Pick's Disease

What is Pick’s disease?

Pick’s disease is a specific type of frontotemporal dementia (FTD), a degenerative brain disease that happens most commonly in people under age 65. In years past, Pick’s disease was referred to as frontotemporal dementia itself. However, today, experts only use Pick’s name when the condition meets certain criteria.

What is the difference between Pick’s disease and Niemann-Pick disease?

These two diseases have no connection other than the coincidence of the name “Pick.”

Pick’s disease gets its name from Arnold Pick, the Czech neurologist and psychiatrist who first identified and described the condition in 1892. It’s a type of frontotemporal dementia, meaning it only affects your brain.

Niemann-Pick disease gets its name from two German physicians, Albert Niemann and Ludwig Pick. This disease disrupts how your body stores and processes lipids (the chemical molecules that make up fats, oils, waxes, fatty acids, etc.). As lipids build up in your body, they affect your brain, liver, spleen, bone marrow, lungs, etc.

Who does it affect?

Pick’s disease affects people at younger ages than dementia or similar conditions. People diagnosed with Pick’s disease are most likely in their 50s or 60s. But there are cases of this condition in people as young as 20 or as old as 80.

There’s also some evidence that Pick’s disease can run in families. Researchers have found evidence that connects it with at least three specific gene mutations. However, most cases of Pick’s disease are “sporadic,” meaning the condition wasn’t inherited.

How common is Pick’s disease?

Experts estimate there are between 15 and 22 cases of Pick’s disease per 100,000 people. But experts also believe the accuracy of this number is in doubt and is likely higher than the available numbers suggest. That’s because Pick’s disease is impossible to diagnose while you’re alive and extremely difficult to diagnose even after death (more about this is available under the Diagnosis & Tests section below).

How does this condition affect my body?

Pick’s disease is a type of frontotemporal dementia, a neurodegenerative disease. That means the affected neurons (brain or nerve cells) gradually stop working. As brain cells in the affected areas fail, those areas atrophy (shrink or wither), and you lose the abilities those areas once controlled. It has some similarities to Alzheimer’s disease but usually happens earlier and has some key differences.

Pick’s disease tends to affect specific parts of your brain and not others, causing behavior or language ability changes. People with Pick’s disease often can’t recognize they have a problem or medical condition. That’s because their brains can’t process information related to the condition as it happens.

What are the symptoms of Pick’s disease?

There are two main ways that Pick’s disease happens. One way is that it causes the behavioral variant of frontotemporal dementia (bvFTD). The other is when it affects the language areas of your brain, causing a condition known as primary progressive aphasia.

Behavioral variant frontotemporal dementia

The symptoms of bvFTD fall under six categories:

• Loss of inhibitions.
• Apathy.
• Loss of empathy.
• Compulsive behaviors.
• Changes in diet or mouth-centered behaviors.
• Loss of executive function.

Loss of inhibitions

When your brain tells you not to do something, that’s known as “inhibition.” Damage to these areas means you lose inhibitions, meaning you can’t hold back from saying or doing things that aren’t appropriate. That usually happens in one of the following ways:

• Loss of a “filter” for what they say. When this happens, a person might say things that are out of character. The things they say are often hurtful, rude or offensive.
• Lack of respect for others. This can involve disregarding others’ need for personal space or touching people in an unwelcome way. It can also include inappropriate sexual touching or behaviors. Outbursts of anger or frustration are also possible.
• Impulsive actions and behaviors. Examples of this include irresponsible spending or even shoplifting.

Apathy

Healthcare providers often diagnose depression when a person has the symptom of apathy because the two look so alike. Apathy usually involves the following:

• Loss of motivation. This usually looks like a decreased interest in things you previously enjoyed, such as hobbies or relationships.
• Social isolation. Avoiding spending time with others, including friends, family and other loved ones.
• Decline in self-care. This can look like a lack of interest in bathing and grooming or otherwise neglecting your appearance.

Loss of empathy

Another name for this is “emotional blunting.” People who have this symptom seem to have trouble reading the emotions of others. They also might not react to your emotions or offer support when previously they would have.

Compulsive behaviors

People with Pick’s disease often behave in noticeably different ways from people without this condition. Sometimes, behavior changes are small and happen in very limited ways. For others, the changes might be more complicated, involving multiple steps or a strict routine. Some examples include:

• Repetitive motions. People with Pick’s disease often repeat small-scale movements, such as clapping their hands, tapping their feet, pacing, etc.
• Complex or ritual-like behaviors.Compulsively watching the same movies, reading the same books or collecting types of items. Hoarding items also falls under this category.
• Speech repetition.A person with Pick’s disease may vocalize or say the same sounds, words or phrases repeatedly.

