About pick's disease

What is pick's disease?

Frontotemporal dementia (FTD), or Pick's disease, is a syndrome featuring shrinking of the frontal and temporal anterior lobes of the brain.

  • The symptoms of frontotemporal dementia fall into two clinical patterns that involve either: (1) changes in behavior, or (2) problems with language.
  • Frontotemporal dementia is often inherited and runs in families.
  • There is no treatment for frontotemporal dementia and treatment is directed toward minimizing symptoms.
  • Frontotemporal dementia progresses steadily and often rapidly and is fatal. The duration of disease ranges from less than 2 years in some individuals to more than 10 years in others

What is frontotemporal dementia (Pick's disease)?

Frontotemporal dementia (FTD) describes a clinical syndrome associated with shrinking of the frontal and temporal anterior lobes of the brain. Originally known as Pick's disease, the name and classification of frontotemporal dementia has been a topic of discussion for over a century. The current designation of the syndrome groups together Pick's disease, primary progressive aphasia, and semantic dementia as frontotemporal dementia. Some doctors propose adding corticobasal degeneration and progressive supranuclear palsy to frontotemporal dementia and calling the group Pick Complex. These designations will continue to be debated.

What are the symptoms for pick's disease?

The three main types of Niemann-Pick are types A, B and C. The signs and symptoms you experience depend on the type and severity of your condition. Some infants with type A will show signs and symptoms within the first few months of life. Those with type B may not show signs for years and have a better chance of surviving to adulthood. People with type C may not experience any symptoms until adulthood.

What are the causes for pick's disease?

Niemann-Pick is caused by mutations in specific genes related to how the body metabolizes fat (cholesterol and lipids). The Niemann-Pick gene mutations are passed from parents to children in a pattern called autosomal recessive inheritance. This means that both the mother and the father must pass on the defective form of the gene for the child to be affected.

Niemann-Pick is a progressive disease, and there is no cure. It can occur at any age.

Types of Niemann-Pick

Types A and B

Types A and B are caused by a missing or malfunctioning enzyme called sphingomyelinase. This affects the body's ability to metabolize fat (cholesterol and lipids), resulting in a buildup of fat in cells. This causes cell dysfunction and, over time, cell death. Type A occurs mainly in infants, who show severe, progressive brain disease. There is no cure, so most children do not live beyond their first few years. Type B usually occurs later in childhood and is not associated with primary brain disease. Most people affected with type B survive into adulthood.

Type C

Niemann-Pick type C is a rare inherited disease. The genetic mutations of this type cause cholesterol and other fats to accumulate in the liver, spleen or lungs. The brain is eventually affected too.

What are the treatments for pick's disease?

No treatment has been shown to slow the progression of frontotemporal dementia. Behavior modification may help control unacceptable or dangerous behaviors. Aggressive, agitated, or dangerous behaviors could require medication. Antidepressants have been shown to improve some symptoms.

What are the risk factors for pick's disease?

Pick's disease, also known as frontotemporal dementia, is the most common cause of dementia in patients under 60 and the third most common cause in patients over 65. It presents with neuropsychiatric symptoms that affect the patient's personality and those that cropped cognitive functions. It causes the degeneration of frontal and temporal cortices that reflects behavioral changes and language deficits. It is a spectrum of dementia that can be associated with other neurological faculties, such as motor abilities.

Risk factors
Pick's disease is highly heritable. Studies show up to 40 percent of patients inherit dementia. Hence, assessing the patients for the characteristic signs and symptoms may help them identify the hints of the disease development in descendants of the patients.
1. A family history of genetic factors for the disease is a potential risk. Mutations in C9ORF72, microtubule-associated protein tau (MAPT), and progranulin (GRN) are the prominent genetic factors to cause the disease. These mutations can pass from generation to generation in an autosomal dominant fashion.
2. Few studies have identified epigenetic or environmental risk factors. They include diabetes mellitus, a history of head injury, and autoimmune disorders.
3. Though medical professionals commonly propose obesity, smoking, history of cerebrovascular injuries, and hypertension to pose risks, further study is required to establish them.

Behavioral variant: Perseverative, stereotyped, or compulsive behaviors manifest as repetitive movements, compulsive and ritualistic routines, stereotyped speech, hyperorality and dietary changes. Socially inappropriate behavior, loss of social decorum, impulsive, and careless actions,Language variant: impaired object knowledge, surface dyslexia, and dysgraphia, spared repetition, anomia, impaired single-word comprehension, and impaired complex sentence understanding
Frontotemporal dementia,Neurodegenerative disease
Low-dose atypical anti-psychotic drugs for aggressive behavior

Is there a cure/medications for pick's disease?

Type A

Some children with the type A form of Niemann-Pick live to the age of 4. However, a majority of children with this type die in infancy.

Type B

Those with Niemann-Pick type B may survive into late childhood or early adulthood, but they often have health problems such as respiratory failure or complications related to an enlarged liver or spleen.

Type C

Niemann-Pick type C is always fatal. However, life expectancy depends on when symptoms begin. If symptoms appear in infancy, your child isn’t likely to live past the age of 5. If symptoms appear after 5 years of age, your child is likely to live until about 20 years of age. Each person may have a slightly different outlook depending on their symptoms and the severity of the disease.

Whichever type of Niemann-Pick disease you or your child may have, the sooner it’s recognized and diagnosed, the better. Research on the disease continues, and scientists are working toward more effective management and treatment of the disease.

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