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Pick’s disease was first described in 1892 by Arnold Pick, a Czech neurologist and psychiatrist. Some early medical texts refer to Pick’s disease as frontotemporal dementia, but it is now understood to be one of three very specific causes of frontotemporal dementia. Although similar to Alzheimer’s disease, in that it is a tau-related dementia, Pick’s is far less common.
Pick’s disease is a rare and irreversible form of progressive dementia that affects the frontal and temporal lobes of the brain and is characterized by progressive changes in personality, behavior difficulties and speech problems like aphasia. The disease quickly and steadily becomes worse, leading to total disability early in the disease process and death within eight to ten years. After the onset of symptoms, it may take a couple years to receive an official diagnosis. As a result, the average span of time between diagnosis and death is around five years.
Although the disease can occur in a person’s early 20s and as late in life as 80, Pick’s disease is typically diagnosed between the ages of 40 and 60, with the average age being 54. Approximately 50,000 to 60,000 people in the United States have Pick’s disease. Two to three percent of all dementia cases are frontotemporal in nature.
Symptoms of Pick’s disease slowly worsen over time as more and more of the brain is affected. The symptom of aphasia often enables doctors to distinguish Pick’s disease from other types of frontotemporal dementia which exhibit symptoms of behavior problems and personality changes as their first primary symptoms, although Pick’s disease can also cause behavior problems as well.
The symptoms of Pick’s disease vary from person to person, but individuals with Pick’s disease tend to behave poorly in social situations. Behavioral problems, which continue to worsen, can be one of the most disturbing characteristics of the disease.
Pick’s disease behavioral symptoms and changes include:
Emotional symptoms and changes include:
Symptoms and changes in language include:
Symptoms and changes in nervous system include:
Other symptoms and changes include:
As the disease progresses, symptoms become profoundly and increasingly more noticeable and severe. Over time, almost every aspect of cognition and daily function can become extremely impaired.
Individuals with Pick’s disease have an abnormal form of protein called tau (referred to as Pick bodies or Pick cells) inside the nerve cells in the damaged parts of the brain. Tau is present is all nerve cells; however, individuals with Pick’s disease have an unusual type or abnormal amount of tau.
The underlying cause of Pick’s disease is unknown; however, up to 25% of cases appear to have a familial or hereditary component.
Pick’s disease is diagnosed based on symptoms experienced and testing. A brain biopsy is the only way to confirm a diagnosis. Additional testing, which is often used to rule out other causes of dementia, may include:
Although there is no known cure and no treatment to slow the progression of Pick’s disease, medications can be used to manage symptoms, including many of the same used to treat symptoms of other types of dementia. These may include:
Your doctor will need to test for and treat other problems that can make Pick’s disease symptoms worse. These may include:
Medications may be required to control agitated, aggressive and dangerous behaviors.
Behavior modification (rewarding appropriate or positive behaviors and ignoring inappropriate behaviors) may be beneficial.
Psychotherapy may be helpful but can cause disorientation and confusion for some individuals.
To reduce disorientation, reality orientation can be used to reinforce environmental and other cues.
As symptoms become worse, assistance with personal hygiene and self-care will become essential. Eventually, 24-hour care will be required.
Individuals with Pick’s disease should seek legal advice early following diagnosis of the disease. Setting up an advance care directive, power of attorney and other legal actions will make it easier to navigate decisions that need to be made as the disease progresses.
Pick’s disease can be particularly challenging for care providers, especially family and friends. The personality changes can be so extreme that loved ones don’t recognize the individual as the person they once knew. As the disease progresses, it becomes more and more difficult for a person with Pick’s disease to complete even the most fundamental care for themselves. These intense care needs and extreme personality changes often lead to placement in a facility that provides dementia care.
Pick’s disease and Alzheimer’s disease are both types of illnesses that cause dementia. Although they have some of the same symptoms, there are key differences.
Unlike individuals with Alzheimer’s, people with Pick’s disease:
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