Aphasia - primary progressive

Definition

Pick's disease is a rare and irreversible form of dementia. It causes personality changes, loss of social skills, loss of intellectual function, and speech disturbances.

Alternative Names

Causes

In some ways, Pick's disease is similar to Alzheimer's disease. However, Pick's disease tends to affect only certain areas of the brain, while Alzheimer's can affect any part.

It is the early appearance of a personality changes that help distinguish Pick's disease from Alzheimer's. (Memory loss is often the main symptom of Alzheimer's.) People with Pick's disease tend to behave inappropriately in different social settings. The changes in behavior continue to get worse and are often one of the most disturbing symptoms of the disease. 

Pick's disease is rare. It is more common in women than men. It may occur in people as young as 20, but usually begins between ages 40 and 60. The average age of onset is 54.

The disease can progress slowly. Over time, the tissues in two parts of the brain (the temporal and frontal lobes) shrink. This shrinking is called atrophy. Symptoms such as behavioral changes, speech difficulty, and impaired intellect occur gradually, but continue to get worse.

People with Pick's disease have abnormalities 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 tau. The exact cause of these abnormalities is unknown.

A genetic basis for the disease has not yet been determined. Most cases of Pick's disease are not inherited.

Symptoms

Behavior changes

• Inappropriate behavior
• Compulsive behaviors (for example, overeating or only eating one type of food)
• Repetitive behavior
• Withdrawal from social interaction
• Inability to function or interact in social or personal situations
• Can't keep a job
• Problems with personal hygiene

Emotional changes

• Abrupt mood changes
• Does not show emotional warmth, concern, empathy, sympathy
• Indifference to events or environment (apathy)
• Does not recognize behavioral changes
• Inappropriate mood
• Decreased interest in daily living activities

Language changes

• Echolalia (person repeats anything spoken to them)
• Aphasia (decreased language ability, difficulty speaking or understanding speech)
• Difficulty finding a word
• Shrinking vocabulary
• Weak, uncoordinated speech sounds
• Can't speak (mutism)
• Decreased ability to read or write

Neurological problems

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

Other problems

• Urinary incontinence

Exams and Tests

The doctor will ask you about your medical history and symptoms.

Tests may be ordered to help rule out other causes of dementia, including dementia due to metabolic causes. A neurological examination can help reveal what part of the brain is affected. Psychological studies and tests of sensation, cognitive function, and motor function may be abnormal.

• Neuropsychological assessment shows a pattern of mental decline that suggests selective loss of cognitive function that can be localized to the frontal and/or temporal lobes of the brain.
• An EEG (electroencephalogram) shows nonspecific changes in electrical activity of the brain.
• A head CT scan shows loss of tissue mass of affected lobes of the brain.
• A brain MRI can detect mild degrees of frontal and temporal atrophy missed by the head CT scan.
• Cerebrospinal fluid examination after a lumbar puncture is usually normal.

It is important to note that the definitive diagnosis can only be made with a brain biopsy. However, ongoing efforts are directed at developing a biological marker that would allow a firm diagnosis without the need for such an invasive procedure.

Treatment

There is no proven effective treatment for Pick's disease.

Depending on the symptoms and how bad the disease is, 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 an institutionalized care setting. Family counseling may help in coping with the changes required 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 communities, there may be access to support groups (such as the Alzheimer's - support group, elder care - support group, or others).

In some cases, cognitive function may be improved by stopping or changing medications that worsen confusion or that are not essential to the care of the person. This may include medications such as anticholinergics, analgesics, cimetidine, central nervous system depressants, and lidocaine.

Disorders that contribute to confusion should be treated. These may include heart failure, decreased oxygen (hypoxia), thyroid disorders, anemia

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 may 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 may 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.

Outlook (Prognosis)

The probable outcome is poor. The disorder progresses steadily and relatively rapidly. Total disability occurs early. Commonly, Pick's disease results in death within 2 to 10 years, usually from infection and occasionally from general failure of body systems.

Possible Complications

• loss of ability to care for self or perform normal activities
• progressive loss of ability to function
• loss of ability to interact with others
• infection
• reduced life span
• abuse by an over-stressed caregiver
• side effects of medications used to treat the disorder

When to Contact a Medical Professional

Call your health care provider if symptoms of Pick's disease develop.

Call your health care provider or go to the emergency room if acute deterioration in mental function occurs (this symptom may indicate development of another disorder).

Prevention

There is no known prevention for this rare disorder.

Moore DP, Jefferson JW. Handbook of Medical Psychiatry. 2nd ed. St. Louis, MO: Mosby; 2004.

Pierce JM. Pick's disease. J Neurol Neurosurg Psychiatry. 2003 Feb;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;15:1-5.

McKhann G, Albert M, Grossman M, Miller B, Dickson D, Trojanowski J. Clinical and pathological diagnosis of frontotemporal dementia. Arch Neurology. 2001;58:1803-1809.