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Frontotemporal Dementia

Frontotemporal Dementia (FTD)

FTD is one of the most common causes of early onset dementia, producing changes in behavior, language, and executive function

Disease Background

  • FTD is a disorder that affects the frontal and temporal lobes of the brain, areas that control personality, behavior, and language

    • FTD is often misdiagnosed as more common disorders, such as Alzheimer’s disease, but FTD tends to occur at a younger age (40-65 years) than Alzheimer’s

  • FTD presents as a rapidly worsening clinical syndrome. Changes in personal and social conduct occur in the early stages of the disease and may include:

    • Loss of inhibition

    • Apathy

    • Social withdrawal

    • Hyperorality (mouthing of objects)

    • Ritualistic compulsive behaviors

  • Symptoms are severely disabling and may lead to misdiagnosis as a psychological or emotionally based problem, or, in the elderly, be mistaken for withdrawal or eccentricity

  • FTD may progress to immobility and loss of speech and expression

  • Survival averages 8 years after the onset of neurocognitive deterioration

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Mechanism Of Disease

  • Up to 40% of all people with FTD have a family history of dementia; approximately 30% of patients with FTD show a clear autosomal dominant inheritance pattern, where one copy of a mutated gene causes the genetic condition

  • The three genes most commonly involved in hereditary FTD are the granulin (GRN), C9orf72 and MAPT

  • In up to 10% of people with FTD, the disease is caused by mutations in the GRN, causing deficiency of progranulin (PGRN)

  • PGRN is a complex and highly conserved protein thought to play several critical roles in lysosomal function

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People with frontotemporal dementia may be eligible for enrollment in clinical trials of investigative therapies, so early diagnosis is critical. Caregivers should reach out to their healthcare professional to learn more.

Incidence

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Rare and underserved populations with estimated U.S. prevalence of ~3,000 to 6,000 people.

Management

  • There are currently no disease-modifying therapies approved for the treatment of FTD

  • Passage Bio is investigating PBFT02, a novel gene therapy for FTD-GRN

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Key collaboration that facilitates genetic screening and counseling

Informed DNA, a partnership that involves designing and supporting a genetic screening and counseling program that will be free of charge to patients with frontotemporal disease.

Learn more at informeddna.com.

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Learn how we are supporting patients and caregivers