Semantic dementia; Dementia - semantic; Frontotemporal dementia; FTD; Arnold Pick disease; 3R tauopathy
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 down through families.
Pick disease is rare. It can occur in people as young as 20. But it usually begins between ages 40 and 60. The average age at which it begins is 54.
The disease gets worse slowly. Tissues in parts of the brain shrink over time. Symptoms such as behavior changes, speech difficulty, and problems thinking occur slowly and 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 include:
- Not able to keep 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
- Cannot speak (mutism)
- Decreased ability to read or write
- Difficulty finding a word
- Difficulty speaking or understanding speech (aphasia)
- Repeating anything spoken to them (echolalia)
- Shrinking vocabulary
- Weak, uncoordinated speech sounds
NERVOUS SYSTEM PROBLEMS
- 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 doctor might order tests to help rule out other causes of dementia, including dementia due to metabolic causes. Pick disease is 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, people with Pick disease take the same medicines used to treat other types of dementia.
In some cases, stopping or changing medicines that worsen confusion or that are not needed can improve thinking and other mental functions. Medicines include:
It is important to treat any disorders that can cause confusion. These include:
- Decreased oxygen (hypoxia) level
- Heart failure
- High carbon dioxide level
- Kidney failure
- Liver failure
- Nutritional disorders
- Thyroid disorders
- Mood disorders, such as depression
Medicines may be needed to control aggressive, dangerous, or agitated behaviors.
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 is safe to do so).
Talk therapy (psychotherapy) does not always work. This is 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, monitoring and help with personal hygiene and self-care may be needed. 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 with Pick disease and their family may need to seek legal advice early in the course of the disorder. Advance care 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. The person becomes totally disabled early in the course of the disease.
Pick disease commonly causes death within 8 to 10 years, usually from infection, or sometimes because body systems fail.
When to Contact a Medical Professional
Call your doctor or go to the emergency room if mental function gets worse.
There is no known prevention.
Bang J, Spina S, Miller BL. Frontotemporal dementia. Lancet. 2015;386(10004):1672-1682. PMID: 26595641 www.ncbi.nlm.nih.gov/pubmed/26595641.
Peterson R, Graff-Radford J. Alzheimer disease and other dementias. In: Daroff RB, Jankovic J, Mazziotta JC, Pomeroy SK, eds. Bradley's Neurology in Clinical Practice. 7th ed. Philadelphia, PA: Elsevier; 2016:chap 95.
Reviewed By: Amit M. Shelat, DO, FACP, Attending Neurologist and Assistant Professor of Clinical Neurology, SUNY Stony Brook, School of Medicine, Stony Brook, NY. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.