Changes in diet or mouth-centered behaviors

People with Pick’s disease often show “hyperorality,” an unusual level of mouth-centered interest or behavior. This often involves the following:

• Changes in diet. People with Pick’s disease may become much more focused on eating or drinking things they like, especially snacks, sweets or alcohol. Weight gain is a common symptom that follows this.
• Mouth-related compulsive behaviors. Compulsive behaviors include smoking or binge eating. It also includes oral exploration (feeling items with your lips or putting them in your mouth). This behavior is normal in babies as they explore objects and the world around them, but is unusual behavior in adults.
• Pica. This condition is where a person compulsively eats non-food items with no nutritional value. Common items that people with pica eat include coins, dirt and pebbles.

Loss of executive function without losing other abilities

Executive function is your ability to plan out your day, solve problems and carry out tasks. It also contributes to your judgment and how you organize things. People with Pick’s disease have trouble with executive function, but other abilities aren’t heavily affected.

A key part of this criteria is that other abilities, especially memory and how your brain processes what you see, aren’t as affected. This is one of the key ways to tell frontotemporal dementia apart from Alzheimer’s disease.

Primary progressive aphasia

Pick’s disease can cause primary progressive aphasia (PPA). This condition involves a gradual loss of language skills, including your ability to speak, understand other people speaking and more.

There are three main subtypes of PPA, two of which can sometimes happen with Pick’s disease:

• Nonfluent variant (nfvPPA): This type of PPA means you have trouble forming words and using correct grammar. You can understand individual words and simple sentences, but might struggle to understand complicated sentences.
• Semantic variant primary progressive aphasia (svPPA): If you have this type of PPA, you have trouble choosing or understanding the right word. Some of what you say might not make sense, and you might have trouble understanding others. You also might have problems with reading or writing, but not with repeating back what others say to you.

What causes the condition?

Pick’s disease is a type of frontotemporal dementia with a very specific cause. All nerve and brain cells contain a specific protein, tau (named for a Greek alphabet letter). Proteins like tau need to have a specific shape, or they won’t work properly.

When tau proteins malfunction and tangle together, they build up inside neurons, damaging and destroying those cells. These destroyed neurons, known as “Pick cells,” have a swollen, balloon-like shape. Tangles of tau proteins inside cells, known as “Pick bodies,” are also visible under a microscope. Pick cells and Pick bodies don’t happen with any other condition.

Why the tau proteins malfunction is still a mystery. Researchers have linked Pick’s disease to certain genetic mutations, but most cases of the disease aren’t inherited.

Is it contagious?

Pick’s disease isn’t contagious, and you can’t pass it from person to person. While there’s some evidence people can inherit the disease or pass it on to their children, most cases of Pick’s disease aren’t inherited.

How is Pick’s disease diagnosed?

Healthcare providers can diagnose someone with frontotemporal dementia using physical and neurological exams and diagnostic and imaging tests. Depending on the results, they can diagnose a person with either behavioral FTD or one of the subtypes of primary progressive aphasia (PPA).

However, the only way to know if a person’s frontotemporal dementia happened because of Pick’s disease is to see if they have Pick bodies and Pick cells in their brain tissue. The only way to see those is by looking at brain tissue samples under a microscope, which means Pick’s disease is only diagnosable with an autopsy after a person has died.

What tests will be done to diagnose this condition?

Several tests are possible to diagnose FTD. These include the following:

• Blood tests (these look for “biomarkers,” which are chemical molecules that indicate certain types of diseases).
• Cerebrospinal fluid tests after a spinal tap (lumbar puncture).
• Computerized tomography (CT) scan.
• Electroencephalogram (EEG).
• Genetic testing.
• Magnetic resonance imaging (MRI).
• Positron emission tomography (PET) scan.

How is Pick’s disease treated, and is there a cure?

Currently, there isn’t a way to treat or cure Pick’s disease or slow the disease’s progress. Healthcare providers may use medication to treat symptoms such as depression, apathy or aggressive behavior. Your healthcare provider is the best person to explain the possible treatments because these will vary from case to case.

How can I reduce my risk?

Pick’s disease happens unpredictably and for unknown reasons. Because of that, there’s no way to prevent it or reduce your risk of developing it.

What can I expect if I have this condition?

