Pick diseaseSemantic dementia; Dementia - semantic; Frontotemporal dementia; Arnold Pick disease
People with Pick 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 disease have an abnormal amount or type of this protein.
The exact cause of the abnormal form of the protein is unknown. Many different abnormal genes have been found that can cause Pick disease.Some cases of Pick disease are passed downin families.
Pick disease is rare. It can occur in people as young as 20. But itusually begins between ages 40 and 60. The average age at which it begins is 54.
The disease gets worse slowly. Tissues in the temporal and frontal lobes of the brain start to shrink over time. Symptoms such as behavior changes, speech difficulty, andproblems thinking occur slowly, but continue to get worse.
Early personality changes can help doctors tell Pick disease apart from Alzheimer disease. (Memory loss is often the main, and earliest, symptom of Alzheimer disease.)
People with Pick 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 persons have more difficulty with decision making, complex tasks, or language (trouble finding or understanding words or writing).
General symptoms are listed below.
- Not able tokeep a job
- Compulsive behaviors
- Inappropriate behavior
- Inability to function or interact in social or personal situations
- Problems with personal hygiene
- Repetitive behavior
- Withdrawal from social interaction
- Abrupt mood changes
- Decreased interest in daily living activities
- Failure to recognize changes in behavior
- Failure to show emotional warmth, concern, empathy, sympathy
- Inappropriate mood
- Not caring about events or environment
- Can notspeak (mutism)
- Decreased ability to read or write
- Difficulty finding a word
- Difficulty speaking or understanding speech (aphasia)
- Repeat anything spoken to them (echolalia)
- Shrinking vocabulary
- Weak, uncoordinated speech sounds
- Increased muscle tone (rigidity)
- Memory loss that gets worse
- Movement/coordination difficulties (apraxia)
Exams and Tests
The doctor will ask 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. Pick disease is tentatively diagnosed based on symptoms and results of tests, including:
- Assessment of the mind and behavior (neuropsychological assessment)
- Brain MRI
- Electroencephalogram (EEG)
- Examination of the brain and nervous system (neurological exam)
- Examination of the fluid around the central nervous system (cerebrospinal fluid) after a lumbar puncture
- Head CT scan
- Tests of sensation, thinking and reasoning (cognitive function), and motor function
A brain biopsy is the only test that can confirm the diagnosis.
There is no specific treatment for Pick disease. Medicines may help manage mood swings.
Sometimes patients with Pick diseasetake the same medicines used to treat other types of dementia.
In some cases, stopping or changing medicines that worsen confusion or that are notneeded can improve thinking and other mental functions. Medicines include:
It isimportant to treat any disorders thatcan cause confusion. These include:
- Decreased oxygen (hypoxia) levels
- Heart failure
- High carbon dioxide levels
- Kidney failure
- Liver failure
- Nutritional disorders
- Thyroid disorders
- Psychiatric conditions such as depression
Medicines may be needed to control aggressive, dangerous, or agitated behaviors.
Some patients may benefit from 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 issafe to do so).
Psychotherapy (talk therapy) does notalways work. This isbecause 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:
- Adult protective services
- Community resources
- Visiting nurses or aides
- Volunteer services
People may need legal advice early in the course of the disorder. Advance directive, power of attorney, and other legal actions can make it easier to make decisions regarding the care of the person with Pick disease.
The disorder quickly and steadily becomes worse. Patients become totally disabled early in the course of the disease.
Pick disease commonly causes death within8 to 10 years, usually from infection but sometimes from failure of the body systems.
- Abuse by an over-stressed caregiver
- Loss of ability to care for self or perform normal activities
- Loss of ability to interact with others
- Progressive loss of ability to function
- Side effects of medications used to treat the disorder
- Reduced life span
When to Contact a Medical Professional
Callthe health care provider or go to the emergency room if mental function gets worse.
There is no known prevention.
Apostolova LG, DeKosky ST, Cummings JL. Dementias. In: Daroff RB, Fenichel GM, Jankovic J, Mazziotta JC. Bradley’s Neurology in Clinical Practice. 6th ed. Philadelphia, PA:Elsevier Saunders; 2012:chap 66.
Munoz DG, Morris HR, Rossor M. Pick's disease. In: Dickson D, Weller RO, eds. Neurodegeneration: The Molecular Pathology of Dementia and Movement Disorders. 2nd ed. Hoboken NJ: Wiley-Blackwell; 2011:chap 16.
Review Date: 2/27/2013
Reviewed By: Luc Jasmin, MD, PhD, Department of Neurosurgery, Cedars Sinai Medical Center, Los Angeles and Department of Anatomy, University of California, San Francisco, CA. Review provided by VeriMed Healthcare Network. Also reviewed by A.D.A.M. Health Solutions, Ebix, Inc., Editorial Team: David Zieve, MD, MHA, Bethanne Black, Stephanie Slon, and Nissi Wang.