Pick Disease

Pick disease is a brain disorder that causes slowly worsening decline of mental abilities. It gradually damages brain cells and impairs their function. It disturbs cognitive processes, such as reasoning, problem solving, and memory. The disease often affects a person’s ability to use and understand spoken, written, and even signed language. It also affects personality, emotions, and social behavior. When the decline in mental abilities is severe enough to interfere with a person’s ability to carry out everyday activities, it is called dementia.

Pick disease is named after Arnold Pick, the doctor who first described the disease in 1892. It is often compared to Alzheimer disease. However, Pick disease is different from Alzheimer disease in several ways.

• First, the diseases affect different parts of the brain. Pick disease usually affects only the frontal and temporal lobes of the brain, the part from the forehead back to the ears. For this reason it is sometimes called “frontotemporal dementia.” Pick disease is only one of several types of frontotemporal dementia.
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• Second, the diseases damage the brain in different ways. The changes they cause in the brain are distinct. Both diseases cause severe shrinkage (atrophy) of brain tissue and death of nerve cells called neurons. In Pick disease, the neurons contain abnormal protein accumulations called Pick bodies. Neurons may swell as they stop functioning.
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• These differences translate to somewhat different symptoms for the two diseases. Memory loss, usually the first symptom in Alzheimer disease, may not occur in Pick disease until later in the disease. People with Pick disease may have early changes in mood, behavior, and use of language and speech (aphasia).
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• On average, Pick disease occurs at a somewhat younger age than Alzheimer disease. In Pick disease, the first symptoms typically appear in middle age, in people aged 40-60 years. However, it can occur in adults of any age.

Unfortunately, Pick disease is similar to Alzheimer disease in several ways.

• It is a progressive disease, meaning that the symptoms gradually worsen over time and do not get better.
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• The two diseases are equally devastating, causing gradual decline of mental functions and disability.
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• Neither disease is curable.

Much less is known about Pick disease than about Alzheimer disease. This is partly because Pick disease is a much less common disease. Also, Pick bodies and neuron swelling are difficult to detect in a living person, so Pick disease may go undiagnosed or be misdiagnosed. People with Pick disease are sometimes thought to have Alzheimer disease. This is changing as medical professionals learn more about Pick disease.

The specific cause of Pick disease is unknown. In a small number of cases, the disease is hereditary, meaning that it runs in the family. This is not true of most cases of the disease.

The frontal lobes of the brain are the source of our rational emotional responses and the way we act in response to the world around us. This region of the brain also controls our use of speech and how we express language in all forms. Because Pick disease mostly affects this region of the brain, it is these thinking functions that are most often abnormal in people with the disease.

The symptoms of Pick disease vary widely from person to person. The symptoms are usually very subtle at first and worsen slowly. The rate of worsening also varies from person to person.

The earliest symptoms are usually changes in behavior, mood, or personality. The person may act unlike his or her usual self. The following are common behavioral changes:

• Apathy (indifference) or withdrawal
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• Severe depression in a person who has never been depressed before
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• Blunting or dullness of emotions
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• Loss of inhibition
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• Impulsive behavior in a usually cautious person
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• Bad manners, rudeness
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• Saying or doing inappropriate things in public
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• Impatience
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• Becoming extroverted, very talkative
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• Inappropriate joking
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• Aggressiveness
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• Restlessness or agitation
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• Poor judgment
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• Paranoia
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• Selfishness
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• Difficulty coping with changes from routine
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• Development of obsessive routines
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• Childlike behavior
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• Changes in sexual behavior

Other common symptoms in Pick disease include the following:

• Speech problems: The person may have trouble finding the right word, may speak less, or may stop speaking altogether. His or her sentences may be incomplete or organized strangely. The person may lose the ability to understand written, spoken, or signed language.
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• Changes in eating habits: The person may begin overeating, eating greedily, eating excessive amounts of sweets, or drinking large amounts of alcohol. He or she may gain weight.
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• Poor attention: The person may have problems paying attention, concentrating, or carrying on a sustained conversation.

Any change in behavior, mood, or personality in a middle-aged person may signal a problem. A visit to the person’s health care provider is a good idea if the change interferes with any of the following:

• The person’s ability to take care of himself or herself
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• The person’s ability to maintain health and safety
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• The person’s ability to sustain social relationships
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• The person’s ability to work effectively at his or her job
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• The person’s ability or interest in participating in activities that he or she enjoys
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• The person’s ability to drive or carry out other complex tasks

Many conditions can cause dementia or dementia-like symptoms in a middle-aged person, including both medical and psychological problems. Some of these conditions can be reversed, or at least stopped or slowed down. Therefore, it is extremely important that the person with symptoms be checked thoroughly to rule out treatable conditions.

