Frontotemporal Lobar Degeneration

About Frontotemporal Lobar Degeneration

The outer portion of your brain, the cerebral cortex, is responsible for cognition - an umbrella term for the functions your brain performs related to thinking, learning, and memory. The cerebral cortex is divided into four portions called lobes, which each have different roles in cognition. Frontotemporal lobar degeneration (FTLD) is a form of cognitive decline caused by pathological changes to two of these lobes: the frontal lobe, which is responsible for higher-level cognitive tasks such as planning, problem solving, social reasoning, and movement, and the temporal lobe, which is responsible for tasks including language and memory acquisition. In FTLD, the buildup of proteins, such as tau and TDP-43, causes cells in these lobes to die.

FTLD differs from the most common form of dementia, Alzheimer’s disease, in that while memory loss is more common in early stages of Alzheimer’s disease, difficulties with language and/or behavioral changes may be the first signs of FTLD. Additionally, the age of FTLD onset is usually younger than that of Alzheimer’s disease.

Types of Frontotemporal Lobar Degeneration:

Frontotemporal lobar degeneration is categorized by what the initial symptoms are and which parts of the brain are impacted early on.

Types of frontotemporal lobar degeneration include:

• Behavioral variant frontotemporal dementia (bvFTD): FTLD associated with personality and behavioral changes and shrinkage/dysfunction in the frontal lobes
• Language variant frontotemporal dementia (lvFTD): FTLD characterized by impaired language skills, which accounts for many cases of primary progressive aphasia
• Movement variants of frontotemporal dementia (mvFTD): FTLD characterized by changes in thinking as well as movement symptoms like falls, eye movement problems, or problems with skilled movements of the hands. Other labels for this condition can include Progressive Supranuclear Palsy (PSP) or Cortico-Basal Degeneration (CBD).

Symptoms of Frontotemporal Lobar Degeneration

Symptoms of FTLD depend on which lobes of the brain are impacted but can include changes in behavior, personality, reasoning, language, and movement.

Symptoms of behavioral variant frontotemporal dementia may include:

• Apathy
• Difficulty planning or prioritizing tasks
• Impulsive behavior
• Difficulties understanding social behavior
• Repetitive/compulsive behaviors

Symptoms of language variant frontotemporal dementia may include:

• Difficulty producing speech
• Difficulty understanding words

Symptoms of movement variant frontotemporal dementia may include:

• Falls and eye movement problems
• Problems with skilled movements of the hands

Risk Factors for Frontotemporal Lobar Degeneration

While researchers have not yet determined the exact cause of frontotemporal lobar degeneration, certain people are more likely to develop these conditions.

Risk factors for frontotemporal lobar degeneration may include:

• Age: FTLD typically affects individuals earlier in life than other dementias, most commonly emerging in individuals in their 50’s and 60’s; sometimes though the disease comes on later in life.
• Family history: Scientists have identified a handful of genetic mutations linked to different forms of FTLD, though not all cases have a clear genetic linkage

Diagnosing Frontotemporal Lobar Degeneration

FTLD is diagnosed with a diagnostic interview (with both the person experiencing the changes and family) to learn more about the person’s symptoms, a neurological evaluation, neuropsychological evaluation, brain imaging, and sometimes other specialized tests. Diagnosing bvFTD can be challenging and can be misdiagnosed as a mood or psychiatric disorder in the earliest stages.

Treating Frontotemporal Lobar Degeneration at UT Health Austin

While there is no cure for FTLD, some medications can help manage behavioral symptoms. Speech therapy may help with language difficulties. Caregiver support and education, long term care planning, and connection to social supports can help families cope as well.

Our clinic uses an interprofessional approach, which means our patients have contact with multiple team members coming from multiple specialties. We discuss each patient as a team to identify the diagnosis and to develop a tailored treatment plan specific to your needs. Whatever your needs are, our team is here to listen and work with you to develop a treatment plan that is right for you.

Care Team Approach

At UT Health Austin, we take a multidisciplinary approach to your care. This means you will benefit from the expertise of multiple specialists across a variety of disciplines caring for you in one place to avoid having to schedule multiple appointments with providers at locations all over the city. The Comprehensive Memory Center care team includes neurologists, a geriatric psychiatrist, neuropsychologists, nurses, a speech-language pathologist, social workers, and more who work together to help you get back to the things in your life that matter most to you.

We collaborate with our colleagues at the Dell Medical School and The University of Texas at Austin to utilize the latest research, diagnostic, and treatment techniques, allowing us to identify new therapies to improve treatment outcomes. We are committed to communicating and coordinating your care with referring physicians and other partners in the community to ensure that we are providing you with comprehensive, whole-person care.

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