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Pick disease

Semantic dementia; Dementia - semantic; Frontotemporal dementia; FTD; Arnold Pick disease; 3R tauopathy

 

Pick disease is a rare form of dementia that is similar to Alzheimer disease , except that it tends to affect only certain areas of the brain.

Causes

 

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.

 

Symptoms

 

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:

BEHAVIORAL CHANGES:

  • 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

EMOTIONAL CHANGES

  • 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

LANGUAGE CHANGES

  • 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)
  • Weakness

OTHER PROBLEMS

  • Urinary incontinence

 

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.

 

Treatment

 

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:

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

It is important to treat any disorders that can cause confusion. These include:

  • Anemia
  • Decreased oxygen ( hypoxia ) level
  • Heart failure
  • High carbon dioxide level
  • Infections
  • 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
  • Homemakers
  • 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.

 

Support Groups

 

Some communities may have support groups, such as for Alzheimer disease and elder care .

 

Outlook (Prognosis)

 

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.

 

Prevention

 

There is no known prevention.

 

 

Open References

References

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.

 
  • Central nervous system

    Central nervous system - illustration

    The central nervous system is comprised of the brain and spinal cord. The peripheral nervous system includes all peripheral nerves.

    Central nervous system

    illustration

    • Central nervous system

      Central nervous system - illustration

      The central nervous system is comprised of the brain and spinal cord. The peripheral nervous system includes all peripheral nerves.

      Central nervous system

      illustration

    A Closer Look

     

      Talking to your MD

       

        Self Care

         

          Tests for Pick disease

           
             

            Review Date: 2/27/2016

            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.

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