Introduction

Frontotemporal disorders are currently widely underdiagnosed. FTD can be an inherited disease, but if no one in the family had it, perhaps the genetic mutation started with the person under your care. If so, his or her children have a 50% chance of getting it too. FTD is fatal, and most people die within five to 15 years after their first symptoms. There is no treatment, no cure, and currently no way to slow the ongoing course of the disease.

Frontotemporal Dementia (FTD) describes a clinical syndrome associated with shrinking of the frontal and temporal anterior lobes of the brain. Originally known as Pick's disease, the name and classification of FTD has been a topic of discussion for over a century. The presence of abnormalities in the nerve cells of the brain, called Pick bodies, distinguishes frontal lobe dementia from other types of dementia.

As it is defined today, the symptoms of FTD fall into two clinical patterns that involve either:

The first type features behavior that can be either impulsive (disinhibited) or bored and listless (apathetic). It also includes inappropriate social behavior; lack of social tact; lack of empathy; distractibility; loss of insight into the behaviors of oneself and others; an increased interest in sex; changes in food preferences; agitation or, conversely, blunted emotions; neglect of personal hygiene; repetitive or compulsive behavior, and decreased energy and motivation. Often, the patient with FTD now has no personality, no likes, no dislikes, and will never smile. They will no longer recognize their family members. The doctors may diagnose this person with major depression, schizophrenia, bipolar and use other psychiatric terms.

Frontotemporal Dementia

The second type primarily features symptoms of language disturbance, including difficulty making or understanding speech, often in conjunction with the behavioral type's symptoms. Spatial skills and memory remain intact.

A higher proportion of FTD cases seem to have a familial component than more common neurodegenerative diseases like Alzheimer's disease. There is a strong genetic component to the disease, and FTD often runs in families. A variety of mutations on several different genes have been linked to specific subtypes of frontotemporal dementia. However, more than half the people who develop frontotemporal dementia have no family history of dementia. The risk of developing frontotemporal dementia is higher if you have a family history of dementia. There are no other known risk factors.

More mutations and genetic variants are being identified all the time, so the list of genetic influences requires constant and consistent updating. Signs and symptoms typically manifest in late adulthood, usually between the ages of 45 and 64, equally affecting men and women. FTD accounts for about 20% of young onset dementia cases. Currently, there is no cure for FTD.

Caregiving Challenges

Often the FTD patient will become incontinent. They do not shower, brush their teeth or change clothes without supervision and prompting. They may even eat anything and everything in sight to include paper, pens and plants. The daily ongoing challenges can be daunting for the caregivers. Creating a safe environment is going to be the utmost goal. The other challenge is finding a place for them to live. A secure residence is a must, but may be more difficult to find since these disorders affect younger persons and will require more homework to find the right community that is also affordable.



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