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Kluver-Bucy syndrome

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Kluver-Bucy syndrome

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USMLE® Step 1 style questions USMLE

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USMLE® Step 2 style questions USMLE

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A 25-year-old right-handed man is brought to the clinic by his parent for evaluation of "abnormal behavior.” The patient’s parent reports that the patient has been eating uncontrollably. The patient has also demonstrated inappropriate sexual behavior towards family members. Also of concern to the patient’s parent is that the patient seemed indifferent to the news that the patient’s other parent had passed away a few weeks ago. The patient’s parent denies any recent head trauma sustained by the patient but does state that the patient was hospitalized two months ago for a “brain infection.” A follow-up MRI is ordered. Which of the following parts of the brain is most likely to demonstrate abnormal findings?  

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Rishi Desai, MD, MPH,Yifan Xiao, MD

Klüver-Bucy syndrome is neurobehavioral condition first observed in the 1930s by Heinrich Klüver and neurosurgeon Paul Bucy.

They discovered that monkeys that had bilateral temporal lobe lobectomies developed dramatic behavior changes in their memory, as well as their social and sexual behaviors.

In retrospect, it’s not surprising that having a significant portion of the brain removed caused behavior changes in monkeys - the biggest one was probably anger at Klüver and Bucy for putting them through that!

The temporal lobe is one of the four major lobes of the brain.

It has a variety of functions which are involved in sensory processing.

The ventral, or anterior, part of the temporal lobe helps process visual information, such as the recognition of objects and faces.

Whereas, the medial, or middle portion, of the temporal lobe, along with the hippocampus, is responsible for the formation of new memories.

The temporal lobe also houses the primary auditory cortex, which is needed for speech comprehension.

Other important structures found within the temporal lobe include the olfactory cortex, which processes the sensation of smell, and the amygdala, which processes emotions.

The amygdala also plays a role in the reward center of the brain which motivates and reinforces behaviors that elicit positive feelings.

In Klüver-Bucy syndrome there’s a bilateral lesion of the temporal lobe.

The most common cause of such a brain lesion is herpes simplex encephalitis - a viral infection that particularly affects the temporal lobes.

Other causes of Klüver-Bucy syndrome include trauma, stroke, Alzheimer's disease, and Niemann Pick disease, which is a rare metabolic condition that affects various organs of the body, including the brain.

The damage affects the amygdala which is a part of the brain that regulates emotions and reinforces behaviors related to food and sex.

Summary

Kluver-Bucy syndrome is a rare neurological disorder that is caused by damage to the temporal lobes of the brain. The syndrome is characterized by a number of symptoms, including hypersexuality, excessive eating, and memory impairment. Some people with Kluver-Bucy syndrome also experience changes in their personality and become emotionally unstable.

Sources

  1. "Robbins Basic Pathology" Elsevier (2017)
  2. "Harrison's Principles of Internal Medicine, Twentieth Edition (Vol.1 & Vol.2)" McGraw-Hill Education / Medical (2018)
  3. "Pathophysiology of Disease: An Introduction to Clinical Medicine 8E" McGraw-Hill Education / Medical (2018)
  4. "CURRENT Medical Diagnosis and Treatment 2020" McGraw-Hill Education / Medical (2019)
  5. "Complete Klüver-Bucy Syndrome in Man" Cortex (1975)
  6. "Klüver-Bucy Syndrome Following Traumatic Brain Injury: A Systematic Synthesis and Review of Pharmacological Treatment From Cases in Adolescents and Adults" The Journal of Neuropsychiatry and Clinical Neurosciences (2019)