Frontotemporal dementia

Frontotemporal dementia tends to occur at a younger age than Alzheimer's disease and can affect both men and women. The average length of the disease can vary. This type of dementia resembles Alzheimer's disease in that it also involves a progressive degeneration of brain cells that is irreversible.

With this form of dementia, a person may have symptoms such as sudden onset of memory loss, behaviour changes, or difficulties with speech and movement.

Unlike Alzheimer's disease, which generally affects most areas of the brain, frontotemporal dementia is an umbrella term for a group of rare disorders that primarily affect the frontal and temporal lobes of the brain – the areas generally associated with personality and behaviour. In some cases, brain cells in these areas shrink or die. In other cases, the brain cells in these areas get larger, containing round, silver "Pick's bodies". Pick's disease refers to a subtype of frontotemporal dementia that has these specific abnormalities. In frontotemporal dementia, the changes in the brain affect the person's ability to function. Researchers estimate that approximately two to five per cent of all dementia cases are frontotemporal dementia.

Other names often used for frontotemporal dementia, in addition to Pick's disease, include:

  • Semantic dementia
  • Frontal Lobe dementia
  • Primary progressive aphasia
  • Corticobasal degeneration
  • Pick's complex

Symptoms of frontotemporal dementia

Since the frontal and temporal areas of the brain can be affected, early symptoms often affect either behaviour and/or speech (language).

  • Changes in behaviour may include becoming either withdrawn or disinhibited (e.g., losing the ability to restrain one's behaviour and actions). The person may lose interest in personal hygiene, become easily distracted or repeat the same action over and over again. Overeating or compulsively putting objects in the mouth may occur. Sometimes incontinence is an early symptom of the disease.

Other possible changes in behaviour include

    • Inappropriate social behaviour, e.g. they may say inappropriate things or come across as rude or tactless
    • Distractability
    • Loss of insight into the behaviours of oneself and others (making it seem as if they don’t care)
    • Changes in food preferences
    • Blunted emotions
    • Decreased energy and motivation
    • Changes in personality, e.g. more outgoing people may become quieter and quiet people may become more extroverted

  • Problems with speech (language) can range from speaking less to total loss of speech, i.e. becoming mute. They may have difficulty finding the right words and may use circumlocution, i.e., talking around the words or describing what they mean. Echoing what has been said by others and stuttering are common symptoms. The person may have difficulty sustaining a train of thought or maintaining a conversation for any length of time. Writing and reading are also affected.

In the early stage of frontotemporal dementia, behaviour changes or problems with speech (language) can appear separately. As the disease progresses, these two areas will overlap. Unlike Alzheimer's disease, a person with frontotemporal dementia often remains oriented to time and memory is not a problem in the early stages. In the later stages of the disease, general symptoms of dementia arise, i.e. confusion and forgetfulness. Motor skills are lost and swallowing difficulties occur.

Diagnosis of frontotemporal dementia

Assessment is important to rule out other problems, for example, primary psychiatric disorder, for which it may be mistaken. No single test can diagnose frontotemporal dementia. Doctors diagnose the disease through a process of identifying characteristic features of the disease and ruling out other possible causes. Cognitive (thinking) tests may be done to assess which brain functions are affected. A history will be taken from the person, family and caregivers, to get a detailed picture of what has been happening. Brain imaging (such as with an MRI) can also be helpful in making a diagnosis of frontotemporal dementia.

Risk factors for frontotemporal dementia

Little is known about the cause of frontotemporal dementia and risk factors have yet to be identified. While most cases are not inherited, there is one extremely rare type of frontotemporal dementia which can be passed from generation to generation.

Treatment for frontotemporal dementia

At present, there is no known cure and no effective way to slow its progression. Cholinesterase inhibitors, used as treatment for Alzheimer's disease, usually do not work for the treatment of frontotemporal dementia, as different areas of the brain are affected.

Much can be done, however, to help manage the symptoms. It is important to learn as much as possible about the disease, to help understand and respond to the person’s new behaviours. People around the individual will need to seek support to learn coping strategies to work around the individual’s behaviour, rather than trying to get the person to change. Speech pathologists may be helpful in teaching strategies to work around the communication problems.

These three videos share the personal stories of caregivers to people living with frontotemporal dementia.

For more information:

[The contents of this page are provided for information purposes only, and do not represent advice, an endorsement or a recommendation, with respect to any product, service or enterprise, and/or the claims and properties thereof, by the Alzheimer Society of Canada. This information sheet is not intended to replace clinical diagnosis by a health professional.]

Source: National Institute of Neurological Disorders and Stroke
http://www.ninds.nih.gov/disorders/picks/picks.htm


Last Updated: 04/03/14