What are the other Names for this Condition? (Also known as/Synonyms)

• Cerebral Atrophy Diffuse (due to Pick’s Disease)
• Lobar Atrophy of the Brain (due to Pick’s Disease)
• Pick’s Disease of Brain

What is Pick's Disease? (Definition/Background Information)

• Pick’s Disease (PiD) occurs due to the accumulation of a type of protein in the frontotemporal regions of brain, resulting in (sometimes) sudden and steadily worsening indications of personality changes and behavior pattern, affecting thinking, besides inducing memory loss
• PiD is a highly infrequent, terminal disorder. It is one of the many disorders that are directly responsible for causing frontotemporal dementia. The condition causes irreversible withering (atrophy) of the affected brain area
• Individuals with a family history of frontotemporal dementia, may inherit the condition in an autosomal dominant manner
• Pick’s Disease does not respond to any treatment, and with a steady decline of the individual’s health and mental status; the course and outcome of the disorder is often grim

Who gets Pick's Disease? (Age and Sex Distribution)

• Pick’s Disease affects adults between the ages 40-60 years (average age being 54 years). It is rare below this age range, though adults as young as 20 years, are known to be affected
• Generally, PiD affects the male sex slightly more than the female sex
• No particular race or ethnic preference has been observed. However, the northern Europe regions of Sweden, Norway, and Denmark, show a higher prevalence of PiD

What are the Risk Factors for Pick's Disease? (Predisposing Factors)

Risk factors for Pick’s Disease are unknown, since the condition is not yet completely understood. However, the following factors are thought to play a role:

• An advancing age; this factor may enhance the risk
• Genetic causes, family history of PiD; though these factors are still being researched

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Pick's Disease? (Etiology)

• Pick’s Disease develops on account of mass formation of unusually large quantities of an abnormal form of protein (called tau-protein), in the frontotemporal region nerve cells of the brain
• These protein formations are termed as Pick’s bodies and they are observed in Pick cells. The reason behind this occurrence has not been medically understood
• The progression of the disease may cause the nerve cells (neurons) to lose their function over many years. Sometimes, a sudden advancement of the condition may occur, where more and more neurons die increasingly faster, causing a kind of brain shrinkage (cerebral atrophy)
• Individuals with a family history of frontotemporal lobar degeneration (due to Pick’s Disease) may pass on the anomalous genes to their offspring in an autosomal dominant condition, 50% of the time. However, Pick’s Disease is responsible for only 5% of all the frontotemporal dementia cases

What are the Signs and Symptoms of Pick's Disease?

The presentations of Pick’s Disease may be initially mild, but they deteriorate quickly. Dementia occurs inevitably as a result of PiD. The key signs and symptoms include:

• Extremely irrational mental/emotional/physical behavior (may be completely inappropriate for the situation); lack of control and awareness, sexual hyperactivity, or absence of sexual drive, tendency to roam/wander away
• Complete loss of social abilities, social awkwardness, and withdrawal
• Changes in overall personality; regression or absence of reasoning/rationale, agitation, delusions, depression, aggression
• Progressive deterioration of the senses, memory loss, communication difficulties, incoherence (difficulty speaking or unable to speak)
• Muscle rigidity, contraction, difficulty walking, maintaining balance, performing basic and routine activities becomes very challenging; loss of basic motor (physical and spatial) skills

How is Pick's Disease Diagnosed?

To examine for Pick’s Disease, a variety of tests are usually conducted. The evaluation is usually based on the set of signs and symptoms presented. Frequently, PiD is confused with dementia caused by Alzheimer’s, or other such disorders. The main diagnostic tools include:

• Physical exam with a comprehensive evaluation of medical history
• Neurological and cognitive assessment: Checking intellectual ability, memory, mental health and function, language skills, judgment and reasoning, coordination and balance, reflexes, sensory perceptions (space, sight, hearing, touch)
• Psychiatric examination
• Imaging studies performed are: MRI scan of the CNS (brain and spine), CT scan (head), PET imaging
• Electroencephalogram (EEG), cerebrospinal fluid analysis
• Brain biopsy; required to conclude on the study analysis

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Pick's Disease?

