A rare type of neurodegenerative disease, Pick’s disease (also known as Pick disease or PiD) is a kind of frontotemporal dementia that is caused by the destruction of specific nerve cells in the brain. It typically presents among patients as language problems, difficulty with social functioning, and cognitive impairment.
Definition & Facts
Pick’s disease is characterized by a buildup of tau proteins in the neurons of the brain. The buildup of these proteins creates silver clusters that are referred to a pick bodies. While the term Pick’s disease is sometimes used in a general sense to describe any condition relating to frontotemporal lobar degeneration, Pick's disease more specifically refers to a condition of localized dementia caused by pick bodies.
Pick’s disease affects less than 200,000 people in the United States. Worldwide, the incidences of Pick's disease tend to be higher in Northern European and specifically in Scandinavian countries, where its statistical frequency ranges between 7 and 43 cases out of every 100,000 people in the population.
Similar to other forms of dementia, Pick's disease progresses slowly. Those diagnosed with Pick's live on average for about six years following diagnosis.
Symptoms & Complaints
Though similar to Alzheimer’s disease, Pick's disease is typically identified differently due to changes in the patient’s personality and social interactions. Disturbing symptoms arise which pertain to social conduct. Symptoms typically include the following:
- Language and speech disorders
- Increased desire to dissociate from family
- Changes in typical behavior
- Irrational anxiety or fear
- Compulsive buying disorder
- Difficulty with proper social conduct (vulgar language, tactlessness, disinhibition, misperception)
- Having little or no motivation
- Pacing or wandering
- Urinary incontinence
Pick's disease is caused by the buildup of tau proteins in the neurons of the brain. This buildup inside the cells results in inflammation and destruction of the cells as a result of the response of the immune system. The tau protein buildup is related to a variety of genetic mutations. Statistics indicate that 50% of persons who are diagnosed with Pick's disease have a family history of frontotemporal dementia. Additionally, some linkage has been found between Pick's disease and the mutation or degeneration of three particular chromosomes, but the role they play in causing the disease is not yet identified.
Diagnosis & Tests
The doctor will inquire about family history and medical history. As with the diagnosis of most medical conditions, Pick’s is identified by a battery of tests, which rule out other types of dementia and zero in on the specific portion of the brain that is being affected. The following tests are typically ordered in the process of identifying the proper diagnosis of the disease:
- Neuropsychological assessment, which is an assessment of both the mental faculties and behavior of the patient.
- A magnetic resonance imaging (MRI) of the brain
- A computed tomography (CT) scan of the head in order to assess brain damage
- A positron emission tomography (PET) scan which is another type of medical imaging exam which can reveal the metabolic activity of the brain
- Electroencephalogram (EEG) which measures the electrical activity of the brain
- Neurological examination of the brain and central nervous system which measures sensory and motor responses as well as a patient's balance, coordination, and mental status
- A lumbar puncture (also known as a spinal tap) from which cerebrospinal fluid is examined
Once Pick's disease is identified as a possible cause, a brain biopsy, which will be examined for the presence of pick bodies, may be ordered to confirm the diagnosis.
Treatment & Therapy
There is no cure for Pick's disease, and treatments and therapies for patients with Pick’s are directed at alleviating or lessening the effects of the symptoms. Occupational therapy and speech therapy may be helpful in addressing a patient's problems with everyday functioning and with verbal and written communication, respectively.
Since many of the symptoms are related to behavioral disorders, talk therapy (psychotherapy) is often mistaken as the sole means of treating patients with Pick's disease. However, it can still be a valuable tool in helping a patient cope with the challenges of their disorder. Behavior modification approaches, which reward good behavior, are sometimes but not always effective.
Reality orientation therapy, which makes use of environment cues, is sometimes effective in helping to alleviate disorientation among those with dementia. Regular exercise, regular sleep, and healthy diet may promote cognitive functioning. Medications that can be utilized in the treatment of Pick's disease include:
- Analgesics which are pain medications
- Anticholinergics which inhibits the nerve impulses of acetylcholine, a type of neurotransmitter
Depending upon how the patient responds, some of these medications can worsen confusion; therefore, it is important to find a regimen that works. Additional treatments may include:
- Iron supplements to combat anemia
- Oxygen therapy for hypoxia
- Prompt treatment of any infections
- Guarding against acute kidney failure and/or acute liver failure
- Maintaining proper nutrition
- Treatment of thyroid disorders
- Treatment of mood disorders, such as depression or other negative emotions such as aggression or agitation.
It is often critical for patients with Pick's disease to be closely monitored and assisted in hygiene and dietary functions. As the disease progresses, care facilities and home health care services are often needed.
Prevention & Prophylaxis
- Eating a well-balanced diet
- Maintaining a healthy weight
- Regular exercise
- Avoiding excessive alcohol consumption
- Quitting smoking or never beginning to smoke and avoiding secondhand smoke
- Maintaining a healthy blood pressure
Though there is no guarantee that these factors will help prevent Pick’s disease, these healthy habits will certainly help a person's overall well-being.