Does someone close to you have these changes? Let's learn about Pick's Disease

We often feel surprised when we see unexpected changes in the behavior and speech of some of our loved ones, don't we? Today we are going to talk about a medical condition that may be behind such changes. Although this is a somewhat complex topic, let's try to understand it simply.

What is Pick's Disease? How is it different from Niemann-Pick disease?

Simply put, Pick's Disease is a condition that affects the brain, gradually killing cells. It is a type of dementia called frontotemporal dementia (FTD) . It is most often seen in people under the age of 65. In the past, frontotemporal dementia itself was also called Pick's disease. But now, doctors only use this name in cases where certain criteria are met.

Now you might be thinking, "Oh... is Pick's disease the same as Niemann-Pick disease?" No, they are two completely different diseases. Even though the "Pick" part of the name is coincidentally the same, the two diseases are not related.

• Pick's Disease: It's named after Arnold Pick, a Czech neurologist and psychiatrist who first identified the disease in 1892. It's a type of frontotemporal dementia (FTD), meaning it only affects your brain.
• Niemann-Pick Disease: Named after two German doctors, Albert Niemann and Ludwig Pick, this disease is characterized by the accumulation of lipids in the body and a disorder in the way they are controlled. When lipids accumulate, they can affect many organs, including the brain, liver, spleen, bone marrow, and lungs.

Do you understand the difference? One is something limited to the brain, and the other is something that affects different parts of the body.

Who gets this disease? How common is it?

Pick's disease is usually diagnosed at a younger age than other conditions such as dementia. It is most often diagnosed in people in their 50s and 60s. However, it can sometimes occur in people as young as 20 or as old as 80.

There is some evidence that the disease can run in families. Researchers have identified at least three gene mutations that are associated with it. However, in most cases, the disease is sporadic. This means that someone can develop it without any family history.

It is difficult to say exactly how common this disease is. Experts estimate that there are between 15 and 22 cases per 100,000 people. However, there are doubts about the accuracy of these statistics, because the disease is very difficult to diagnose during life, and sometimes even after death, it is a challenge to diagnose. So the actual number of patients may be higher.

How does Pick's Disease affect the body?

Pick's disease is a neurodegenerative disease, a type of dementia called frontotemporal dementia (FTD). Simply put, the nerve cells (neurons) in the brain gradually stop working. The affected parts of the brain begin to shrink (atrophy). Then, you lose the abilities that those parts used to control. Although it has some similarities to Alzheimer's disease, Pick's disease usually starts at a younger age, and there are several important differences.

Because Pick's Disease affects only certain parts of the brain, the main symptoms are changes in behavior or language skills . Often, people with Pick's Disease are unable to recognize that they have a problem or condition. This is because their brains are unable to process information about their condition. This is called "lack of insight."

What are the symptoms of Pick's Disease?

Pick's Disease occurs in two main forms: one is behavioral variant frontotemporal dementia (bvFTD) , and the other is primary progressive aphasia (PPA), which affects the language-related parts of the brain.

Behavioral variant frontotemporal dementia (bvFTD)

The symptoms of this bvFTD condition can be divided into six main categories:

• Loss of inhibitions: "Inhibition" is the part of the brain that tells us "don't do this." When these parts are damaged, we can't stop ourselves from saying or doing inappropriate things.
• Lack of a "filter" for what is said: Saying what is on your mind, sometimes in a way that hurts others, is rude, or inappropriate. For example, suddenly insulting someone at a family gathering.
• Lack of respect for others: They may not respect the privacy of others, may touch them unwillingly, may try to behave in a sexually inappropriate manner, and may suddenly become angry or agitated.
• Reckless actions and behaviors: This can include spending money irresponsibly, and perhaps even shoplifting.

• Apathy: This may look like depression, but it's a little different.
• Loss of interest in anything: Loss of interest in things you used to enjoy (hobbies, spending time with friends).
• Social withdrawal: Avoiding being with friends and family.
• Decreased self-care: Loss of interest in bathing, changing clothes, and staying tidy.

• Loss of empathy: This is also called "emotional blunting." It becomes difficult to understand the feelings of others. You don't show sympathy for others' sorrows or joys like you used to.