Complications with Pick’s Disease depend on many factors, such as the severity of the condition, age, and overall health of the individual. These include:

• The main complication, which occurs on account of memory loss and neurological function impairment, is that institutionalized care might be required for prolonged periods, or for the rest of an individual’s life. This is due to complete degeneration of mental and emotional health, since there is a progressive inability of individuals to take care of themselves
• Loss of inhibition, spontaneous incontinence (involuntary bowel or bladder movement)
• Loss of sleep, decreased level of personal safety due to lack of awareness, prone to fall-related injuries
• The individual may become susceptible to opportunistic infections; organ failure chances are higher; due to a reduced sense of hygiene, decreased intake of food/nutrition. Often, these factors  lead to an overall reduced lifespan
• An individual may have persistent pain, which is often under-treated due to lack of good communication between the individual and their healthcare providers
• Feeding and swallowing problems; food may get blocked in the airways/lungs resulting in pneumonia, choking
• Treatment medication may have significant side effects

How is Pick's Disease Treated?

There is no standard cure or treatment of the condition. Patients receive supportive care and may be given medications to control abnormal spasmodic movements and pain, if any present. Pick’s Disease management is undertaken on a case-by-case basis. Some of the methods include:

• Addressing general health problems that are mostly linked to mental balance and well-being. This includes the treatment of anemia, dietary or vitamin deficiencies, heart-related ailments, infections, thyroid disorders, hormonal imbalances, and regulating blood pressure
• Treatment of the underlying illness, to prevent progression of Dementia
• Ensuring that the individual has a nutritious diet with medical supplements (balanced diet that include proteins, fiber-rich fruits and vegetables, milk shakes, juices, and plenty of water)
• Ensuring that the individual is well-cared for in a safe environment (at home, or outside) and is under regular (and maybe constant) care and support
• Use of medications to control behavioral issues and prevent further loss of mental stability, like stimulants, mood stabilizers, cholinesterase inhibitors, NMDA receptor antagonists, antipsychotic drugs, sedatives, anti-depressants (selective serotonin reuptake inhibitors), etc. Many of these medications may cause side effects like nausea, stomach problems, drowsiness, and vomiting
• Physiotherapy, exercises, to help strengthen the muscles; speech therapy to help muscles in the neck, throat have control of oral activities like chewing, swallowing, and speaking
• Home and work environment can be suitably modified to make it a lot safer and convenient for performing regular activities. This includes safety features normally designed for the physically challenged or the elderly, furniture rearrangement, and use of ramps
• Wheelchairs, walkers, braces are used to provide physical support and promote mobility
• Hospitalization may be required (for short or long duration), depending on the condition of the individual. This is usually followed by regular check-ups

A healthcare provider may utilize the following treatment measures on a case-by-case basis. These are meant to provide mental and emotional support, and develop or retain communication skills:

• Psychiatric treatment
• Group therapy interactive sessions
• Support groups

How can Pick's Disease be Prevented?

Current medical research has not indicated effective preventive measures for Pick's Disease. Nevertheless, in almost all cases of the disorder with irreversible factors; the progression of the condition can be delayed or controlled, to some extent, by ensuring that:

• Factors associated with diet, lifestyle, and health in general, like alcoholism, smoking, obesity, cholesterol levels, stress, nutritional deficiencies, hormonal problems, use of certain medications, diabetes, etc. may be regulated and/or controlled
• Any injuries to the brain, or the presence of brain tumors, have to be treated immediately and adequate follow-up maintained. There is a possibility that these may significantly contribute to Dementia development, in the future
• Individuals who fall in the high risk category (and all others over the age of 50 years) should be encouraged to be socially active and physically fit (with regular exercising). A healthy diet rich in fruits, vegetables, fish, whole grains, and low in fats and sugar, is recommended
• High-risk individuals should constantly maintain a healthy mind, by undertaking mentally stimulating activities like; reading/writing/teaching, solving puzzles, playing video games, listening to music, learning new skills (painting, language, playing an instrument), etc.

What is the Prognosis of Pick's Disease? (Outcomes/Resolutions)

The prognosis for an individual with Pick’s Disease is normally grim. But, the outcome is also based on a combination of many factors, which include:

• Age of the adult
• Primary signs and symptoms observed; individuals in whom key signs are disturbed speech and impaired communication skills, generally live longer than those, in whom serious behavior problems are manifested
• Degree of severity; often rapidly progressing PiD bring about a speedy decline in the condition
• General health and medical history
• Tolerance level/health of the individual, when subjected to various medications; response to dementia management
• Complications observed, if any

An early detection and aggressive management of Pick’s Disease should be attempted. This may prevent a rapid deterioration of the condition, and help sustain the quality of life, for some more additional time.

Patients diagnosed with PiD may live anywhere between 2-10 years following the onset of symptoms. Death usually results from infections, or failure of vital organs.

Additional and Relevant Useful Information for Pick's Disease:

There are voluntary organizations and support groups for Dementia (due to Pick’s Disease) that provides counsel, help, and understanding, to the affected individuals and their families.