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