• Compulsive behaviors: People with this disorder exhibit behavior patterns that are markedly different from others.
• Repetitive movements: Repeatedly making small movements, such as clapping hands, stomping feet, and walking back and forth.
• Complex or ritualistic behaviors: watching the same movies over and over again, reading the same books, collecting the same types of items. This includes hoarding.
• Repetition: Repeating the same sounds, words, and sentences over and over again.

• Changes in diet or mouth-centered behaviors: People with this disease may experience a condition called "hyperorality," which is an unusual interest in things related to the mouth.
• Changes in eating habits: People tend to overindulge in their favorite foods, especially snacks, sweets, and alcohol. This can lead to weight gain.
• Compulsive oral behaviors: Compulsive smoking, binge eating, etc. Also, oral exploration. While this is normal for young children to do as they explore the world, it is unusual for adults to do it.
• Pica: This is the compulsive eating of inedible, non-nutritious substances (coins, dirt, pebbles).

• Loss of executive function but other abilities intact: "Executive function" refers to the ability to plan the day, solve problems, and get things done. Although these abilities are impaired in people with this disease, other abilities, such as memory and visual processing, are not affected as much in the early stages. This is a key feature that distinguishes frontotemporal dementia from Alzheimer's disease.

Primary Progressive Aphasia (PPA)

PPA can also be caused by Pick's disease. This causes a gradual loss of language skills, such as the ability to speak and understand what others are saying. Although there are three main subtypes of PPA, two main types that can be associated with Pick's disease are:

• Nonfluent variant PPA (nfvPPA): In this type, it is difficult to match words and use correct grammar. Although single words and simple sentences can be understood, complex sentences have difficulty understanding.
• Semantic variant PPA (svPPA): In this type, it is difficult to choose the right word or understand the meaning of words. Some things said may not make sense, and it is also difficult to understand what others say. There may be problems reading and writing, but they can repeat what others say in the same way.

Imagine, your father suddenly becomes different from the person he used to be, scolds everyone, spends money recklessly, or hums the same song all day. Or, when you talk to him, his words get tangled up, and he feels like he doesn't understand what you're saying. These are examples of the things we've been talking about.

What causes this condition? Is it contagious?

Pick's Disease is a type of frontotemporal dementia (FTD) that has a very specific cause. Our brains and nerve cells contain a special protein called tau . These proteins need to have a specific shape, or they won't work properly.

When tau proteins malfunction, tangle together, and accumulate inside neurons, those cells are damaged and destroyed. The damaged neurons are called "Pick cells." They become swollen and balloon-shaped. The clumps of tau proteins that are tangled inside the cells are called "Pick bodies." These can be seen under a microscope. Importantly, these Pick cells and Pick bodies are not seen in any other disease.

Why these tau proteins malfunction is still a mystery. Although researchers have linked some genetic mutations to this, in most cases the disease is not hereditary.

This disease is not contagious. It is not transmitted from one person to another. Although there is a possibility of it being passed down through generations, it does not happen in most cases.

How is Pick's Disease diagnosed? What tests are done?

Doctors can diagnose frontotemporal dementia (FTD) through physical and neurological examinations, diagnostic and imaging tests, and the results can determine whether it is behavioral FTD or a subtype of PPA.

However, the only way to know for sure whether someone's frontotemporal dementia (FTD) is caused by Pick's disease is to look for Pick bodies and Pick cells in their brain tissue. This can only be seen by examining a sample of brain tissue under a microscope. This means that Pick's disease can only be definitively diagnosed after someone has died, at an autopsy.

Tests such as these can be done to diagnose FTD:

• Blood tests: These look for chemical particles called "biomarkers" that can help diagnose certain diseases.
• Cerebrospinal fluid tests: This is done by taking fluid from the spine (spinal tap / lumbar puncture).
• CT scan (Computerized tomography - CT scan)
• EEG (Electroencephalogram)
• Genetic testing
• MRI scan (Magnetic resonance imaging - MRI)
• PET scan (Positron emission tomography - PET scan)

Is there a treatment for Pick's Disease? Can it be cured?

Unfortunately, there is no cure, treatment, or prevention for Pick's disease. Doctors can prescribe medications to help manage symptoms such as depression, apathy, and aggressive behavior. However, since these treatments vary from person to person, your doctor is the best person to talk to.

Can the risk of developing this disease be reduced?

