Pick Disease of the Brain

Pick’s disease is a rare, progressive and irreversible form of dementia. Dementia means that the brain does not function normally and patients can have difficulty with language, behavior, cognition (thinking), judgment, and memory. Memory changes are seen in other forms of dementia like Alzheimer’s disease. Unlike Alzheimer’s which can affect many different parts of the brain, Pick’s disease only affects certain areas of the brain.

Pick’s disease is also known as frontotemporal dementia, meaning that it only affects the frontal and temporal lobes of the brain. The brain’s frontal lobe controls important facets of everyday life, including planning, judgment, emotions, speaking, understanding speech, and some forms of movement.

Pick’s disease and other forms of frontotemporal dementia are believed to account for up to 15 percent of all dementia cases. (Alzheimer’s Association). Like patients with other types of dementia, a person with Pick’s disease may suffer from drastic personality changes.

Pick’s disease is caused by abnormal amounts or types of a nerve cell protein (tau) that is found in all nerve cells. This abnormal protein is call Pick bodies or Pick cells. Abnormal amounts or different types of this protein in the brain’s frontal and temporal lobe nerve cells causes degeneration of nerve cells which results in shrinkage =of the brain tissue. This shrinkage causes the dementia symptoms of Pick’s disease.

Scientists cannot explain what causes of the abnormal protein. However, geneticists have found abnormal genes that can cause Pick’s disease and they have documented the occurrence of the disease in family members.

Further research into the cause of Pick’s disease is being done through the National Institute of Neurological Disorders and Stroke (NINDS) and other branches of the National Institutes of Health (NIH).

Symptoms of Pick’s disease get progressively worse as brain tissue continues to shrink. Many of the symptoms can make social interaction difficult, as behavioral changes make conducting oneself in a socially acceptable manner challenging.

Behavioral and emotional changes caused by Pick’s disease include:

• abrupt mood changes
• compulsive or inappropriate behavior
• depression-like symptoms, such as disinterest in daily activities
• difficulty keeping a job
• poor personal and social skills
• poor personal hygiene
• repetitive behavior
• withdrawal from social interaction

Language and neurological changes caused by Pick’s disease include:

• decrease in writing or reading skills
• “echoing,” or repeating what was just said
• inability to speak, difficulty speaking, or trouble understanding speech
• increased memory loss
• physical weakness
• shrinking vocabulary
• trouble finding the right word

The early onset of personality changes helps your doctor differentiate between Pick’s disease and Alzheimer’s. Pick’s disease can also occur at any earlier age than Alzheimer’s. Cases have been reported as early as 20 years of age and symptoms can begin between the age of 40 and 60 years. The average age at onset is 54. (PubMed)

There is no single test to determine if a person has Pick’s disease. A doctor will use history, physical findings and special imaging tests to diagnose Pick’s disease. These include:

• a complete medical history
• interviews with family members about the person’s behavior
• a physical examination and detailed neurologic examination
• speech and writing tests
• magnetic resonance imaging (MRI) scan using magnetic and radio waves to show details of the brain
• computed tomography (CT) a specialized type of x-ray used to look at tissue and bone structure
• positron emission tomography (PET) scan that uses a radioactive chemical to look at the function of the brain tissue

Imaging tests can help your doctor see the shape of the brain and any changes that may be present. They can also help your doctor rule out other conditions that could be causing dementia symptoms, such as brain tumors or stroke. Blood tests may also be taken to rule out dementia caused by too-little thyroid hormone (hypothyroidism) or a vitamin B12 deficiency, both common causes of dementia in the elderly.

Currently, there are no treatments that effectively slow the progression of Pick’s disease.

There are, however, several treatments that can help diminish the behavioral symptoms associated with the condition. Antidepressants and antipsychotics are two of the most common types of medications used to treat the symptoms of Pick’s disease.

Doctors will also test for and treat other problems that could worsen confusion. These may include:

• anemia, a reduced amount of red blood cells (cells that carry oxygen to the body’s tissues), which can cause fatigue, headaches, moodiness, and difficulty concentrating
• decreased oxygen levels
• depression and other mood disorders
• heart failure
• kidney or liver failure
• nutritional disorders
• thyroid disorders

Unfortunately, the outlook for people with Pick’s disease and other types of frontotemporal dementia is poor. Pick’s disease is a rapidly progressive disease without a cure.

Those with advanced cases of Pick’s disease typically need to live in an assisted living facility where 24-hour care is available. In the worst cases of Pick’s disease, the patient becomes mute and bedbound.

The survival rate for people with Pick’s disease averages about six to eight years. Rapidly progressing cases can shorten a person’s life to as little as two years, but some patients have lived for up to 20 years.