An early diagnosis allows treatment to begin earlier in the disease, when it has the best chance of improving symptoms. Early diagnosis also allows the affected person to plan activities and make arrangements for care while he or she can still take part in making decisions.

The only way to confirm the diagnosis of Pick disease is to look at the brain directly and to identify Pick bodies and swollen neurons. This is possible only if the brain is biopsied. This means taking a small sample of brain tissue for testing. This is done by a neurosurgeon, a surgeon who specializes in operating on the brain. The sample is examined under a microscope by a pathologist, a doctor who specializes in diagnosing diseases by looking at tissues in this way. The brain can also be examined in this way at autopsy, after a person’s death.

Biopsy offers a definite diagnosis while the person is still alive, and many people and their families are choosing to undergo this procedure. Without biopsy, the diagnosis in a living person is usually made on the basis of symptoms and ruling out other conditions. This is done by a combination of medical interview, physical and mental examinations, lab tests, imaging studies, and other tests. A doctor should discuss the potential risks and benefits of each approach with the patient and family.

The medical interview involves detailed questions about the symptoms and how they have changed over time. Your health care provider will also ask about medical and mental problems now and in the past, family medical problems, medications taken now and in the past, work and travel history, habits, and lifestyle.

A detailed physical examination is done to rule out medical problems that might cause dementia. The examination should include a mental status evaluation. This involves answering the examiner’s questions and following simple directions. In some cases, the health care provider will conduct neuropsychological testing or refer the person to a psychologist for such testing.

At any point in this process, your primary care provider may refer you to a specialist in brain disorders (neurologist or psychiatrist).

Neuropsychological testing

Neuropsychological testing is the most accurate method of pinpointing and documenting a person’s cognitive problems and strengths.

• This can help give a more accurate diagnosis of the problems and thus can help in treatment planning.
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• The testing involves answering questions and performing tasks that have been carefully prepared for this purpose. The test is given by a neurologist, psychologist, or other specially trained professional.
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• It addresses the individual’s appearance, mood, anxiety level, and experience of delusions or hallucinations.
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• It assesses cognitive abilities such as memory, attention, orientation to time and place, use of language, and abilities to carry out various tasks and follow instructions.
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• Reasoning, abstract thinking, and problem solving also are tested.

Lab tests

These include blood tests to rule out infections, blood disorders, chemical abnormalities, hormonal disorders, and liver or kidney problems that could cause dementia symptoms.

Imaging studies

Brain scans cannot detect Pick disease specifically, but they can detect atrophy and other abnormalities in the frontal and temporal lobes. The scan also helps rule out other conditions that can cause dementia.

• MRI or CT scan of the brain
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• Single-photon emission computed tomography (SPECT) or positron-emission tomography (PET) scan: This scans are used in certain cases when the diagnosis is doubtful. They are especially good at detecting abnormal brain function. SPECT and PET scans are available only at some large medical centers.

Other tests

Any of these tests may be ordered as part of the evaluation of dementia.

• Electroencephalography (EEG) is a measurement of the electrical activity of the brain. It can be useful in some cases to rule out other conditions.
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• Spinal tap (lumbar puncture) is a method of obtaining a sample of cerebrospinal fluid. This may be done to rule out certain other brain conditions that can cause dementia.

There is no cure for Pick disease. Treatment is aimed at improving behavior and mood problems and relieving other symptoms. For example, a speech therapist may be able to help the person improve his or her ability to communicate. Medication is helpful in many patients in easing mood and emotional difficulties. For more information, see Dementia.

|Self-Care at Home|

Individuals with Pick disease should remain physically, mentally, and socially active as long as they are able.

• Daily physical exercise helps maximize body and mind functions and maintains a healthy weight. This can be as simple as a daily walk. The walk should be at a brisk pace and last at least 20 minutes.
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• The individual should engage in as much mental activity as he or she can handle. It is believed that mental activity and stimulation may slow the progression of the disease. Puzzles, games, reading, and safe hobbies and crafts are good choices. Ideally, these activities should be interactive. They should be of an appropriate level of difficulty to make sure that the person does not become overly frustrated.
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• Social interaction is stimulating and enjoyable for most people with early or intermediate stages of the disease. Most senior centers or community centers have scheduled activities that are suitable for those with dementia.

