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Frontotemporal Dementia

Definition


Disease: Frontotemporal Dementia Frontotemporal Dementia
Category: Neurological diseases
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Disease Definition:

Also called frontotemporal lobar degeneration, fontotemporal dementia is a general term for a diverse group of uncommon disorders that primarily affect the frontal and temporal lobes of the brain. These are the areas that are generally associated with language, behavior and personality.

 

Portions of these lobes waste away (atrophy) or shrink in frontotemporal dementia. Depending on the portion of the brain that is affected, the signs and symptoms of this condition could vary. In some cases, people with frontotemporal dementia lose their ability to use and understand language and in other cases, they undergo dramatic changes in their personality and become impulsive, emotionally blunted or socially inappropriate.

 

Usually, frontoterporal dementia is misdiagnosed as Alzheimer's disease or a psychiatric problem. In most cases, frontotemporal dementia occurs between the ages of 40 and 70, while Alzheimer's disease occurs later in life.
 

Work Group:


Prepared by: Scientific Section

Symptoms, Causes

Symptoms:

It is quite difficult for scientists to identify exactly which diseases fall into the catergory of frontotemporal dementia. The signs and symptoms could vary greatly from one person to another. Several clusters of symptoms have been identified which occur together and be dominant in subgroups of people with the disorder. In the same person, more than one cluster could be apparent.

 

BEHAVIORAL CHANGES:

Extreme changes in behavior and personality are the most common signs and symptoms of frontotemporal dementia, such as:

 

  • Euphoria
  • Apathy
  • A decline in personal hygiene
  • Increasingly inappropriate actions
  • Lack of judgment and inhibition
  • Lack of awareness of behavioral changes or thinking
  • Repetitive compulsive behavior

 

SPEECH AND LANGUAGE PROBLEMS:

The impairment or loss of speech and linguistic abilities are what some subtypes of frontotemporal dementia are marked by. Some people with semantic dementia, which is a subtype of frontotemporal dementia, usually utter grammatically correct speech, which has nothing to do with the conversation at hand. People with primary progressive aphasia find using and understanding written and spoken language very difficult.

 

MOVEMENT DISORDERS:

Problems with movement, which could be found in Parkinson's disease or amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease, are problems that some rare subtypes of frontotemporal dementia are characterized by. These are some of the signs and symptoms which are related to movements:

 

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

Causes:

Some specific subtypes of frontotemporal dementia have been linked with a variety of mutations on several different genes. However, no family history of dementia could be found in more than half the people with frontotemporal dementia.

 

The affected parts of the brain contain microscopic Pick bodies in some cases of frontotemporal dementia, which are abnormal protein-filled structures that develop within brain cells. The term Pick's disease is reserved for the subtype that actually features these abnormal structures; however, in the past, frontotemporal dementia as a whole was called Pick's disease.
 

Complications

Complications:

Almost always over years, the signs and symptoms of frontotemporal dementia progressively worsen, and eventually require 24-hour care.
 

Treatments:

Frontotemporal dementia has no cure and no effective way of slowing its progression. Managing the symptoms is what treatment for this condition depends on.

 

MEDICATIONS:

 
Antipsychotics:

These drugs are used in some cases to combat the behavioral problems of frontotemporal dementia. An increased risk of mortality in older people is one of their serious side effects.

 

Antidepressants:

The behavioral problems that are associated with frontotemporal dementia could be reduced by some types of antidepressants, such as trazodone. Despite the fact that study results have been mixed, but in some cases, SSRIs (selective serotonin reuptake inhibitors) have also been effective.

 

THERAPY:

In order to learn alternate strategies for communication, people that are having difficulties with language could benefit from speech therapy.
 

Prognosis:

Not available

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