What Are the Stages of Frontotemporal Dementia?

Learn about the symptoms associated with the early, middle, and late stages of frontotemporal dementia.

On the surface, Joseph, Barbara, and Lloyd may not seem to be experiencing related changes. Each of them, though, turned out to be in the early stage of frontotemporal dementia (FTD). FTD is the diagnosis for about 5 percent of people with major neurocognitive disorders (dementia). About 70 percent of cases begin before age 65, so it is a more common dementia among the “young old.” FTD involves degeneration of the frontal and temporal lobes of the brain. The frontal lobes are important regulators of behavior and the temporal lobes assist in our understanding and expression of language. The symptoms of FTD, therefore, include major changes in behavior, impairment of language, or both.

People diagnosed with FTD and family members often ask “What stage of dementia is occurring?” and the answer to this question can be useful in knowing what to expect in the future. FTD often begins during years when adults expect to be healthy and productive. The average course of the disease takes 6 to 8 years after diagnosis, so patients and their families must confront very serious and increasingly difficult needs.  Detailed and complex timelines of the stages of FTD are not very accurate, but it is useful to think of the development of FTD through early, middle, and late stages. Let’s look at the stages of frontotemporal dementias to see how these related disorders evolve:

Early-Stage Frontotemporal Dementia

It is in the early stage of FTD that each syndrome shows its most unique features. Memory is often spared at the beginning, and perhaps for this reason early stage FTD can easily be overlooked or misdiagnosed as a psychiatric condition.

The behavioral type of FTD, called behavioral variant FTD, affects social and personal behavior early on. Like Joseph, a person with behavioral variant FTD can start to disregard the usual social boundaries and say or do inappropriate things. They can behave impulsively, carelessly, or even criminally. Judgment and handling of money may deteriorate. Apathy is common and the person loses interest in hobbies and self-care. Empathy or concern for others’ feelings and needs often diminishes.

The language-affecting types of FTD come in two varieties, paired together as primary progressive aphasia:

• Semantic variant primary progressive aphasia, in the early stage, is characterized by loss of names for people, places and objects, word-finding difficulties, and difficulty understanding specific single words. As in Barbara’s case, grammar may remain correct despite trouble grasping the meaning of specific words. Behavioral changes are common, too, including irritability, trouble sleeping, depression, and emotional withdrawal. Selective eating and compulsive behaviors can develop.




• Non-fluent variant primary progressive aphasia, on the other hand, shows itself through the development of labored and halting speech, like Lloyd’s. Grammar is misused and speech sounds can be distorted. Patients are able to understand single words and simple sentences but get confused with more complicated sentences such as “The dog that belonged to Billy was running away.”

Middle-Stage Frontotemporal Dementia

In its later phases, the symptoms of FTD variants become more similar and FTD also looks more similar to other dementias such as Alzheimer’s disease. In behavioral variant FTD, people are likely to need more assistance with basic daily tasks, the so-called “activities of daily living” or ADLs such as dressing, bathing, and grooming. Disturbances of behavior become more frequent and consistent. Those whose problems were initially more behavioral can develop language difficulties and those whose language was more impaired early can develop behavior problems.

Late-Stage Frontotemporal Dementia

In the late stage, people with FTD look more similar to those whose dementia is due to Alzheimer’s disease. Both language and behavior are affected and memory deterioration often occurs as well. It may be necessary to have care 24 hours per day to assure safety and adequate care. Death may eventually occur as a result of infections such as pneumonia.

Summary

As yet, FTD has no specific medication or treatment, but there are valuable information resources for caregivers and patients through the Association for Frontotemporal Dementia (AFTD). The behavioral symptoms of FTD sometimes respond to off-label medications to help with apathy, depression, mania, agitation, irritability, aggression, or delusions. Cognitive rehabilitation and speech therapy may address some language difficulties. Many researchers are seeking the understand the genetics, pathophysiology, and potential treatments for FTD, in the hope that research will eventually identify disease-modifying or preventive treatments.