A balanced diet that includes low-fat protein foods and plenty of fruits and vegetables will help maintain a healthy weight and prevent malnutrition and constipation. An individual with Pick disease should not smoke, both for health and safety reasons.

|Medical Treatment|

Even though Pick disease is not reversible, treatment can slow the progression of symptoms in some people. Relieving symptoms can improve function significantly. Some of the important treatment strategies in dementia are described here.

Nondrug treatment

Behavior disorders such as agitation and aggression may improve with various interventions. Some interventions focus on helping the individual adjust or control his or her behavior. Others focus on helping caregivers and other family members change the person’s behavior. These approaches sometimes work better when combined with drug treatment.

Drug treatment

The symptoms of Pick disease can sometimes be relieved, at least temporarily, by medication. Many different types of medications have been or are being tried in dementia. One group of drugs used in Alzheimer disease, the cholinesterase inhibitors, has been tried in Pick disease.

• These drugs increase the levels of acetylcholine, a brain hormone related to memory and attention.
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• These drugs are not a cure. They improve speech and language in some people with the disease. In other people, they have no effect.
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• Many people with Pick disease cannot take one of these drugs, donepezil (Aricept), because the drug can worsen symptoms such as agitation and aggression.

Other drugs are being used in people with Pick disease. On the basis of what we know from research about the disease, these drugs may help.

• The antioxidant tocopherol (vitamin E) may counteract the damage in brain cells that causes Pick disease and slow the worsening of the disease.
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• Antidepressants known as selective serotonin reuptake inhibitors (SSRIs) are used in many people with the disease to increase levels of a brain hormone called serotonin. This hormone may improve thinking.

Other, more experimental, treatments are being used by some specialists who treat Pick disease. These treatments are not widely accepted as helpful in the disease.

• Anti-inflammatory drugs are being tried on the premise that inflammation is one cause of the brain damage in Pick disease.
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• Hormone replacement therapy has been given to some women who have been through menopause and have dementia, but this approach has been questioned by many experts. The rationale is that the loss of estrogen at menopause takes away one line of protection from the disease.

Certain drugs are used to treat specific symptoms or behavior changes.

• Mood swings and emotional outbursts may improve with antidepressant or mood-stabilizing drugs.
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• Agitation, anger, and disruptive or psychotic behavior are often relieved by antipsychotic medication or mood stabilizers.

|Medications|

These are some of the most commonly used drugs in Pick disease.

• Cholinesterase inhibitors - Donepezil (Aricept) (may worsen agitation and aggression), rivastigmine (Exelon), and galantamine/galanthamine (Reminyl). These drugs have in effect replaced an older drug called tacrine (Cognex).
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• Antidepressants/anxiolytics – Mirtazapine (Remeron), venlafaxine (Effexor), fluoxetine (Prozac), sertraline (Zoloft), paroxetine (Paxil), citalopram (Celexa),
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• Antipsychotics – Haloperidol (Haldol), risperidone (Risperdal), quetiapine (Seroquel), olanzapine (Zyprexa)

All drugs cause side effects. The aim in prescribing a drug is that the benefits of the drug outweigh the side effects. People with dementia who are taking any of these drugs must be checked often to make sure that if side effects occur, they are tolerated and do not cause serious problems.

These drugs may interact with each other or with other drugs. This is especially important in middle-aged and older people, who may take several different drugs for various medical disorders. Side effects may be due not to one specific drug, but to combinations of drugs.

|Follow-up|

After Pick disease has been diagnosed and treatment begun, the individual requires regular checkups with his or her health care provider.

• These checkups allow the health care provider to see how well treatment is working and to make adjustments as necessary.
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• They allow detection of new medical and behavior problems that could benefit from treatment.
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• These visits also give the family caregiver(s) an opportunity to discuss problems in the individual’s care.

Eventually, the person with Pick disease will become unable to care for himself or herself, or even to make decisions about his or her care.

• It is best for the person to discuss future care arrangements with family members as early as possible, so that his or her wishes can be clarified and documented for the future.
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• Your health care provider can advise you about legal arrangements that should be made to ensure that these wishes are observed.

|Prevention|

There is no known way to prevent Pick disease. Being alert for symptoms and signs may allow earlier diagnosis and treatment. Appropriate treatment can slow or relieve symptoms and behavior problems in some people.

