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Frontotemporal Dementia

Frontotemporal dementia (FTD) refers to a group of disorders that result in progressive nerve cell deterioration of the frontal and temporal lobes of the brain. The name “frontotemporal” is applicable as it describes the areas of the brain that are most affected. FTD was once commonly referred to as Pick’s disease, although it actually encompasses a wide range of rare brain disorders that cause nerve cell damage leading to the loss of multiple cognitive functions controlled by these brain regions. Other names for this disorder include frontal lobe disorders, frontotemporal degenerations and frontotemporal disorders.

Similarities exist between FTD and Alzheimer’s disease. One of the key differences between the two, however, is the age at which symptoms present. FTD symptoms generally begin between the ages of 45 and 65, whereas, Alzheimer’s symptoms typically don’t manifest prior to the age of 65.

Frontotemporal deterioration can be the result of several dementia variations, including Pick’s disease, corticobasal degeneration and progressive aphasia. FTD disorders fall into three subtypes:

The first is behavior variant frontotemporal dementia (bvFTD). In bvFTD, brain cell damage is most prominent in areas that control behavior, judgment, foresight, empathy and other abilities resulting in pronounced changes in personality, conduct and interpersonal relationships.

In the second, primary progressive aphasia (PPA), brain cell deterioration affects speaking, language skills, writing and comprehension skills.

The third causes disturbances of motor (movement or muscle) function but may or may not also affect language (PPA) or behavior (bvFTD). This FTD spectrum itself has three disorders:

  • Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig’s disease, results in muscle wasting away and weakness
  • Corticobasal syndrome which produces stiffness and incoordination of the arms and legs
  • Progressive supranuclear palsy (PSP) produces muscle stiffness, changes in posture, walking difficulties and affects eye movements

Symptoms of Frontotemporal Dementia

Common symptoms of frontotemporal dementia include:

  • Memory loss (some memory loss is common as we age, but if memory loss causes concern, see your doctor)
  • Lack of inhibition
  • Socially inappropriate behaviors
  • Lack or loss of empathy
  • Speech difficulties
  • Compulsive or repetitive behaviors
  • Balance issues
  • Abrupt or frequent mood swings
  • Problems with concentration or in planning

Primary progressive aphasia has two distinct forms which have different symptoms. In the semantic variant of PPA, a person loses their ability to formulate words or understand spoken words. In the nonfluent or agrammatic variant of PPA, a person’s speech is very hesitant, ungrammatical and/or labored.

Brain or Physical Changes Associated with Frontotemporal Dementia

As brain cells deteriorate and die in the frontal and temporal lobes of the brain, the pathways within the brain change, resulting in brain signals becoming lost. This causes the resulting symptoms of the disease. As more brain cells die, the size of the frontal and temporal lobes is reduced.

Causes/Risk Factors of Frontotemporal Dementia

Family history or the presence of a similar disease is the only known risk factor for FTD. Almost one-third of all FTD cases are inherited.

Diagnosis, Treatment and Care of Frontotemporal Dementia

Diagnosis is best made by a medical professional who is familiar with frontotemporal disorders. To diagnose FTD, the medical professional will generally:

  • Take a full patient history
  • Perform a neurological examination
  • Order a magnetic resonance imagining (MRI)
  • Order a glucose positron emission tomography (PET) scan

Currently, there are no specific treatments for frontotemporal disorders. Once a diagnosis has been made, speech and language therapies can be beneficial, as well as putting behavioral management strategies into place. Medications can be used to reduce depression, irritability and agitation.

FTD progressively worsens over time, but the speed of decline varies from person to person. The disease may progress for years before physical changes cause skin, lung and/or urinary tract infections which often become the cause of death.

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