FTD and 11/13 lecture

What is FTD?

  • Frontotemporal Degeneration Dementia (FTD) - An umbrella term that encompasses various forms of dementia characterized by changes in thinking, language, memory, behavior, and more.

    • Includes several types:

    • Alzheimer's disease

    • Lewy body dementia

    • Frontotemporal dementia

    • Vascular dementia

    • Mixed dementias

Impact of FTD on the Brain

  • Specific areas affected: - Frontal lobe functions impacted include:

    • Reasoning

    • Decision making

    • Behavior control

    • Executive functions

    • Problem solving

    • Attention and concentration

Types of FTD

  • Three different types of FTD: 1. Progressive Behavior/Personality Change

    • Behavioral variant, which may resemble mental illness or criminal behavior.

    1. Progressive Language Change

    • Primary Progressive Aphasia (PPA) with subtypes: - Nonfluent/Agrammatic variant

      • Semantic variant

      • Logopenic variant

      • Example: Bruce Willis has been diagnosed with this variant.

    1. Progressive Motor Function/Movement Change

    • Includes conditions like Corticobasal syndrome and Progressive Supranuclear Palsy (PSP)

Diagnosis of FTD

  • Challenges in Diagnosis: - Diagnosis is often difficult.

    • Can be diagnosed through:

    • Imaging studies

    • Neurophysiological testing

    • Evaluating symptoms

    • Definitive diagnosis is usually obtained through autopsy or genetic testing.

Behavioral Symptoms of FTD

  • Common behavioral symptoms include: - Apathy

    • Disinhibition

    • Loss of empathy

    • Executive dysfunction

    • Compulsive behavior

    • Hyperorality

Language Symptoms of FTD

  • Language symptoms may comprise: - Changes in finding words

    • Changes in understanding words

    • Changes in word order

Movement Symptoms of FTD

  • Physical symptoms can include: - Muscle weakness

    • Muscle rigidity

Interventions for FTD

  • Current state of treatment: - There is no definitive treatment available currently.

    • Active research is ongoing to find biomarkers for diagnosis and treatment.

    • Few effective treatments exist to manage symptoms, including:

    • Off-label medication usage

    • Supportive therapies: - Speech therapy

      • Physical therapy

      • Occupational therapy

      • Support groups

      • Palliative care

      • Creation of dementia-friendly spaces

    • Life Expectancy: FTD is a terminal disease with a life expectancy around the 50s.

Biomedical Perspective on FTD

  • FTD is intertwined with genetics, pathology, and brain circuitry.

  • The study of early-onset dementias is essential for developing future therapies.

Case Studies and Personal Accounts

  • FTD Fighters: - Example: Steve, husband of an advocate, was a Fairfax firefighter who became ill after exposure to burn pits post 9/11.

    • Symptoms included writing issues, speech decline, inability to talk, dysphasia, short term memory loss, and loss of speech (PPA).

    • He became a brain donor for an FTD study at the Mayo Clinic.

    • Example: Natasha Jasso Smith, diagnosed at 47, became a passionate advocate for FTD awareness.

    • Took 3 years for diagnosis and has shared her story at national conferences.

Advocacy for FTD

  • Federal Advocacy Efforts:

    • Promoting innovation in FTD research and drug development.

    • Testifying in Congress to increase federal funding for FTD research.

    • Support for the NAPA Act: a plan emphasizing ongoing funding for national strategies addressing Alzheimer’s disease.

    • Aiming to improve quality dementia care, access to services, and support for family caregivers.

  • State Advocacy Initiatives:

    • Proclamations for FTD Awareness Week.

    • Education programs targeting first responders regarding FTD.

    • Joint resolutions to recognize FTD week at state levels, including lobby days for rare diseases and Alzheimer’s/FTD.

    • Meetings with legislators to reform laws in Virginia related to Temporary Detention Orders (TDOs).

  • W&M Campus Advocacy:

    • Neuroscience student organization involved in hosting a 5K event to raise awareness for FTD.

Neuroscience Concepts Related to FTD

  • Balint Syndrome:

    • Caused by a stroke in the right parietal lobe, leading to difficulties in moving the right hand to specified areas in space.

    • Results in the ability to only see one visual stimulus at a time.

  • Hemispatial Neglect:

    • Occurs due to damage on one side of the parietal lobe, leading to ignoring stimuli from one side of space, including people, objects, and sounds.

    • Individuals may be drawn only to the non-neglected side.

  • Anagnosia:

    • A condition in which an individual fails to recognize their illness or acknowledge a problem.

Circadian Rhythms and Sleep

  • Overview of Circadian Rhythms:

    • Humans have historically studied the passage of time, characterized by daily, seasonal, and yearly periodicities.

    • Daily cycles generally last approximately 24 hours and involve various changes, such as:

    • Genes turning on or off

    • Behavioral changes

    • Mood fluctuations

    • Variations in motivation and cognition

  • Molecular Clock in Mammals:

    • Every mammalian cell has a molecular clock regulated by a master clock—the Suprachiasmatic Nucleus (SCN) located in the hypothalamus, where both optic nerves converge.

    • Important functions regulated by the SCN include:

    • Eating

    • Sleeping

    • Drinking

    • Physical activity

    • Sex drive

    • Hormonal activities

    • Social interactions

  • Pineal Gland Role:

    • When active during daylight, the SCN causes the pineal gland to be inactive, resulting in decreased melatonin production (leading to wakefulness).

    • Conversely, when the SCN is inactive at night, the pineal gland is active, producing more melatonin, which promotes sleep.

Defining Sleep

  • Sleep Characteristics: - Sleep is defined as both a behavioral state and a physiological state encompassing consciousness.

    • It should not be confused with unconsciousness.