Some experts think that education and other forms of intellectual challenge may help protect people against the disease. People with low levels of education and mental/intellectual activity are said to be at a higher risk for the disease and to be more likely to have more severe disease, but this has not been proven conclusively.

|Outlook|

Pick disease starts slowly but finally results in severe brain damage.

• People with the disease gradually lose cognitive functions, ability to carry out everyday activities, and ability to respond appropriately to their surroundings.
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• Many lose their ability to communicate and understand language.
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• They eventually become completely dependent on others for care.
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• These losses occur in everyone with the disease, but the speed with which they occur varies from person to person and may be slowed by treatment.

Pick disease is considered to be a terminal disease.

• The actual cause of death usually is a physical illness such as pneumonia. Such illnesses can be debilitating in a person who is already weakened by the effects of the disease.
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• On average, a person with Pick disease lives about 7 years after the disease is diagnosed. In some people, the disease progresses to death much more rapidly. Others live 10 years or longer after onset of the disease. The differences in the speed at which the condition worsens have not been explained.

|Support Groups and Counseling|

If you are a caregiver for a person with Pick disease, you know that the disease tends to be more stressful for the family members than for the affected person. Caring for a person with dementia can be very difficult. It affects every aspect of your life, including family relationships, work, financial status, social life, and physical and mental health.

• You may feel unable to cope with the demands of caring for a dependent, difficult relative.
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• Besides the sadness of seeing the effects of your loved one’s disease, you may feel frustrated, overwhelmed, resentful, and angry.
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• These feelings may in turn leave you feeling guilty, ashamed, and anxious.
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• Depression is not uncommon but usually gets better with treatment.

Caregivers have different thresholds for tolerating these challenges.

• For many caregivers, just “venting” or talking about the frustrations of caregiving can be enormously helpful.
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• Others need more support but may feel uneasy about asking for the help they need.
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• One thing is certain, though: If the caregiver is given no relief, he or she can burn out, develop his or her own mental and physical problems, and become unable to care for the affected person.

This is why support groups were invented. Support groups are groups of people who have lived through the same difficult experiences and want to help themselves and others by sharing coping strategies. Mental health professionals strongly recommend that family caregivers take part in support groups. Support groups serve a number of different purposes for a person living with the extreme stress of being a caregiver for a person with Pick disease.

• The group allows the person to express his or her true feelings in an accepting, nonjudgmental atmosphere.
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• The group’s shared experiences allow the caregiver to feel less alone and isolated.
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• The group can offer fresh ideas for coping with specific problems.
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• The group can introduce the caregiver to resources that may be able to provide some relief.
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• The group can give the caregiver the strength he or she needs to ask for help.

Support groups meet in person, on the telephone, or on the Internet. To find a support group that works for you, contact the organizations listed below. You can also ask your health care provider or behavior therapist, or go on the Internet. If you do not have access to the Internet, go to the public library.

For more information about support groups, contact the following agencies:

• Eldercare Locator Service – (800) 677-1116
  
  
• Family Caregiver Alliance, National Center on Caregiving - (800) 445-8106
  
  
• Picksdisease.org
  
  
• Pick’s Information Page
  
  
• National Alliance for Caregiving
  
  
• Supportpath, Dementia

|Web Links|

Alzheimer Society (Canada), Pick's Disease

National Institute of Neurological Diseases and Stroke (NINDS), Pick's Disease Information Page

Pick's Disease Support Group (United Kingdom)

Media file 1: Motor perseveration in a patient with Pick disease. The patient is asked to copy the loops (as demonstrated by the examiner in the first line).

Media type:  Illustration

Alzheimer disease, Alzheimer’s disease, aphasia, brain degeneration, brain disorder, brain damage, cognitive problems, frontal lobe degeneration, frontal lobe dementia, frontotemporal dementia, language disorder, lobar atrophy, neurons, Pick bodies, Pick complex, Pick’s disease, primary progressive aphasia, speech disorder, Pick Disease

Author: Anna M Barrett, MD, Director, Stroke Rehabilitation Research Program, Kessler Medical Rehabilitation Research and Education Corporation.

Editors: Nestor Galvez-Jimenez, MD, Program Director of Movement Disorders, Director of Neurology Residency Training Program, Department of Neurology, Division of Medicine, Cleveland Clinic Florida; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, ; Helmi L Lutsep, MD, Associate Director, Oregon Stroke Center; Associate Professor, Department of Neurology, Oregon Health and Science University.