Frontotemporal Dementia

What is frontotemporal dementia?

Frontotemporal dementia (FTD), also called frontotemporal degeneration, is an uncommon neurodegenerative disease characterized by behavioral and language disturbances. When behavioral disturbances are the most prominent early symptoms in a patient, we refer to the condition as behavioral-variant FD (bvFTD). When disruption of language is the most prominent early symptom, we use the term primary progressive aphasia (PPA).

On average, symptoms of frontotemporal dementia begin in the 50s or early 60s, although  symptoms have been seen in patients as young as 20 and in others over 80 .

Additional information related to clinical manifestations, information for caregivers, recent research findings, patient advocacy and links to other Internet-based resources may be obtained through the Association for Frontotemporal Degeneration:

E-mail: info@ftd-picks.org
Website: http://www.ftd-picks.org/
Telephone: (866) 507-7222

What are the symptoms of frontotemporal dementia?

The frontal lobe of the brain, which has connections with many different regions in the brain, helps to direct and maintain our attention and concentration. It is important in our logical reasoning and judgment. It helps us inhibit socially improper words and actions, provides our internal sense of motivation, and regulates our emotions.

Damage to the frontal lobe of the brain may impact any or all of these important functions. Common symptoms include dramatic changes in behavior and personality. These may include:

  • An increased tendency to make socially inappropriate comments or actions
  • Decreased empathy, or new difficulties understanding how one’s actions may impact others
  • Difficulties with logical judgments or understanding the relationship between cause and effect
  • Changes in sexual behaviors
  • Aggressive behaviors or actions
  • Decline in personal hygiene, toileting habits, etc.
  • Severe mental rigidity
  • Language abnormalities, such as being unable to express language, find words or understand the meaning of words
  • Inattention, increased distractibility or a tendency to jump from one topic to another
  • Difficulty initiating or completing tasks
  • Significant changes in eating patterns

In addition to cognitive impairments, neurologic manifestations may occur, although these typically manifest later in the disease. These include:

  • Parkinsonism: slowness of movement (bradykinesia), increased rigidity in the arms and/or legs, problems with walking (short stride length or a “shuffling” gait)
  • Tremor
  • Muscle spasms and/or rippling of the muscles underneath the skin
  • Seizures

What causes frontotemporal dementia?

Frontotemporal dementia is a neurodegenerative disease that affects the brain. In some people, this is related to abnormal biochemical processing and resulting deposition of a protein called tau, while in others it is related to a protein referred to as TDP-43.

The majority of cases of frontotemporal dementia are sporadic, meaning that the disease is neither inherited nor caused by a genetic mutation.

Approximately 10 percent of cases are associated with a known genetic mutation, either in the gene encoding the microtubule associated protein tau (MAPT) or in the gene encoding progranulin.

About 20 percent of individuals affected by frontotemporal dementia have a family history of neurodegenerative disease, such as ALS (Lou Gehrig’s disease), Alzheimer’s disease or Parkinson’s disease. These individuals may have some increased hereditary predisposition for developing a neurodegenerative disease.

What are risk factors for frontotemporal dementia?

Having a family history of frontotemporal dementia is associated with an increased risk of developing the disease. There are no other known risk factors for frontotemporal dementia.

How is frontotemporal dementia diagnosed?

Historically, frontotemporal dementia has been difficult to distinguish from Alzheimer’s disease or from psychiatric disease.

As our clinical and medical understanding of frontotemporal dementia has improved, important distinctions have become more widely appreciated. There is increased recognition that:

  • behavioral symptoms present early in the course of frontotemporal dementia are not typically present early in Alzheimer’s disease; and
  • memory deficits that typically represent the earliest deficits of Alzheimer’s disease are not typical of early frontotemporal dementia.

An MRI scan of the brain’s structure may identify atrophy (shrinking) in parts of the brain characteristic of frontotemporal dementia. Functional brain imaging with FDG-PET or SPECT may also provide corroborative evidence to support the diagnosis.

Laboratory investigations, cerebrospinal fluid analysis, EEG or other testing may be recommended to exclude other medical or neurologic conditions. These tests may or may not be recommended, depending upon an individual’s clinical presentation. If ALS (Lou Gehrig’s disease) is suspected, electromyography (EMG) may be recommended to confirm this diagnosis.

What treatments exist for frontotemporal dementia?

There is no cure for frontotemporal dementia. Treatment focuses on medications that may alleviate the severity of symptoms associated with the disease and help ensure the safety of patients and those around them.

Trials of medicines typically used for Alzheimer’s disease have not demonstrated a definite benefit for people with frontotemporal dementia.