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Alternative Names Return to topSemantic dementia; Dementia - semantic; Frontotemporal dementia; Arnold Pick's disease
Definition Return to top
Pick's disease is a rare and permanent form of dementia that is similar to Alzheimer's disease, except that it tends to affect only certain areas of the brain.
Causes Return to top
People with Pick's disease have abnormal substances (called Pick bodies and Pick cells) inside nerve cells in the damaged areas of the brain.
Pick bodies and Pick cells contain an abnormal form of a protein called tau. This protein is found in all nerve cells. But some people with Pick's disease have an abnormal amount or type of this protein.
The exact cause of the abnormal form of the protein is unknown. A gene for the disease has not yet been found. Most cases of Pick's disease are not passed down through families.
Pick's disease is rare. It is more common in women than men. It can occur in people as young as 20, but usually begins between ages 40 and 60. The average age at which it begins is 54.
Symptoms Return to top
The disease can get worse slowly. Tissues in the temporal and frontal lobes of the brain start to shrink over time. Symptoms such as behavior changes, speech difficulty, and impaired thinking occur slowly, but continue to get worse.
The early personality changes can help doctors tell Pick's disease apart from Alzheimer’s. Memory loss is often the main, and earliest, symptom of Alzheimer's.
People with Pick's disease tend to behave the wrong way in different social settings. The changes in behavior continue to get worse and are often one of the most disturbing symptoms of the disease. Some patients will have difficulty with language (trouble finding or understanding words or writing).
General symptoms are listed below.
Exams and Tests Return to top
The doctor will ask you about your medical history and symptoms.
Your health care provider might order tests to help rule out other causes of dementia, including dementia due to metabolic causes. These tests can include:
A brain biopsy is the only test that can confirm the diagnosis.
Treatment Return to top
There is no specific treatment for Pick's disease. Certain antidepressants may help manage mood swings related to Pick's disease, but further research is needed.
Sometimes patients with Pick's take the same medications used to treat other types of dementia, such as medications that decrease the breakdown of the chemical messenger, acetylcholine (anticholinesterase inhibitors), and memantine (Namenda).
In some cases, stopping or changing medications that worsen confusion or that are not essential can improve thinking and other cognitive functions. This may include medications such as:
It's important to treat any disorders that contribute to confusion. These may include:
Treating any medical and psychiatric disorders often helps improve mental function.
Medications may be needed to control aggressive, dangerous, or agitated behaviors.
Some patients may need hearing aids, glasses, cataract surgery, or other treatments.
Behavior modification can help some people control unacceptable or dangerous behaviors. This consists of rewarding appropriate or positive behaviors and ignoring inappropriate behaviors (when it's safe to do so).
Formal psychotherapy treatment doesn't always work, because it can cause further confusion or disorientation.
Reality orientation, which reinforces environmental and other cues, may help reduce disorientation.
Depending on the symptoms and severity of the disease, the patient may need monitoring and help with personal hygiene and self-care. Eventually, there may be a need for 24-hour care and monitoring at home or in a special facility. Family counseling can help the person cope with the changes needed for home care.
Care may include:
People may need legal advice early in the course of the disorder. Advance directives, power of attorney, and other legal actions can make it easier to make ethical decisions regarding the care of the person with Pick's disease.
Support Groups Return to top
Some communities may have support groups (such as the Alzheimer's - support group, elder care - support group, or others).
Outlook (Prognosis) Return to top
The disorder quickly and steadily becomes worse. Patients become totally disabled early in the course of the disease.
Commonly, Pick's disease causes death within 2 - 10 years, usually from infection and sometimes from general failure of the body systems.
Possible Complications Return to top
When to Contact a Medical Professional Return to top
Call your health care provider if you develop symptoms of Pick's disease.
Call your health care provider or go to the emergency room if mental function gets worse (which may mean that another disorder has developed).
Prevention Return to top
There is no known prevention.
References Return to top
Moore DP, Jefferson JW. Handbook of Medical Psychiatry. 2nd ed. St. Louis, Mo: Mosby; 2004.
Pearce JM. Pick's disease. J Neurol Neurosurg Psychiatry. 2003;74(2):169.
Grossman M. Frontotemporal dementia: a review. J Intl Neuropsychol Soc. 2002;8:566-583.
Grossman M. Progressive aphasic syndromes: clinical and theoretical advances. Curr Opin Neurol. 2002;15:409-413.
McKhann G, Albert M, Grossman M, Miller B, Dickson D, Trojanowski J, et al. Clinical and pathological diagnosis of frontotemporal dementia. Arch Neurol. 2001;58:1803-1809.
Goetz CG. Textbook of Clinical Neurology. 3rd ed. Philadelphia, Pa: Saunders; 2007.Update Date: 2/6/2008 Updated by: Daniel Kantor, MD, Director of the Comprehensive MS Center, Neuroscience Institute, University of Florida Health Science Center, Jacksonville, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.