Understanding Frontotemporal Dementia (FTD)

What is Frontotemporal Dementia?

Frontotemporal dementia (FTD) is a group of brain disorders that primarily affect the frontal and temporal lobes of the brain. These areas control behavior, personality, emotions, language, judgment, and decision-making.

Unlike Alzheimer's disease, which often begins with memory loss, the first signs of FTD are usually changes in behavior, personality, or language. FTD most commonly develops at a younger age than most other dementias, usually between the ages of 45 and 65, though in some individuals it can occur earlier or later in life.

While historically and commonly referred to as one disease, it should be noted that FTD represents several different syndromes with different underlying disease pathologies in the brain:

The major clinical variants of frontotemporal dementia (FTD) are:

1. Behavioral Variant Frontotemporal Dementia (bvFTD) (most common)
2. Primary Progressive Aphasia (PPA)
   • Nonfluent/Agrammatic Variant PPA (nfvPPA)
   • Semantic Variant PPA (svPPA)
   • Logopenic Variant PPA (lvPPA)* (usually due to Alzheimer's disease pathology rather than FTLD)
3. Frontotemporal Dementia with Motor Neuron Disease (FTD-MND or FTD-ALS)
4. Corticobasal Syndrome (CBS)
5. Progressive Supranuclear Palsy (PSP)

Common Symptoms

Symptoms vary depending on which subtype of FTD, and which part of the brain is affected.

Changes in Behavior (bvFTD)

A person with FTD may:

• Act impulsively or make poor decisions.
• Show less empathy or concern for others.
• Become socially inappropriate.
• Lose interest in activities they once enjoyed.
• Develop repetitive behaviors or routines.
• Overeat or crave sweets and carbohydrates.
• Have difficulty planning, organizing, or completing tasks.
• Have an artistic bloom - become very creative

Changes in Language (PPA)

Some people develop problems with communication instead of behavior. They may:

• Have trouble finding the right words.
• Speak less than usual.
• Have difficulty understanding words or conversations.
• Speak in short, simple sentences.
• Eventually have increasing difficulty communicating

Movement Problems (others)

A smaller number of people with FTD develop movement symptoms such as:

• Stiffness or slowed movements.
• Muscle weakness.
• Balance problems or falls.
• Difficulty swallowing.

The following link may be a place to start keeping track of symptoms if you believe your loved one may have FTD. It is not meant to diagnose FTD, as this can only be done by a medical provider:
https://www.theaftd.org/what-is-ftd/ftd-diagnostic-checklist/

How is FTD Different from Alzheimer's Disease?

What Causes FTD?

The root cause is still largely unknown, though like Alzheimer's, FTD seems to involve abnormalities in certain proteins in affected brain cells. The specific protein involved varies by type of FTD. These protein abnormalities damage the brain cells, which then degenerate over time.

Some people with FTD have a family history of the condition. In some families, genetic testing may be recommended after discussing the benefits and limitations with a healthcare provider or genetic counselor.

How is FTD Diagnosed?

There is no single test for FTD. Unlike Alzheimer's, specific biomarker testing does not widely exist for FTD. Diagnosis usually includes:

• A detailed medical history and physical examination.
• Interviews with family members or caregivers.
• Memory and thinking tests.
• Brain imaging (MRI or CT scan).
• Specialized PET scans (tau-PET, FGD-PET)
• Genetic testing (MAPT, GRN, C9orf72)
• Limited biomarkers (plasma neurofilament light chain + neuro-imaging)

Because symptoms can resemble depression, psychiatric illness, or other neurologic disorders, diagnosis may take time. Many patients with FTD are first diagnosed with schizophrenic or bipolar disease, before realization that it is a dementia disease.

Is There a Treatment?

There is currently no cure for FTD, but treatment focuses on managing symptoms and improving quality of life. There are clinical trials. 

Treatment may include:

• Medications to help with anxiety, depression, impulsivity, or compulsive behaviors.
• Speech-language therapy for communication difficulties.
• Physical or occupational therapy for movement problems.
• Structured daily routines.
• Education and support for caregivers.

Unlike Alzheimer's disease, medications such as donepezil (Aricept®) or memantine (Namenda®) are generally not helpful for most people with FTD and may sometimes worsen behavior. Newer Alzheimer anti-amyloid therapies are also not helpful since FTD does not involve amyloid.

Tips for Families and Caregivers

• Remember that behavior changes are caused by the disease—not intentional actions.
• Keep routines simple and consistent.
• Use short, clear sentences when communicating.
• Avoid arguing or trying to reason during difficult behaviors.
• Reduce distractions and create a calm environment.
• If language difficulties are present, get a referral for specialized speech therapy
• Escalate physically violent behavior: seen in up to 40% of those with FTD. Prioritize caregiver safety
• Escalate frequent falls, gait or balance difficulties
• Plan ahead for future medical, legal, and financial decisions while the person can still participate.
• Seek FTD specific caregiver support: https:/www.theaftd.org/living-with-ftd/aftd-support-groups/. 
  Free virtual and in-person options. 

Outlook

FTD is a progressive condition, meaning symptoms gradually worsen over time. The rate of progression varies from person to person. The order of symptom progression will vary from person to person as well, and it is less predictable than in Alzheimer's. Aspects such as behavioral changes, falls, mobility, hygiene, continence, speech, swallowing may become affected. Severe stages can involve immobility, malnutrition, infection, and recurrent ER visits/hospitalizations.

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Resources

• The Association for Frontotemporal Degeneration (AFTD): Living with FTD and Support Resources – Comprehensive information about FTD, caregiving, support groups, diagnostic checklists, educational materials, and practical resources. 
• National Institute on Aging (NIA): Frontotemporal Disorders – Evidence-based information about the causes, symptoms, diagnosis, treatment, and caregiving for people living with FTD. 
• Alzheimer's Association: Frontotemporal Dementia – Information about the different types of FTD, common symptoms, diagnosis, treatment, and caregiving.
• https://www.caregiver.org/resource/frontotemporal-dementia/ – Caregiver education, practical tips, support services, and additional resources for families caring for someone with FTD.