This article will provide the reader with a brief introduction to Pick’s disease. The reader will learn about the causes, symptoms, recommended treatments, and what the family can do to help a loved one with a diagnosis of Pick’s. Suggestions will be provided for how to prepare for your medical appointments, questions to ask your physician and what your physician may expect from you.
Pick’s Disease is characterized by deterioration of the frontotemporal lobe. The term “Pick’s Disease” is used to describe a specific condition where tau proteins (Pick bodies) collect in the brain. In the late 19th century, Frontotemporal dementia was initially referred to as Pick’s Disease. As more research into Frontotemporal diseases has developed, Pick’s Disease has come to be classified as a type of FTD where abnormal aggregates and collections of the protein Tau are present in someone’s brain.
Pick’s disease is a rare and irreversible form of dementia. Alzheimer’s and other types of dementia affect the functioning of the brain, and individuals with Pick’s disease have progressive problems with language, behavior, thinking, judgment and memory. In addition, individuals with Pick’s disease may experience noticeable personality changes.
Pick’s disease affects the frontotemporal lobe of the brain which controls important functions including planning, judgment, emotions, verbal communication, behavior, inhibition and some forms of movement. The temporal lobe is located on both side of the frontal lobe. The temporal lobe is involved in our knowledge and memory of objects, people, words and faces. The temporal lobe also plays a role in language and emotions.
Pick’s disease is caused by an abnormal collection of tau proteins found in all of your nerve cells. When tau proteins build up in the brain, they cause the cells in the brain to degenerate, and this leads to the brain shrinking. Tau proteins normally occur in the brain and are needed for healthy function. In Pick’s disease, these proteins build up, clump together and become misshapen. The buildup of these proteins interferes with normal functioning of the brain.
There is no known cause for the buildup of tau proteins that cause Pick’s Disease. The disease has been reported in people as young as 20 years old. More commonly, the symptoms begin in people between the ages of 40 and 50. It is also the second most common form of dementia in people under 65 (after early-onset Alzheimer’s).
People with Pick’s disease get progressively worse over time. Many of the symptoms make social interaction difficult. The person may have behavioral changes that make it hard to perform in a socially acceptable manner. Tissues in parts of the brain shrink over time. Early personality changes can help doctors distinguish Pick’s from other forms of dementia. People with Pick’s often behave the wrong way in social settings. The changes in behavior get worse and are often among the most disturbing symptoms of the disease.
Individuals with Pick’s disease may experience abrupt behavioral and emotional symptoms such as:
- Mood swings
- Compulsive or inappropriate behavior
- Depression-like symptoms such as disinterest in daily activities
- Withdrawal from social interaction
- Difficulty keeping a job
- Poor social skills
- Poor personal hygiene
- Repetitive behavior
Individuals may experience changes such as:
- Reduced reading or writing skills
- Echoing, or repeating what’s been said to patient
- Inability to speak, difficulty speaking, or trouble understanding speech
- Diminishing vocabulary
- Accelerated memory loss
- Physical weakness
The rate of progress for symptoms and the length of the disease can vary from one person to the next. In general, the symptoms that are seen in the mild stage get more pronounced and disabling over a 10- year period of time.
In the first few years, the individual will exhibit noticeable mood and behavioral changes. They may also exhibit lack of inhibition, apathy, loss of sympathy/empathy for others, and possible overeating. In addition, the person may have problems with planning and organization. The person may experience social withdrawal and lose interest in hobbies and interaction with friends and family. People with Pick’s may also behave inappropriately with strangers, act impulsively, and even break laws. At this stage, an MRI may show mild atrophy in the frontal lobes.
The symptoms in mild stages get more profound over the next few years. The person may exhibit compulsive behaviors such as hoarding, compulsive cleaning, or repetitive movements (like stomping on ants). He or she may experience weight problems or other health issues due to binge eating. The person will also likely experience more dementia- related problems such as forgetfulness, and problems with planning and attention.
At this stage, an MRI will show frontotemporal changes such as shrinking brain tissue that has expanded to larger areas of the frontal lobes, as well as tips of the temporal lobes and deeper brain structures involved in motor coordination, emotions and learning.
At this stage, the individual may experience profound behavioral symptoms (apathy, loss of empathy, disinhibition) in association with language difficulty and memory loss. The time from the first symptom to the end is usually eight to ten years, although this can vary from person to person.
