Frontotemporal Disorders: Information for Patients, Families, and Caregivers

Types of Frontotemporal Disorders

Frontotemporal disorders can be grouped into three types, defined by the earliest symptoms physicians identify when they examine patients.

In the early stages it can be hard to know which of these disorders a person has because symptoms and the order in which they appear can vary widely from one person to the next. Also, the same symptoms can appear in different disorders. For example, language problems are most typical of primary progressive aphasia but can also appear later in the course of behavioral variant frontotemporal dementia. The table below summarizes the three types of frontotemporal disorders and lists the various terms that could be used when clinicians diagnose these disorders.

Trouble with words

person's mouthAlicia's first symptom was trouble talking. She spoke more slowly and thought she sounded stilted. She could understand people well enough, but finding the right words when she was talking became harder and harder. Also, Alicia, 49, could not write words like "and" and "it" but could write words like "alligator." Her doctor recommended a neurological exam, which helped diagnose agrammatic PPA.

 

Types of Frontotemporal Disorders
Diagnostic Terms Main Early Symptoms
Progressive Behavior/Personality Decline
  • Behavioral variant frontotemporal dementia (bvFTD)
  • Temporal/frontal variant FTD (tvFTD, fvFTD)
  • Pick’s disease
  • Apathy, reduced initiative
  • Inappropriate and impulsive behaviors
  • Emotional flatness or excessive emotions
  • Memory generally intact
Progressive Language Decline
  • Primary progressive aphasia (PPA)
  • Progressive nonfluent aphasia
  • Semantic dementia
  • Semantic PPA (also called semantic dementia): can’t understand words or recognize familiar people and objects
  • Agrammatic PPA (also called progressive nonfluent aphasia): omits words that link nouns and verbs (such as to, from, the)
  • Logopenic PPA: trouble finding the right words while speaking, hesitation, and/or pauses in speech
Progressive Motor Decline
  • Corticobasal syndrome (CBS)
  • Muscle rigidity
  • Difficulty closing buttons, operating simple appliances; difficulty swallowing
  • Language or spatial orientation problems
  • Progressive supranuclear palsy (PSP)
  • Progressive problems with balance and walking
  • Slow movement, falling, body stiffness
  • Restricted eye movements
  • FTD with parkinsonism
  • Movement problems similar to Parkinson’s disease, such as slowed movement and stiffness
  • Changes in behavior or language
  • FTD with amyotrophic lateral sclerosis (FTD-ALS)
  • Combination of FTD and ALS (Lou Gehrig’s disease)
  • Changes in behavior and/or language
  • Muscle weakness and loss, fine jerks, wiggling in muscles

Behavioral Variant Frontotemporal Dementia

The most common frontotemporal disorder, behavioral variant frontotemporal dementia (bvFTD), involves changes in personality, behavior, and judgment. People with this dementia can act strangely around other people, resulting in embarrassing social situations. Often, they don’t know or care that their behavior is unusual and don’t show any consideration for the feelings of others. Over time, language and/or movement problems may occur, and the person needs more care and supervision.

In the past, bvFTD was called Pick's disease, named after Arnold Pick, the German scientist who first described it in 1892. The term Pick’s disease is now used to describe abnormal collections in the brain of the protein tau, called "Pick bodies." Some patients with bvFTD have Pick bodies in the brain, and some do not.

Primary Progressive Aphasia

Asian couple, woman and man
Three types of frontotemporal disorders—behavioral variant frontotemporal dementia, primary progressive aphasia, and movement disorders—can affect middle-aged and older adults.

Primary progressive aphasia (PPA) involves changes in the ability to communicate—to use language to speak, read, write, and understand what others are saying. Problems with memory, reasoning, and judgment are not apparent at first but can develop over time. In addition, some people with PPA may experience significant behavioral changes, similar to those seen in bvFTD, as the disease progresses.

There are three types of PPA, categorized by the kind of language problems seen at first. Researchers do not fully understand the biological basis of the different types of PPA. But they hope one day to link specific language problems with the abnormalities in the brain that cause them.

"What do you mean by salt?"

salt shaker spilling saltJane, 62, a university professor, began having trouble remembering the names of common objects while she lectured. She also had a hard time following conversations, especially when more than one person was involved. Her family and co-workers were unaware of Jane’s difficulties—until she had a hard time recognizing longtime colleagues. One night at the dinner table, when Jane’s husband asked her to pass the salt, she said, "Salt? What do you mean by salt?" He took her to a neurologist, who diagnosed semantic PPA. As her illness progressed, Jane developed behavioral symptoms and had to retire early.

In semantic PPA, also called semantic dementia, a person slowly loses the ability to understand single words and sometimes to recognize the faces of familiar people and common objects.

In agrammatic PPA, also called progressive nonfluent aphasia, a person has more and more trouble producing speech. Eventually, the person may no longer be able to speak at all. He or she may eventually develop movement symptoms similar to those seen in corticobasal syndrome.

In logopenic PPA, a person has trouble finding the right words during conversation but can understand words and sentences. The person does not have problems with grammar.

Movement Disorders

Two rare neurological disorders associated with FTLD, corticobasal syndrome (CBS) and progressive supranuclear palsy (PSP), occur when the parts of the brain that control movement are affected. The disorders may affect thinking and language abilities, too.

CBS can be caused by corticobasal degeneration—gradual atrophy and loss of nerve cells in specific parts of the brain. This degeneration causes progressive loss of the ability to control movement, typically beginning around age 60. The most prominent symptom may be the inability to use the hands or arms to perform a movement despite normal strength (called apraxia). Symptoms may appear first on one side of the body, but eventually both sides are affected. Occasionally, a person with CBS first has language problems or trouble orienting objects in space and later develops movement symptoms.

Confusing symptoms

woman's hand writing on paperCarol had a tingling sensation and numbness in her upper right arm. Then her arm became stiff. She had to change from cursive handwriting to printing. Carol, 61, told her doctor that she had trouble getting her thoughts out and described her speech as “stumbling.” She had increasing trouble talking but could still understand others. Eventually, she was diagnosed with CBS.

PSP causes problems with balance and walking. People with the disorder typically move slowly, experience unexplained falls, lose facial expression, and have body stiffness, especially in the neck and upper body—symptoms similar to those of Parkinson’s disease. A hallmark sign of PSP is trouble with eye movements, particularly looking down. These symptoms may give the face a fixed stare. Behavior problems can also develop.

Other movement-related frontotemporal disorders include frontotemporal dementia with parkinsonism and frontotemporal dementia with amyotrophic lateral sclerosis (FTD-ALS).

Frontotemporal dementia with parkinsonism can be an inherited disease caused by a genetic tau mutation. Symptoms include movement problems similar to those of Parkinson’s disease, such as slowed movement, stiffness, and balance problems, and changes in behavior or language.

FTD-ALS is a combination of bvFTD and ALS, commonly called Lou Gehrig’s disease. Symptoms include the behavioral and/or language changes seen in bvFTD as well as the progressive muscle weakness seen in ALS. Symptoms of either disease may appear first, with other symptoms developing over time. Mutations in certain genes have been found in some patients with FTD-ALS.

Trouble with walking

foot in boot on stepFor a year and a half, John had trouble walking and fell several times. He also had trouble concentrating. He couldn’t read because the words merged together on the page. John, 73, also seemed less interested in social activities and projects around the house. His wife noticed that he was more irritable than usual and sometimes said uncharacteristically inappropriate things. John’s primary care doctor did several tests, then referred him to a neurologist, who noted abnormalities in his eye movements and diagnosed PSP.

Fecha de publicación: Junio 2014
Última actualización: Agosto 11, 2014