Pick’s disease Health Article

Definition

Pick's disease is a rare and irreversible form of dementia that is somewhat similar to Alzheimer's disease. However, Pick's disease tends to affect only certain areas of the brain, whereas Alzheimer's can affect any part.

Alternative Names

Primary progressive aphasia; Aphasia - primary progressive; Semantic dementia; Dementia - semantic; Frontotemporal dementia; Arnold Pick's disease

Causes, incidence, and risk factors

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 genetic basis 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 of onset is 54.

Symptoms

The disease can progress 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 appearance of personality changes help distinguish Pick's disease from Alzheimer’s. Memory loss is often the main, and earliest symptom of Alzheimer's. People with Pick's disease tend to behave inappropriately in different social settings. The changes in behavior continue to worsen and are often one of the most disturbing symptoms of the disease. Some patients will begin with worsening language impairment (trouble finding or understanding words or writing).

General symptoms are listed below.

Behavioral changes:

• Can't 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

Emotional changes:

• Abrupt mood changes
• Decreased interest in daily living activities
• Failure to recognize behavioral changes
• Failure to show emotional warmth, concern, empathy, sympathy
• Inappropriate mood
• Indifference to events or environment

Language changes:

• Can't speak (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

Neurological problems:

• Increased muscle tone (rigidity)
• Memory loss that gets worse
• Movement/coordination difficulties (apraxia)
• Weakness

Other problems:

• Urinary incontinence

Signs and tests

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:

• Brain MRI
• Cerebrospinal fluid examination after a lumbar puncture
• EEG (electroencephalogram)
• Head CT scan
• Neurological exam
• Neuropsychological assessment
• Psychological studies
• Tests of sensation, cognitive function, and motor function

A brain biopsy is currently the only test that can confirm the diagnosis.

Treatment

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.

Depending on the symptoms and severity of the disease, the patient might 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 an institutionalized care setting. Family counseling can help the person cope with the changes needed for home care.

Visiting nurses or aides, volunteer services, homemakers, adult protective services, and other community resources may be helpful in caring for the person.

In some cases, stopping or changing medications that worsen confusion or that are not essential can improve cognitive function. This may include medications such as:

• Analgesics
• Anticholinergics
• Central nervous system depressants
• Cimetidine
• Lidocaine

It's important to treat any disorders that contribute to confusion. These may include:

• Anemia
• Decreased oxygen (hypoxia)
• Heart failure
• Infections
• Nutritional disorders
• Thyroid disorders
• Psychiatric conditions such as depression

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, with repeated reinforcement of environmental and other cues, may help reduce disorientation.

Legal advice may be appropriate 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.