All forms of FTD, including Pick’s disease, cause your brain to deteriorate gradually. As it does, you lose abilities related to the affected areas. With Pick’s disease, this usually affects the areas that help you control how you behave or the areas of your brain that coordinate your ability to speak and understand others when they talk.

It’s also likely that you’ll develop a lack of insight into your condition. That means you’ll lose the ability to understand your symptoms and what’s happening to you because of this condition.

People who have frontotemporal dementia lose abilities over time, including the abilities that allow them to live independently. As the condition progresses, people with this condition may need ’round-the-clock care from loved ones or trained professionals in a long-term care facility or setting.

People with FTD can develop problems swallowing (dysphagia), making eating, drinking and speaking difficult. This could increase the risk of developing conditions like pneumonia or respiratory failure.

How long does Pick’s disease last?

All types of frontotemporal dementia, including Pick’s disease, are life-long conditions.

What’s the outlook for this condition?

Pick’s disease has severe effects, gradually disrupting key areas of your brain. This disease often causes complications that are severe and sometimes fatal. The average life expectancy varies, so it’s often difficult to predict how this disease will affect you and the timeline for its progress. Your healthcare provider (or the provider caring for your loved one) can tell you more about what to expect.

How to ensure your wishes are followed when you can’t choose for yourself

If you have an early diagnosis of frontotemporal dementia of any kind, it’s important to talk to your healthcare provider, your family or loved ones, and to anyone you trust to make important decisions for you as soon as possible. These conversations might feel unpleasant or difficult, but having them sooner rather than later can ensure your loved ones know what you want if you can’t tell them or choose for yourself.

In addition to those conversations, you should put your wishes and decisions in writing. You should also consider preparing documents related to legal issues and what happens if you can’t take care of yourself or make decisions for your own care or well-being. You can consult an attorney for help preparing these documents, but many of them you can prepare on your own (you may need a notary or other official to endorse them, depending on the laws in your area).

How do I take care of myself?

People who have frontotemporal dementia will eventually need some kind of care arrangement because they’ll lose the ability to care for themselves. Your healthcare provider can tell you more about what to expect with this. They can also direct you to resources on care options and services that might help you or your loved one.

What can I do if a loved one shows signs of frontotemporal dementia or a similar condition?

People with FTD often lack insight, which means they can’t recognize their symptoms or condition. Because they can’t see the problem, they often don’t believe they need medical care or treatment. That lack of understanding can lead to frustration or fear for the person with the symptoms and their loved ones.

If you notice a loved one showing signs of FTD or a related condition, you can try helping them by doing the following:

• Ask how you can help. People with FTD may show symptoms without realizing that these are symptoms of a serious brain problem. Listening and offering your help can help them feel connected to people they trust, and it might offer the encouragement they need to see their healthcare provider.
• Encourage them to see someone who can help. Frontotemporal dementia is a condition that isn’t treatable or curable, but there are ways to treat and manage some of the symptoms. These efforts could improve the quality of life for a person with this condition. Seeing their healthcare provider can help a person with FTD get a diagnosis. Specialized care can offer some relief from some of the disabling symptoms for people with this condition, and for their families.
• Stay calm and don’t take things personally. People with FTD often can’t control what they do or say. While this might seem like they’re being deliberately difficult, embarrassing or hurtful, the reality is that this is a medical issue.
• Don’t be afraid to ask for assistance. It can be extremely difficult to care for someone with FTD, especially as the condition worsens over time. Don’t be afraid to ask for help or resources. Many government and private organizations offer support systems and services, such as adult daycare, respite and skilled home nursing care.
• Long-term care might be the best option. For many people, caring for a loved one with FTD can be like a full-time job. Not everyone can devote that kind of time or effort to caring for a loved one. So, it’s important to consider whether or not your loved one needs long-term care in a skilled nursing facility. While it’s a hard choice to consider, that kind of care might be the best way to ensure they’re safe, comfortable and have qualified, trained caregivers nearby.

A note from Cleveland Clinic

Pick’s disease is a type of frontotemporal dementia (FTD), a condition that causes progressive damage and disruptions in a person’s brain at younger ages than expected for age-related brain disorders. FTD also affects parts of the brain that allow you to understand the difference between appropriate and inappropriate behavior. That can lead to frustration, misunderstandings and other severe disruptions in the lives of all involved. Some symptoms may be treatable, but there’s no treatment that can reverse the effects of the disease itself, and people with this condition often eventually need constant ’round-the-clock care.