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About frontotemporal dementia

What is frontotemporal dementia?

  • 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 frontotemporal dementia?

Identifying precisely which diseases fall into the category of frontotemporal dementia presents a particular challenge to scientists. The signs and symptoms may vary greatly from one individual to the next. Researchers have identified several clusters of symptoms that tend to occur together and are dominant in subgroups of people with the disorder.

More than one symptom cluster may be apparent in the same person.

The signs and symptoms of frontotemporal dementia progressively worsen with time, almost always over years. Eventually, people require 24-hour care.

Behavioral changes

The most common signs and symptoms of frontotemporal dementia involve extreme changes in behavior and personality. These include:

  • Increasingly inappropriate actions
  • Loss of empathy and other interpersonal skills
  • Lack of judgment and inhibition
  • Apathy
  • Repetitive compulsive behavior
  • A decline in personal hygiene
  • Changes in eating habits, predominantly overeating
  • Oral exploration and consumption of inedible objects
  • Lack of awareness of thinking or behavioral changes

Speech and language problems

Some subtypes of frontotemporal dementia are marked by the impairment or loss of speech and language difficulties.

Two types of primary progressive aphasia are considered frontotemporal dementia. Primary progressive aphasia is characterized by an increasing difficulty in using and understanding written and spoken language. For example, people may have trouble finding the right word to use in speech or naming objects.

Semantic dementia is one type of primary progressive aphasia. It's also known as semantic variant primary progressive aphasia. Individuals with semantic dementia have prominent difficulty naming (anomia) and may replace a specific word with a more general word such as "it" for pen. They may also lose knowledge of word meaning.

Progressive agrammatic (nonfluent) aphasia is another type of primary progressive aphasia characterized by nonfluent and hesitant speech. Speech may sound telegraphic with misuse of pronouns and errors in sentence construction.

Movement disorders

Rarer subtypes of frontotemporal dementia are characterized by problems with movement, similar to those associated with Parkinson's disease or amyotrophic lateral sclerosis.

Movement-related signs and symptoms may include:

  • Tremor
  • Rigidity
  • Muscle spasms
  • Poor coordination
  • Difficulty swallowing
  • Muscle weakness

What are the causes for frontotemporal dementia?

In frontotemporal dementia, the frontal and temporal lobes of the brain shrink. Usually, the cause is unknown.

A variety of mutations on several different genes have been linked to specific subtypes of frontotemporal dementia. However, more than half the people who develop frontotemporal dementia have no family history of dementia.

Frontotemporal lobar degeneration is divided into two main subtypes, one involving the accumulation in the brain of a protein called tau and one involving the protein TDP-43.

In some cases, the affected parts of the brain contain microscopic abnormal tau protein-filled structures that develop within brain cells (Pick bodies). Frontotemporal dementia was once known as Pick's disease, but now that terminology is reserved for the subtype that actually features these abnormal structures.

Recently, researchers have confirmed shared genetics and molecular pathways between frontotemporal dementia and amyotrophic lateral sclerosis (ALS). The importance of these connection is currently under investigation.

What are the treatments for frontotemporal dementia?

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 frontotemporal dementia?

Your risk of developing frontotemporal dementia is higher if you have a family history of dementia. There are no other known risk factors.

 

Is there a cure/medications for frontotemporal dementia?

Frontotemporal dementia (FTD) is a neurodegenerative disorder that results in loss of memory and intellectual functions such as reasoning, abstract thinking, and executive abilities. It is the third most common cause of dementia and the second most common cause of early onset dementia in elderly persons older than 65 years. Possible causes are genetic mutations, thyroid diseases, and head trauma. There are three types of FTD: semantic variant, nontypical variant, primary progressive aphasia, and behavior variant. The prevalence of frontotemporal dementia in the age group of 60-69 years is 9 per 100,000. Treatment Management of patients with FTD requires a multidimensional approach. Treatments are of two categories: pharmacological and nonpharmacological. 1. Pharmacological treatment • Acetylcholinesterase and N-methyl-D-aspartate inhibitors may help with movement-related problems. • Selective serotonin reuptake inhibitors can improve certain behaviors but do not help cognition. • Selective dopaminergic antagonists can improve motivation and apathy. • Several disease-modifying drugs like salsalate (tau acetylation inhibitor) and Gosuraneb (anti-tau monoclonal antibodies) are under clinical trials. 2. Non Pharmacological treatment • Physical therapy helps with the difficulties of voluntary activities such as holding objects firmly. • Occupational therapy helps patients perform daily activities independently. • Speech therapy helps reduce problems like slurred speech and communication issues. • Cognitive behavioral therapy taps various aspects of behavioral abilities and their psychological angles. • Rehabilitation helps the person restore his characteristic abilities.

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