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Related Dementias

Symptoms of Frontotemporal Disorders

Symptoms of frontotemporal disorders vary from person to person and from one stage of the disease to the next as different parts of the frontal and temporal lobes are affected. In general, changes in the frontal lobe are associated with behavioral symptoms, while changes in the temporal lobe lead to language and emotional disorders.Older woman having difficulty writing

Symptoms are often misunderstood. Family members and friends may think that a person is misbehaving, leading to anger and conflict. For example, a person with bvFTD may neglect personal hygiene or start shoplifting. It is important to understand that people with these disorders cannot control their behaviors and other symptoms. Moreover, they lack any awareness of their illness, making it difficult to get help.

Behavioral Symptoms

  • Problems with executive functioning—Problems with planning and sequencing (thinking through which steps come first, second, third, and so on), prioritizing (doing more important activities first and less important activities last), multitasking (shifting from one activity to another as needed), and self-monitoring and correcting behavior.
  • Perseveration—A tendency to repeat the same activity or to say the same word over and over, even when it no longer makes sense.
  • Social disinhibition—Acting impulsively without considering how others perceive the behavior. For example, a person might hum at a business meeting or laugh at a funeral.
  • Compulsive eating—Gorging on food, especially starchy foods like bread and cookies, or taking food from other people’s plates.
  • Utilization behavior—Difficulty resisting impulses to use or touch objects that one can see and reach. For example, a person picks up the telephone receiver while walking past it when the phone is not ringing and the person does not intend to place a call.

Language Symptoms

  • Aphasia—A language disorder in which the ability to use or understand words is impaired but the physical ability to speak properly is normal.
  • Dysarthria—A language disorder in which the physical ability to speak properly is impaired (e.g., slurring) but the message is normal.

People with PPA may have only problems using and understanding words or also problems with the physical ability to speak. People with both kinds of problems have trouble speaking and writing. They may become mute, or unable to speak. Language problems usually get worse, while other thinking and social skills may remain normal longer before deteriorating.

Emotional Symptoms

  • Apathy—A lack of interest, drive, or initiative. Apathy is often confused with depression, but people with apathy may not be sad. They often have trouble starting activities but can participate if others do the planning.
  • Emotional changes—Emotions are flat, exaggerated, or improper. Emotions may seem completely disconnected from a situation or are expressed at the wrong times or in the wrong circumstances. For example, a person may laugh at sad news. Pseudobulbar affect, a condition of sudden, frequent outbursts of crying or laughing that cannot be controlled, may occur and is treatable.
  • Social-interpersonal changes—Difficulty “reading” social signals, such as facial expressions, and understanding personal relationships. People may lack empathy—the ability to understand how others are feeling—making them seem indifferent, uncaring, or selfish. For example, the person may show no emotional reaction to illnesses or accidents that occur to family members.

Movement Symptoms

  • Dystonia—Abnormal postures of body parts such as the hands or feet. A limb may be bent stiffly or not used when performing activities that are normally done with two hands.
  • Gait disorder—Abnormalities in walking, such as walking with a shuffle, sometimes with frequent falls.
  • Tremor—Shakiness, usually of the hands.
  • Clumsiness—Dropping of small objects or difficulty manipulating small items like buttons or screws.
  • Apraxia—Loss of ability to make common motions, such as combing one's hair or using a knife and fork, despite normal strength.
  • Neuromuscular weakness—Severe weakness, cramps, and rippling movements in the muscles.

 

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

For More Information About Symptoms of Frontotemporal Disorders

NIA Alzheimer’s and related Dementias Education and Referral (ADEAR) Center
1-800-438-4380 (toll-free)
adear@nia.nih.gov
www.nia.nih.gov/alzheimers
The National Institute on Aging’s ADEAR Center offers information and free print publications about Alzheimer’s disease and related dementias for families, caregivers, and health professionals. ADEAR Center staff answer telephone, email, and written requests and make referrals to local and national resources.

Association for Frontotemporal Degeneration
1-866-507-7222 (toll-free)
www.theaftd.org

National Aphasia Association
1-800-922-4622 (toll-free)
naa@aphasia.org
www.aphasia.org

CurePSP
1-800-457-4777
info@curepsp.org
www.curepsp.org

National Institute of Neurological Disorders and Stroke
1-800-352-9424 (toll-free)
braininfo@ninds.nih.gov
www.ninds.nih.gov