Expert

What Are the Stages of Frontotemporal Dementia?

Swank Memory Care Center, Christiana Care Health System
A neurologist talking with a person about the stages of frontotemporal dementia
Learn about the symptoms associated with the early, middle, and late stages of frontotemporal dementia.

Joseph’s wife, Emily, was shocked when he approached a young woman in the street, a stranger to him, and complimented her attractive dress. Looking back, Emily had to admit her husband had been acting strangely for several months. Even before his retirement the year before, at age 62, he’d been getting less tidy, more irritable, and less considerate. When Emily was upset about a conflict at work, Joseph shrugged off her concern and didn’t seem to care as he always had in the past. Joseph didn’t seem to enjoy retirement as much as Emily thought he would. He didn’t show much motivation to keep appointments or do things around the house, and his hobbies held less interest for him than in the past. Emily made an appointment for Joseph’s condition to be evaluated.

Barbara, a former novelist, found it more and more difficult to write as she neared her 60th birthday. She couldn’t find the words she wanted as easily, and couldn’t understand some words that were previously familiar, not just names but also more common nouns. Her spelling seemed worse, too, which was a real change for a former spelling-bee champion like her. She was short- tempered at times, had difficulty sleeping, and seemed to have developed new and peculiar food preferences. Barbara’s son brought her for evaluation.

Lloyd, 59 years old and still working hard to put his children through college, had been a top car salesman for the previous decade. His performance was slipping, for no apparent reason. When he talked with his customers, his speech was different – halting and grammatically incorrect. He wasn’t connecting with them as well as he always had previously. Something seemed to be wrong and his sister suggested he seek evaluation.

*The names and details in this story are composite and fictitious. They do not identify specific individuals.

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.

Resources:

Further Reading:

This content was first posted on: October 7, 2019

The information provided here is a public service of the BrightFocus Foundation and should not in any way substitute for personalized advice of a qualified healthcare professional; it is not intended to constitute medical advice. Please consult your physician for personalized medical advice. BrightFocus Foundation does not endorse any medical product, therapy, or resources mentioned or listed in this article. All medications and supplements should only be taken under medical supervision. Also, although we make every effort to keep the medical information on our website updated, we cannot guarantee that the posted information reflects the most up-to-date research.

These articles do not imply an endorsement of BrightFocus by the author or their institution, nor do they imply an endorsement of the institution or author by BrightFocus.

Some of the content may be adapted from other sources, which will be clearly identified within the article.

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