What is Pick's Disease?

Pick’s disease, also known as Pick disease or PiD, is a rare neurodegenerative disorder involving the progressive destruction of brain cells. It is a type of frontotemporal dementia and is characterized by symptoms of dementia and aphasia.

Causes

Pick’s disease occurs due to changes to the neurons in certain areas of the brain. Tau proteins build up in the neurons, leading to the formation of silver, spherical aggregations known as Pick bodies or Pick cells. This protein usually exists in healthy brain cells, but individuals with Pick’s disease have abnormal quantity or type of the protein.

The origin of the abnormalities in the tau protein in not currently known. Several genes have been associated with a higher risk of Pick’s disease and may have an effect on the protein, but the research on this subject is not clear.

Symptoms

Picks disease can affect adults of all ages, with reported cases as young as twenty. It most commonly presents, however, in patients aged between 40 and 60.

There is a wide range of symptoms associated with the disease, including:

• Behavior changes: compulsiveness, inappropriateness, withdrawal
• Emotional changes: mood swings, disinterest, lack of empathy
• Language changes: aphasia, uncoordinated sounds, inability to write
• Neurologic changes: difficulty making decisions and with complex tasks

In the early stages of the disease, it can be difficult to distinguish Pick’s disease from Alzheimer’s disease. The main differential sign is memory loss, which occurs earlier in Alzheimer’s disease and slowly progresses in Pick’s disease.

In most cases, the symptoms of the disease steadily get worse as it progresses, and it usually becomes fatal within two to ten years of diagnosis.

Diagnosis

Initially, a thorough medical history should be taken, including questions about a family history of Pick’s disease.

The description of signs and symptoms are the most important factors to be considered. Diagnostic testing can then be utilized to differentiate the disease from other conditions with similar symptoms, such as Alzheimers disease.

The following tests may be required in the diagnosis of Pick’s disease:

• Neuropsychological assessment
• Magnetic resonance imaging (MRI) of the brain
• Electroencephalogram (EEG)
• Neurological exam
• Cerebrospinal fluid examination
• Computed tomography (CT) scan of the head
• Cognitive function tests.

However, final diagnosis can only be confirmed with a brain biopsy that is analyzed for the presence of Pick bodies.

Treatment

Although there is no cure for Pick’s disease, treatments are available to manage the symptoms of the disease.

It is common for patients to exhibit agitated and aggressive behavior, which can be difficult for carers to handle. Behavior modification by rewarding positive behaviors is sometimes successful and counseling of the patient may be beneficial. However, pharmacological intervention with psychotherapy depressants is often needed to control aggressive behaviors.

It should be noted that stopping or changing the medication regimen regularly is not recommended, as it can lead to increased confusion and worsening of symptoms.

Treatment is highly individualized to meet the needs of the patient. In the vast majority of cases, they will eventually need ongoing 24-hour care to help manage the symptoms and monitor the progression of the disease.

References

• http://www.ninds.nih.gov/disorders/picks/picks.htm
• http://emedicine.medscape.com/article/1135504-overview
• http://www.healthline.com/health/picks-disease#Overview1
• http://www.nytimes.com/health/guides/disease/picks-disease/overview.html