The symptoms of Pick’s disease usually progress over eight to ten years. Individuals’ family and friends can be instrumental in helping them live with dignity and respect. While the symptoms the person is experiencing may be challenging, always remember to be loving and as patient as possible.
Here are some suggestions for caregivers:
- Have your loved one follow a heart-healthy diet
- Stay active with friends and interests
- Keep your loved one active in regular daily routines
- Make sure that your loved one always has identification with contact information and a notice that he/she has a neurological disorder
- Complete legal documents while your loved one can make informed choices (Power of Attorney, Living Will, etc.)
- Plan transition from work if he or she is still working
- Make sure to apply for Social Security Disability and any Veterans benefits your loved one may be eligible for
- Share information with friends and family
- Keep a log or journal of significant changes in symptoms
- Keep copies of diagnostic evaluations and medications and keep paperwork organized for appointments with healthcare providers
- Find a support group so that you do not feel alone
People suffering from Pick’s disease may have difficulties with daily tasks that were once easy. The difficulties can be caused by lack of motivation, difficulties with planning, or language problems.
Here are some suggestions for those providing care for people with Pick’s disease:
- Keep the person as active as possible and focus on their abilities rather than their disabilities
- Avoid arguing with your loved one and do what is necessary to make things easy for him or her
- Take a break from caregiving on a periodic and regular basis if possible
- Consider offering the person a reward for desired behaviors
The following are useful tips of a practical nature that are relevant to activities of daily living (ADLs):
Bathing a Person with Pick’s Disease
If the person with Pick’s disease becomes difficult to bathe, try the following:
- A sponge bath instead of a shower
- Talk to him or her in a soothing voice while bathing, and talk through each step that is taken
- Let your loved one do as much as possible for themselves
- Bathe your loved one every couple of days instead of every day
- Don’t force him or her to do anything
Dressing a Person with Pick’s Disease
An individual with Pick’s disease may be able to dress themselves for a long time. It may take them longer than usual, but you should let them do as much as possible for themselves, even if it takes a while. Try the following:
- Find clothes with larger buttons
- Replace closures with Velcro
- Find shoes that don’t require laces
- Consider getting a baby monitor; it will allow the person more independence but also alert you to any problems
Grooming a Person with Pick’s Disease
If personal hygiene and grooming are not being attended to, look for ways to simplify the process. Try some of the following tips to make it easier:
- Keep the person’s hair in a simple easy to manage style
- Use an electric razor to avoid the risk of cuts
- Try oral swabs if brushing the teeth becomes difficult
- Consider cold creams or disposable wipes for cleaning the face
- Move slowly and explain what you are doing in a calm voice so that you don’t startle them
Driving a Person with Pick’s Disease
Driving can be a very touchy subject. People with Pick’s disease don’t easily get lost; they do however lose the ability to make sound judgments and process all of the different stimuli they are receiving while driving, and they may not be aware of this decreased ability. The following suggestions may help:
- A steering wheel lock or other safety device that prevents someone with the ignition keys from taking the car
- If the person is upset about losing their driving privileges, take them to the department of motor vehicles to pick up the necessary paperwork to reinstate their license
- Drive home and tell them that when the forms are completed, they can be taken back to the DMV. Rarely do the forms get completed, but the person will usually feel a little better
Household Chores for a Person with Pick’s Disease
Your loved one may lose interest in doing house chores because of their lack of motivation. He or she may also experience difficulties with coping because of problems with planning and completing several steps that are involved with a complex task. Find simpler tasks the person can complete safely such as:
- Folding laundry
- Setting the table
- Stirring cake mix
- Setting a timer
- Seasoning a dish
Reward the person with pleasurable activities or healthy treats after completing the chore.
