and confusion for last 2 days. His family members were frightened and attended emergency and admitted him in Institute of Postgraduate Medical Education and Research Hospital (IPGME and R), Kolkata. On clinical examination, the patient was not maintaining eye contact, not responding to verbal commands. He was disoriented and withdrawing limbs to noxious stimuli. Bilateral normal sized pupils were showing a normal reaction to light. He had global aphasia and symmetrical brisk deep reflexes and bilateral extensor plantar reflexes. There were no abnormal involuntarymovements noticed and no signs suggestive of meningeal irritation. The fundoscopy was normal. His laboratory parameters showed hemoglobin: 14.8 g/dl, total leukocyte count: 7,600 cells/cumm (N59, L38, M01, E02), random blood sugar: 130 mg/dl, and normal serum electrolytes level. Dengue antibody (IgM + IgG)-nonreactive (NR), chikungunya antibody (IgM + IgG)-NR, malaria dual antigen-NR, and Widal test-NR. Cerebrospinal fluid (CSF) examination revealed 16 cells/cumm, protein: 142 mg/dl, glucose: 76 mg/dl, and CSF for polymerase chain reaction (PCR) was confirmed a positive infection with herpes simplex virus Type 1 (HSV1). On the other hand, cranial magnetic resonance imaging showing hyperintensities in the bilateral temporal lobes, (a) T2-weighted, and (b) T1-flair weighted images [Figure 1].
|Figure 1: Cranial magnetic resonance imaging showing hyperintensities in the bilateral temporal lobes, (a) T2-weighted and (b) T1-flair weighted images|
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Thus, confirming the diagnosis of HSE.
Thereafter, he was managed with supportive care and intravenous infusion of acyclovir 10 mg/kg body weight in every 8 h interval for 10 days. Gradually he recovered completely from encephalitis and discharged without any residual symptoms.
After few weeks of discharge, he developed some kind of behavioral abnormality and attended psychiatric department of IPGME and R, Kolkata Hospital. He started obscene behavior by making indecent gestures to the girls. He used to speak abusive languages to the girls and pull their clothes. There were few episodes of touching other females suddenly at inappropriate sites. Side by side, he developed habits of keeping his hands in his pant and tried to squeeze his penis excessively. There were also few episodes of masturbatory activities in front of his family members.
Even, he was exhibiting diminished fear responses or reacting with unusually low aggression to others in spite of beaten by them. Rather he showed his placid emotional attitude to them.
His appetite was increased significantly. He was frequently demanding for foods and ate very fast after mixing all foodstuffs at a time. He took food from other's plates and ate them.
Again, he was unable to recognize his household familiar objects. Thus, to identify them, he performed some repetitive striking movement by his fingers and use to stroke everything, which came close to his visual field. He was treated in the psychiatry department of IPGME and R Hospital, Kolkata as an inpatient. An electroencephalographic was done, and it was suggesting periodic discharge of slow waves in the frontotemporal region. Tablet carbamazepine 200 mg 8 hourly was added. Addition to that tablet risperidone 2 mg once daily was also added to reduce behavioral problems. In the next 1-month, his behavioral problems (particularly hypersexuality, hyperorality, and hypermetamorphosis) were improved mild to a moderate level, and he was discharged with the advice of regular follow-up.
| Discussion|| |
Herpes simplex encephalitis is a known acute or subacute illness that causes both general and focal signs of cerebral dysfunction. The retrograde transmission of the virus to the brain along the nerve axon from the peripheral dormant nerve ganglion, following HSV1 reactivation, is the known mode of CNS infection. The exact cause of the reactivation is not till clear to all of us; although the immune system dysfunction caused by stress is clearly play a role in animal models of the disease. HSV1 is the most common cause of adult HSE. Overall, about 90% of HSE are caused by HSV1. On the other hand, the predominant pathogen of neonatal HSE is HSV2, which is usually acquired by maternal shedding during delivery.
Mortality and morbidity of this fatal viral encephalitis particularly in untreated cases are very severe (70%). Older age, delayed initiation of therapy, prolonged duration of illness, and comatose states are the important poor prognostic factors.
The diagnosis of this HSE can be confirmed by means of PCR or brain biopsy. PCR assay of detection of viral DNA/anti-herpes antibody in CSF for HSV1 and HSV2: Essentially replaced brain biopsy as the criterion standard for diagnosis.,
To prevent relapse and dreadful consequences, a longer duration (14–21 days) and higher dosage of acyclovir may be given although the standard dose of HSE is 10 mg/kg body weight iv acyclovir infusion every 8 hourly for continuous 10 days. Acyclovir is the key drug of therapy for HSE, but this important antivirus is most useful if it is introduced to the patient before the comatose stage.
Treatment of behavioral changes of KBS is truly a big challenge. Patients need careful monitoring to prevent bulimia and consequent obesity. Strict monitoring also prevents uninhibited and inappropriate sexual activity, which has been reported as leading cause of the criminal conviction.
It has been established that all these behavioral changes of KBS may be controlled in mild to moderate level with the help of carbamazepine.
Other medications such as haloperidol and anticholinergics may also be useful in treating behavioral abnormalities associated with KBS.
In our case, delayed onset of treatment, disoriented stage, and longer duration of illness are the real important bad prognostic determinants.
| References|| |
Klüver H, Bucy PC. Preliminary analysis of functions of the temporal lobes in monkeys 1939. J Neuropsychiatry Clin Neurosci 1997;9:606-20.
Pradhan S, Singh MN, Pandey N. Kluver Bucy syndrome in young children. Clin Neurol Neurosurg 1998;100:254-8.
Auvichayapat N, Auvichayapat P, Watanatorn J, Thamaroj J, Jitpimolmard S. Kluver-Bucy syndrome after mycoplasmal bronchitis. Epilepsy Behav 2006;8:320-2.
Terzian H, Ore GD. Syndrome of Klüver and Bucy; reproduced in man by bilateral removal of the temporal lobes. Neurology 1955;5:373-80.
Shraberg D, Weisberg L. The Klüver-Bucy syndrome in man. J Nerv Ment Dis 1978;166:130-4.
Kluver H, Bucy PC. Preliminary analysis of the function of temporal lobe in monkeys. Arch Neurol Psychiatry 1939;42:979-1000.
Whitley RJ. Herpes simplex encephalitis: Adolescents and adults. Antiviral Res 2006;71:141-8.
Lakeman FD, Whitley RJ. Diagnosis of herpes simplex encephalitis: Application of polymerase chain reaction to cerebrospinal fluid from brain-biopsied patients and correlation with disease. National Institute of Allergy and Infectious Diseases Collaborative Antiviral Study Group. J Infect Dis 1995;171:857-63.
Cinque P, Cleator GM, Weber T, Monteyne P, Sindic CJ, van Loon AM. The role of laboratory investigation in the diagnosis and management of patients with suspected herpes simplex encephalitis: A consensus report. The EU concerted action on virus meningitis and encephalitis. J Neurol Neurosurg Psychiatry 1996;61:339-45.
Davis LE. Viral diseases of the nervous system. In: Asbury AK, McKhan G, editors. Diseases of the Nervous System. 3rd
ed. Cambridge: Cambridge University Press; 2002. p. 1669-70.
Devinsky J, Sacks O, Devinsky O. Kluver-Bucy syndrome, hypersexuality, and the law. Neurocase 2010;16:140-5.
Stewart JT. Carbamazepine treatment of a patient with Klüver-Bucy syndrome. J Clin Psychiatry 1985;46:496-7.
Jha S, Patel R. Kluver-Bucy syndrome – An experience with six cases. Neurol India 2004;52:369-71.