Klüver-Bucy Syndrome

What is Klüver-Bucy syndrome?

Klüver-Bucy syndrome (KBS) is an extremely rare brain disorder that can cause memory loss and behavioral problems. Some people with this disease try to eat nonfood items. Others have an unusually extreme sex drive. In severe cases, symptoms include seizures and dementia.

Damage to your temporal lobes, one of the major sections of your brain, causes Klüver-Bucy syndrome. This part of your brain helps you:

• Form new memories.
• Guide behavior related to food and sex.
• Process sensory information and emotions.

Traumatic brain injury can trigger Klüver-Bucy syndrome. Other causes include neurological conditions, including herpes simplex encephalitis. Klüver-Bucy syndrome affects people of all ages.

This condition doesn’t have a cure. But treatment can help reduce or reverse symptoms. Early diagnosis is important.

Are there other names for Klüver-Bucy syndrome?

Other names for Klüver-Bucy syndrome include:

• Bilateral temporal lobe disorder.
• Post-encephalitic Klüver-Bucy syndrome.
• Post-traumatic Klüver-Bucy syndrome.

Who does Klüver-Bucy syndrome affect?

Klüver-Bucy syndrome is a rare disease that affects adults. Less often, children develop the condition.

What are the risk factors for Klüver-Bucy syndrome?

Klüver-Bucy syndrome happens due to traumatic head injury or other neurological problems. You’re more likely to develop symptoms of Klüver-Bucy syndrome if you have:

• Alzheimer’s disease.
• Cerebrovascular disease.
• Epilepsy.
• Head injury or trauma.
• Herpes simplex encephalitis.
• Pick’s disease (frontotemporal dementia).
• Stroke.

Are there conditions similar to Klüver-Bucy syndrome?

KBS has symptoms that are similar to:

• Alzheimer’s disease.
• Kleine-Levin syndrome.
• Korsakoff syndrome.
• Pick’s disease.
• Prader-Willi syndrome.

Because the symptoms resemble other conditions, it’s important to get an accurate diagnosis. The right diagnosis helps you get the proper care for your needs.

What causes Klüver-Bucy syndrome?

Klüver-Bucy syndrome happens when your brain’s temporal lobes, especially your hippocampus and amygdala, are injured. Brain damage may be due to traumatic head injury, infection or disease.

Adults are most likely to develop KBS due to stroke or head injury. Children usually develop Klüver-Bucy syndrome due to herpes simplex encephalitis (brain swelling or inflammation).

What are the symptoms of Klüver-Bucy syndrome?

You may experience a range of symptoms with Klüver-Bucy syndrome, depending on the location and severity of your brain injury. Symptoms typically affect memory, as well as social and sexual behavior. Symptoms may be temporary or permanent.

Many common symptoms relate to eating or your mouth, like:

• Binge eating and purging (bulimia nervosa).
• Compulsive eating (hyperphagia), smoking or drinking alcohol.
• Eating inedible objects (pica).
• Putting objects in your mouth or inappropriate licking (hyperorality).

Other common symptoms include:

• Dementia.
• Hypersexuality, which is an extreme sex drive marked by inappropriate sexual behavior. (In children, this may present as frequent holding or rubbing of their genitalia or thrusting their pelvis back and forth.)
• Inability to recognize familiar people or objects by sight (visual agnosia).
• Memory loss (amnesia).
• Reduced fear or aggression response (docility).
• Seizures.

How is Klüver-Bucy syndrome diagnosed?

You may notice unusual symptoms in yourself or your child. Or your healthcare provider may notice symptoms during a checkup. If you observe any signs or symptoms of Klüver-Bucy syndrome, see your healthcare provider for a full exam and diagnosis.

Healthcare providers diagnose Klüver-Bucy syndrome using a physical exam and other tests. These may include blood tests and imaging studies such as:

• CT scan.
• Electroencephalogram (EEG).
• MRI.

Early detection is essential for effective treatment. In some cases, Klüver-Bucy syndrome may be mistaken for autism. Getting the right diagnosis ensures the best treatment plan for you or your child.

Is Klüver-Bucy syndrome curable?

Klüver-Bucy syndrome isn’t curable. But your healthcare provider can discuss treatment options with you to tailor a care plan for your needs. In some cases, symptoms may resolve over time.

How is Klüver-Bucy syndrome treated?

Treatment for Klüver-Bucy syndrome depends on your symptoms and underlying condition. It may include:

• Antidepressants, mood stabilizers or antipsychotic drugs to manage behavioral issues.
• Antiseizure medications to manage seizures.
• Antivirals to treat infection.
• Carbamazepine or leuprolide to treat sexual behavior problems.
• Occupational therapy to help with managing daily activities.

How can I prevent Klüver-Bucy syndrome?

There’s no known way to prevent KBS. Getting an accurate diagnosis as early as possible helps you get the care you need in the early stages of the disease.

What’s the prognosis for someone with Klüver-Bucy syndrome?

The outlook for Klüver-Bucy syndrome depends on the underlying cause and severity of symptoms. For some people, medication helps improve symptoms. Others experience long-term symptoms that may or may not improve over time.

When should I seek care for Klüver-Bucy syndrome?

It’s important to seek timely diagnosis for Klüver-Bucy syndrome. If you notice any symptoms of KBS, see your healthcare provider. Left untreated, Klüver-Bucy syndrome may result in severe symptoms and complications.

How do I take care of myself?

A diagnosis of Klüver-Bucy syndrome can be challenging, especially because it’s such a rare disease. It occurs due to an underlying condition and may have symptoms similar to other illnesses.

Follow your healthcare provider’s recommendations to manage complications and treat symptoms. They can work with you to develop a personalized care plan.

A note from Cleveland Clinic

It’s natural to feel troubled by a diagnosis of Klüver-Bucy syndrome. It’s an extremely rare condition that can lead to serious health problems. Be sure to tell your healthcare provider about your symptoms as soon as possible, so you can receive the necessary care. Your healthcare provider can recommend treatment options to address your needs and goals.