Frontotemporal
Dementia and Pick's Disease
Introduction
Alzheimer's disease is the most common of a large group of disorders known as "dementias." It is an irreversible disease of the brain in which the progressive degeneration of brain cells causes thinking ability and memory to deteriorate. Alzheimer's disease also affects behaviour, mood and emotions, and the ability to perform daily living activities.
There is currently no cure for Alzheimer's disease, but there are treatment options and lifestyle choices that can slow its progression and, within the next five years, treatments are expected that may well stop the disease in its tracks! Also, the pursuit of new research strategies should one day help restore some lost function and memory.
Alzheimer's disease progresses through early, middle and late stages before reaching the final end of life stage. However, identifying the transition from one stage to another is often difficult. Not only does the disease usually progress slowly, but the symptoms related to each stage tend to overlap and the order in which they appear and how long they last varies from person to person.
The duration following diagnosis is usually seven to ten years. However, when the diagnosis is delayed, as it may be if the affected person fails to see a doctor early on, the disease duration is shorter than this. Conversely, as the ability to diagnose Alzheimer's disease improves and people become more willing to be assessed, survival times following diagnosis are lengthening.
"Related dementias" resemble Alzheimer's disease in that they also involve a progressive degeneration of brain cells that is currently irreversible. They include the dementia associated with Vascular Dementia (the second most common dementia after Alzheimer's disease), Frontotemporal Dementia, Creutzfeldt-Jakob Disease, Lewy body Dementia, Parkinson's disease, and Huntington disease.
Sometimes a person may have symptoms such as sudden onset of memory loss, behaviour changes, or difficulties with speech and movement. These symptoms may suggest a dementia other than Alzheimer's disease. Frontotemporal Dementia is one of these dementias. In any event a person should always seek a thorough medical assessment if any of these symptoms are present.
Regardless of the type of dementia, individuals are encouraged to obtain information and support from the Alzheimer Society.
What
is Frontotemporal Dementia?
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 2-5% 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
How
does Frontotemporal Dementia affect the person?
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.
- Problems with speech (language) can range from reduction of speech to total loss, i.e. becoming mute. 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 has preserved memory 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.
How is Frontotemporal Dementia assessed?
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. Brain imaging (such as with an MRI) can also be helpful in making a diagnosis of Frontotemporal Dementia.
What are the risk factors for 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 is 2-10 years. 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.
Is
there a treatment?
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. Treatment currently focuses on managing symptoms.
For
more information:
[The contents of this document 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. The Information Sheet is not intended to replace clinical diagnosis by a health professional.]
|
