Circumscribed brain atrophy

Alternative names
Lobar sclerosis; Pick’s disease; Fronto-temporal dementia; Arnold Pick’s disease; Focal cerebral atrophy

Definition

Pick’s disease produces deterioration in mental function caused by loss of brain tissue in discrete areas (focal lesions). It is marked by the presence of abnormalities in brain cells (Pick’s bodies) which can be found in the affected areas and elsewhere in the brain.

Causes, incidence, and risk factors

Pick’s disease is a rare disorder similar to senile dementia/Alzheimer’s type. What differentiates it from Alzheimer’s disease is that it seems to affect predominantly circumscribed areas of the brain, not all regions. Alzheimer’s disease is a more diffuse process that can affect any part of the brain as it progresses.

Pick’s disease affects about 1 out of 100,000 people. 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 onset is usually slow and insidious. The disorder involves shrinking of the tissues (atrophy) of the frontal and temporal lobes of the brain, “fronto-temporal dementia.” The neurons (nerve cells) in the affected areas contain abnormal material (Pick’s bodies). These are tangles made of tau protein. The exact cause is unknown.

The symptoms may be similar to Alzheimer’s, with aphasia (loss of language abilities), agnosia (loss of ability to recognize objects or people), and apraxia (loss of skilled movement abilities). Behavioral changes are prominent with loss of inhibition and change in personality, as opposed to Alzheimer’s Disease where memory loss is often the primary feature.

Risk factors may include a having a personal or family history of Pick’s disease or senile dementia, though the genetic basis of the disease has not yet been determined.

Symptoms
Movement/coordination difficulties (apraxia) - may be one of the earliest symptoms

Mood changes, personality changes:

     
  • striking loss of concern and lack of anxiety  
  • loss of initiative  
  • flat affect (does not display any emotion)  
  • indecision  
  • inappropriate mood  
  • lack of spontaneity  
  • decreased interest in daily living activities  
  • impaired judgment

Behavior changes:

     
  • excessive manual exploration of the environment  
  • withdrawal from social interaction  
  • inability to function or interact in social or personal situations  
  • inability to maintain employment  
  • decreased ability to function in self care  
  • behavior that is inappropriate relative to the environment

Language changes:

     
  • echolalia (repetition of anything spoken to the person)  
  • speech is incomprehensible jargon  
  • aphasia (decreased language ability, difficulty speaking or understanding speech)  
  • inability to comprehend speech  
  • difficulty finding a word  
  • shrinking vocabulary  
  • inability to speak  
  • inability to repeat a phrase  
  • poor enunciation  
  • decreased ability to read or write  
  • inability to name objects

Other changes:

     
  • loss of cognitive/intellectual skills  
  • specific disorders of problem solving/learning:       o inability to generalize       o loss of abstract thinking       o impaired calculating ability       o inability to learn  
  • weakness  
  • increased muscle tone  
  • urinary incontinence  
  • progressive dementia  
  • memory loss

Signs and tests

The health care provider bases the initial diagnosis on history and symptoms, signs, and tests, and by ruling out other causes of dementia including dementia due to metabolic causes. Neurologic examination may reveal signs that vary according to the which part of the brain is afffected. Temporal and frontal lobe signs are most common, with resulting behavioral and language changes.

There may be other abnormalities, including frontal release signs (presence of abnormal reflexes) and exacerbation of muscle stretch reflexes. Psychologic studies and tests of sensation, cognitive function, and motor function may be abnormal.

     
  • Neuropsychologic 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 by brain biopsy since the demonstration of Pick bodies must be done with a microscope by an experienced neuropathologist. 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. Monitoring and assistance with self-care may be required depending on symptoms exhibited and progression of the disorder.

Eventually, there may be a need for 24-hour care and monitoring at home or in an institutionalized care setting to provide a safe environment and meet the individual’s physiological needs.

Discontinuation or change in medications that worsen confusion or that are not essential to the care of the person may improve cognitive function. This may include medications such as anticholinergics, analgesics, cimetidine, central nervous system depressants, lidocaine, and other medications.

Disorders that contribute to confusion should be treated as appropriate. These may include heart failure, decreased oxygen (hypoxia), thyroid disorders, anemia, nutritional disorders, infections, and psychiatric conditions (such as depression). Correction of coexisting medical and psychiatric disorders often gives great improvement in mental function.

Medications may be required to control aggressive or agitated behaviors or behaviors that are dangerous to the person or to others.

Sensory function should be evaluated and augmented as needed by the use of hearing-aids, glasses, cataract surgery, etc.

Behavior modification may be helpful for some people in controlling unacceptable or dangerous behaviors. This consists of rewarding appropriate or positive behaviors and ignoring inappropriate behaviors (within the bounds of safety). Formal psychotherapy treatment is seldom effective, because it may overload limited cognitive resources. Reality orientation, with repeated reinforcement of environmental and other cues, may help reduce disorientation. 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).

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.

Expectations (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.

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

Calling your health care provider
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.

Johns Hopkins patient information

Last revised: December 3, 2012
by Gevorg A. Poghosian, Ph.D.

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