Sleeping for a Person with Pick’s Disease
Sleep is important for both the individual and his or her caregivers. To ensure that everyone gets the rest that is needed, try the following:
- Provide daily exercise
- Do a quiet and calming activity before bedtime
- Keep a schedule of wake and sleep times
- Keep the person hydrated during the day but avoid giving drinks before bedtime to decrease the likelihood of accidents
- Avoid television watching during the day, to prevent napping
- Consider hiring someone to sleep over a couple of nights a week to relieve you of night time duty
Using the Telephone with a Person with Pick’s Disease
Phone use can be difficult for people with Pick’s disease because they may feel pressured to make purchases; they may be unable to take messages; they may make inappropriate calls. Try some of the following suggestions:
- Provide them with a script, so they don’t have to come up with the right words
- Contact the phone service provided to block incoming calls from telemarketers and sales people
- Lock the keypad on the cellphone to prevent outgoing calls
- Leave a contact number where messages can be left without difficulty
There is no single diagnostic test that the physician can use to diagnose the disease. The doctor will use the patient’s medical history, imaging tests, and other tools to develop a diagnosis. The doctor will also have the patient do the following:
- Take a complete medical history
- Have the person complete speech and writing tests
- Interview those close with the person to learn about behaviors
- Use magnetic resonance imaging (MRI), computed tomography (CT), or positron emission tomography (PET) scans to examine the person’s brain tissue.
The doctor may use blood tests to rule out other causes of the person’s symptoms. The imaging tests assist the doctor with viewing the shape of the brain and changes that may be occurring, i.e. conditions such as brain tumors or strokes or other traumas that can also cause symptoms of dementia.
There is no definitive test for Pick’s disease, which often leads to the physician testing for other disorders with similar symptoms in order to rule them out. The doctor finally provides the person and his or her family with a clinical diagnosis of Pick’s disease, keeping in mind that the only definitive diagnosis of Pick’s disease comes when the person dies and the brain tissue can be examined in an autopsy.
There are similarly no known treatments that specifically slow the progression of Pick’s disease, and all that the doctor can do is prescribe treatments to help ease some of the symptoms. The first thing that must be done is to set a goal with the family about which symptoms they would like to treat.
Here are medications typically used to alleviate some of the harsher symptoms of Pick’s disease:
Selective serotonin reuptake inhibitors have been found to be useful in treating behavioral symptoms like apathy, depression, anxiety, agitation, and obsessive compulsive behaviors. These medications include Sertraline and Trazodone.
This class of medications is used to treat aggressive and agitated behavior, and they should be used only when they are absolutely necessary because of the risk of life threatening complications in older adults with dementia (stroke, cardiac complications). Haldol is one of the most common medications used.
This class of medications is used for stabilizing cognitive symptoms including aphasia, apathy and complex thinking (planning, following directions etc.) The following medication are included in this class of medications: Donepezil, Rivastigmine, and Galantamine
It is a good idea to keep the information concerning your loved one’s condition organized. Preparing a list of questions will help make the most of the time with the physician. Here is a list of some basic questions:
- Are there other possible causes for my symptoms?
- What kind of tests do I need?
- What treatment do you recommend?
- I have other health conditions; how can they be managed together?
- Are there any brochures or other printed material that I can take home with me?
Your doctor is likely to ask you a number of questions that include:
- What symptoms are you having?
- When did your symptoms begin?
- How severe are your symptoms?
- Do you have a family history of any illnesses? (Find out which family member had which illness).
A Day in the Life
This is Jane’s story. In it she tells how she deals with the apathy she experiences in dealing with her diagnosis. “My apathy is boosted greatly with stress. At times, it would seem so much easier to just live within an apathetic state of mind simply because it takes no energy. Fighting apathy takes mental strength, encouragement, engagement, and the will to live well.”
“It is imperative that you allow loved ones and friends to engage you, for otherwise you would simply be setting yourself up for failure. Reaching out for such support is an accomplishment in and of itself. Keeping a calendar in a common area not only helps you with gearing up to activities, but it also clearly shows where you have large breaks of many hours, even perhaps several days, without activities. Set clear boundaries as to how much time you want to stay engaged and how much time you need quiet and rest. This is a fight for quality of life and, therefore, it is not simple but so worth the time and effort in the end. I am now at 10 years in post-diagnosis life and doing the very best I can. I’m beating the odds regarding longevity of life, as well as quality of life.”
- Alzheimer’s Association. (2017). Frontotemporal Dementia. Retrieved from http://www.alz.org/dementia/fronto-temporal-dementia-ftd-symptoms.asp
- National Organization for Rare Disorders. (2017). Frontotemporal Degeneration. Retrieved from https://rarediseases.org/rare-diseases/frontotemporal-degeneration/
- The Association of Frontotemporal Degeneration. (2017). Newly Diagnosed Pick’s Disease. Retrieved from http://www.theaftd.org/life-with-ftd/newly-diagnosed
- United States National Library of Medicine. (2017). Pick’s Disease. Retrieved from https://medlineplus.gov/ency/article/000744.htm