Pick's Disease occurs in unpredictable, unknown ways. Therefore, there is currently no way to prevent it or reduce the risk of developing it.

What kind of future can someone with this disease expect?

In all forms of FTD, including Pick's Disease, the brain gradually deteriorates. As it does, the affected parts of the brain lose their ability to function. In Pick's Disease, this usually affects the parts of the brain that control behavior or the parts of the brain that coordinate speech and the ability to understand what others are saying.

You may also lose insight into your condition. That is, you may lose the ability to understand the symptoms and what is happening to you because of this disease.

People with frontotemporal dementia (FTD) gradually lose the ability to live independently. As the disease progresses, they may require 24-hour care from loved ones or trained professionals. This may even require long-term care.

People with FTD may experience difficulty swallowing (dysphagia) , which can make it difficult to eat, drink, and speak. This increases the risk of developing conditions such as pneumonia or respiratory failure.

All types of FTD, including Pick's disease, are lifelong conditions. The disease is severe, gradually destroying major parts of the brain. Complications can be severe and even fatal. Because life expectancy varies from person to person, it is difficult to say exactly how the disease will affect you and how long it will last. Your doctor (or the doctor treating your loved one) can tell you more about this.

How can you exercise your will if you can't make decisions for yourself?

If you are diagnosed with frontotemporal dementia (FTD) in its early stages, it is important to talk to your doctor, your family or loved ones, and those you trust who can make important decisions for you as soon as possible. This conversation may be difficult, but talking about these things early rather than later will help your loved ones know what your wishes are if you are unable to talk about your affairs or make decisions.

In addition to that conversation, it's also important to have your wishes and decisions in writing. If you are unable to care for yourself, or if you are unable to make decisions about your care or welfare, consider preparing legal documents. You can get help from a lawyer to prepare these documents, but you can also prepare some of them yourself (depending on the law in your area, they may require the approval of a notary or other official).

What do you do if someone close to you shows these symptoms?

People with FTD often lack insight into their symptoms or condition. Because they don't see that they have a problem, they don't believe they need medical attention. This lack of understanding can be frustrating and frightening for the person with the symptoms and those closest to them.

If someone close to you is showing symptoms of FTD or a similar condition, you can try to help in these ways:

• Ask how you can help: Even though people with FTD are showing symptoms, they may not realize that these are signs of a serious brain problem. By listening to them, offering your support, they can feel connected to people they trust, and may even get the encouragement they need to see a doctor.
• Encourage them to seek help: While frontotemporal dementia (FTD) is a condition that has no cure or treatment, there are ways to treat and manage some of the symptoms. These efforts can go a long way toward improving the quality of life for someone with the condition. Seeing a doctor can help someone with FTD get a diagnosis. Specialized care can provide relief from some of the uncomfortable symptoms for people with the condition and their families.
• Stay calm and don't take things personally: People with FTD often have no control over what they say or do. This may seem like they are intentionally trying to annoy, embarrass, or hurt others, but it is actually a medical problem.
• Don't be afraid to ask for help: Caring for someone with FTD can be very difficult, especially as the condition worsens over time. Don't be afraid to ask for help or resources. Many public and private agencies provide support systems and services, such as adult daycare, respite care, and skilled home nursing care.
• Long-term care may be the best option: For many, caring for a loved one with FTD can feel like a full-time job. Not everyone can devote that much time or effort to caring for a loved one. Therefore, it is important to consider whether your loved one needs care in a long-term care facility with skilled nursing facilities. While this may be a difficult decision to make, such care may be the best way to ensure that they are safe, comfortable, and with qualified, trained caregivers.

Finally, take-home message

Pick's Disease is a condition called frontotemporal dementia (FTD), which is a type of dementia that gradually damages the brain at a younger age than other age-related brain diseases. FTD also affects the parts of the brain that help a person understand the appropriateness and inappropriateness of their behavior. This can cause frustration, misunderstandings, and other serious disruptions to the lives of everyone involved. While some symptoms can be treated, there is no cure that can reverse the disease itself, and people with the condition often require constant, 24-hour care.

If you or someone close to you is experiencing these symptoms, the best thing to do is to seek medical advice. Remember, you are not alone, and there are places to get help.

` Pick's Disease, Frontotemporal Dementia, Dementia, Brain Disease, Neurodegenerative, Tau Protein, Behavior Changes